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however&#44; this role was rejected in later studies&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The literature contains few LyP series and even fewer studies that contrast clinical data with histology and immunohistochemistry findings&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Objectives</span><p id="par0025" class="elsevierStylePara elsevierViewall">Our objectives were to investigate histology and immunohistochemistry findings in skin biopsies from a group of patients with LyP and to describe the clinical presentation&#44; course of the disease&#44; association with other conditions&#44; response to treatment&#44; and potential correlations between these variables and histology findings&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Material and Methods</span><p id="par0030" class="elsevierStylePara elsevierViewall">We performed a retrospective&#44; descriptive&#44; observational study of patients with a clinically and histologically confirmed diagnosis of LyP and with sufficient follow-up data on record&#46; We selected patients using <span class="elsevierStyleItalic">lymphomatoid papulosis</span> as a search term in the histopathology database&#44; which covers the period January 2000 to May 2010&#44; and in the dermatology database&#44; which covers the period January 1995 to May 2010&#46; The databases yielded 44 and 24 patients&#44; respectively&#46; After ruling out patients who did not have an accurate diagnosis&#44; a clinical history&#44; or histology samples and those who were lost to follow-up&#44; we identified 18 patients and 26 biopsy specimens&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">We evaluated the following clinical variables&#58; age at onset&#44; sex&#44; clinical presentation &#40;type of lesions and site&#41;&#44; laboratory results at diagnosis&#44; the course of the lesions&#44; treatments received&#44; and the response to treatment&#46; We also described associations with other lymphomas &#40;both cutaneous and noncutaneous&#41; and the latency period until these associations developed&#46; As for histology findings&#44; we studied type of LyP &#40;A&#44; B&#44; C&#44; or mixed&#41;&#44; type of infiltrate &#40;wedge-shaped or band-like&#41;&#44; presence of epidermotropism&#44; and positivity for CD30 &#40;activated T-cell and B-cell marker and Hodgkin lymphoma cell marker&#41;&#44; CD3 &#40;T-cell marker&#41;&#44; and CD56 &#40;natural killer cell marker&#41;&#44; as well as the intensity of these markers&#46; We also determined the presence of clonal T-cell receptor rearrangement in patients who underwent testing&#46; Immunohistochemistry markers were classified into 5 degrees of intensity &#40;&#43;&#43;&#43;&#44; &#43;&#43;&#44; &#43;&#44; &#43;&#8211;&#44; and &#8211;&#41; based on the consensus reached by 2 observers&#46; These markers were tested using the following reagents&#58;<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">-</span><p id="par0040" class="elsevierStylePara elsevierViewall">CD3&#58; rabbit polyclonal antibody &#40;Dako&#41; IR503&#44; Flex</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">-</span><p id="par0045" class="elsevierStylePara elsevierViewall">CD30&#58; mouse polyclonal antibody &#40;Dako&#41; IR602&#44; Flex</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">-</span><p id="par0050" class="elsevierStylePara elsevierViewall">CD56&#58; synthetic monoclonal antibody diluted 1&#47;25 &#40;Master Diagnostics&#41; Clone IB6</p></li></ul></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Results</span><p id="par0055" class="elsevierStylePara elsevierViewall">The results are summarized in <a class="elsevierStyleCrossRefs" href="#tbl0005">Tables 1 and 2</a>&#46; The study sample comprised 18 patients &#40;10 men and 8 women&#41; with a mean age of 42&#46;7 years &#40;range&#44; 7-70 years&#41;&#46; Onset was mainly in the form of papules &#40;15 patients &#91;83&#37;&#93;&#41; and crusted papules &#40;3 patients &#91;17&#37;&#93;&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The most frequent locations were the extremities &#40;13 patients &#91;72&#37;&#93;&#41;&#44; trunk &#40;4 patients &#91;22&#37;&#93;&#41;&#44; and both &#40;1 patient &#91;6&#37;&#93;&#41;&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0060" class="elsevierStylePara elsevierViewall">The results of a complete blood count and biochemistry &#40;including lactate dehydrogenase&#41; at diagnosis were normal for all patients&#44; as were the results for &#946;<span class="elsevierStyleInf">2</span>-microglobulin in the 5 patients who were tested for it&#46; Mean follow-up after diagnosis was 7 years &#40;range&#44; 2-22 years&#41;&#46; The majority of patients &#40;11 patients&#44; 62&#37;&#41; had a single outbreak that resolved without treatment or with topical treatment&#44; 4 patients &#40;22&#37;&#41; experienced recurrent outbreaks&#44; 2 patients &#40;12&#37;&#41; developed associated mycosis fungoides or had both types of cutaneous T-cell lymphoma as their presenting complaint&#44; and 1 patient continued to be affected by the chronic form&#44; with no response to treatment&#46; Patients who experienced recurrent outbreaks over the years responded well to psoralen&#8211;UV-A during each outbreak&#46; In the patient with chronic disease and the patient with mycosis fungoides presenting simultaneously with LyP&#44; psoralen&#8211;UV-A combined with methotrexate partially controlled the lesions&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">As biopsy had been repeated in some cases during follow-up&#44; 26 specimens were available for the 18 patients&#46; Wedge-shaped infiltrate was more common than band-like infiltrate &#40;78&#37; vs 22&#37;&#41;&#46; The most common histologic pattern was type A &#40;15 patients &#91;83&#37;&#93;&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#59; types B&#44; C&#44; and mixed AB were found in 1 patient each&#46; All the samples were positive for CD30&#59; in most cases this positivity was moderate or intense &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Positivity for CD3 was more intense in the cells of the infiltrate than in the tumor cells &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#44; which were also positive for CD3&#44; although less intensely&#46; Interestingly&#44; among the cells accompanying the tumor cells&#44; one group of cells was positive for CD3 while the other was not&#44; as if the cell populations were different &#40;even though both had similar morphology&#41;&#46; None of the cells were positive for CD56&#44; although in 1 case this finding was doubtful &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Fig&#46; 5</a>&#41;&#46; Epidermotropism was only found in type B and mixed AB&#46; T-cell receptor rearrangement was not detected in the 9 patients who underwent testing&#46; Curiously&#44; findings in repeat biopsy specimens did not vary &#40;3 patients and 7 specimens&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0070" class="elsevierStylePara elsevierViewall"><a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> summarizes the clinical characteristics of the patients and histologic patterns of LyP&#59; no apparent correlation was found between the clinical variables studied and the histologic type&#46; The small sample size prevented us from applying statistical tests with sufficient power to be able to analyze the variables described&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0075" class="elsevierStylePara elsevierViewall">The epidemiologic and clinical findings for our group of patients are consistent with those published in the literature&#44;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> that is&#44; LyP is a lymphoproliferative disease that is slightly more common in men and has a mean age at onset of 40 to 45 years&#46; In most cases&#44; onset takes the form of papules or crusted papules on the limbs&#59; however&#44; several forms have been reported &#40;eg&#44; vesicular&#44; agminated&#44; and ulcerative-necrotic&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">A review of the literature from the last 10 years revealed only 2 series that analyze clinical and histologic findings and their association with other diseases in patients with LyP &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>&#41;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a>&#46; In addition&#44; the Spanish medical literature includes a recently published series comprising 9 pediatric patients with LyP&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Data on the histology of the lesions reported by El Shabrawi-Caelen et al<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> in 2004 are consistent with our results&#44; namely&#44; a clear predominance of type A &#40;75&#37; of patients&#41;&#46; Types B and C were less common&#44; with a frequency of 4&#37; and 13&#37;&#44; respectively&#46; Again&#44; these findings were consistent with those of our series&#44; in which each type affected 6&#37; of patients&#46; Most infiltrates were wedge-shaped&#44; except for those of patients with type B disease&#44; which&#44; given their resemblance to the infiltrates occurring in mycosis fungoides&#44; tended to be band-like&#46; Similarly&#44; in 2000&#44; Bennek et al<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> reported on a series of 118 patients&#44; most of whom were type A &#40;79&#37;&#41;&#46; The frequency of the other types was 5&#37; for type B&#44; 7&#37; for type C&#44; and 9&#37; for the mixed type&#46; The authors provided no immunohistochemistry findings&#46;</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0085" class="elsevierStylePara elsevierViewall">It is important to note that it is not possible to differentiate LyP from other CD30<span class="elsevierStyleSup">&#43;</span> lymphoproliferative disorders on immunohistochemistry findings&#46; Although positivity demonstrates the presence of the disease&#44; some cases are negative&#46; The reasons why LyP is CD30<span class="elsevierStyleSup">&#8211;</span> are not clear and have been reported mainly for type B disease&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> One possible explanation is that manifestations assumed to be compatible with LyP were really those subsequently reported for papular mycosis fungoides and&#44; therefore&#44; indicative of a CD30<span class="elsevierStyleSup">&#8211;</span> disorder&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">CD3 is a pan&#8211;T-cell marker and&#44; as such&#44; positive in almost all cases of LyP&#46; In our series&#44; we found that it was more intensely positive in cells of the infiltrate accompanying large cells&#44; thus suggesting the existence of 2 T-cell populations&#46; However&#44; as this finding was not described in the literature reviewed&#44; further studies are warranted to confirm it&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">We analyzed CD56&#44; because 50&#37; of the LyP biopsy specimens in the series by El Shabrawi-Caelen et al<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> were positive for this marker&#46; In our study&#44; however&#44; we found only 1 case of doubtful CD56 positivity&#46; The literature review showed that positivity for CD56 in LyP remains open to debate and generally tends to be described as very rare&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a> The reasons for such a discordant finding between 2 series published in the last 6 years remain unclear&#44; although one explanation could be the different dilutions of reagent used&#58; 1&#58;20 in the study by El Shabrawi-Caelen et al in 2004&#44; 1&#58;40 in 3 cases of CD56<span class="elsevierStyleSup">&#43;</span> LyP in the study by Flann et al<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a>&#44; and 1&#58;25 in our series&#46;</p><p id="par0100" class="elsevierStylePara elsevierViewall">T-cell receptor testing in patients with LyP is useful for differentiating between lesions involving clonal rearrangement and those of a reactive nature&#46; However&#44; positivity or negativity of the T-cell receptor is merely orientative in clinical practice&#46; Moreover&#44; the role of clonal rearrangement in LyP remains unclear&#58; in 2003&#44; Gellrich et al<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> reported clonality in small CD30<span class="elsevierStyleSup">&#8211;</span> cells in the accompanying infiltrate&#44; whereas in 2002&#44; Steinhoff et al<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> reported clonality in large CD30<span class="elsevierStyleSup">&#43;</span> cells&#46;</p><p id="par0105" class="elsevierStylePara elsevierViewall">Classification of LyP into 3 histologic patterns&#44; both in our study and in the literature reviewed&#44;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a> is more a convention than of relevance to symptoms&#44; prognosis&#44; and response to treatment&#46; No apparent association exists between the histologic pattern and the variables studied&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">Symptoms are self-limiting in most cases &#40;approximately 60&#37; to 70&#37; according to the literature&#41;&#46; However&#44; the risk of progression from LyP to mycosis fungoides has been reported in 10&#37; to 12&#37; of cases&#44; an association with Hodgkin lymphoma in 9&#37; of cases&#44; and an association with cutaneous anaplastic large-cell lymphoma in 9&#37; of cases &#40;<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>&#41;&#46; We detected progression to mycosis fungoides in only 12&#37; of our patients&#59; neither of the other associations was observed&#46; A diagnosis of LyP after a diagnosis of mycosis fungoides must be made with caution&#44; since it may reveal a transformation to large cell disease&#44; thus necessitating a radical change in the treatment and prognosis of the initial mycosis fungoides&#46; Knowledge of these associations in patients with LyP requires us to perform periodic follow-up and 6-monthly monitoring with laboratory tests &#40;including lactate dehydrogenase&#41; and chest x-ray&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">Most cases of LyP present with papular lesions&#44; are self-limiting&#44; and do not require treatment&#46; More persistent outbreaks usually respond well to psoralen&#8211;UV-A&#44; as was the case in 35&#37; of the patients in the series by Bennek et al<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> in 2000 and in 22&#37; of our patients&#46; Low-dose methotrexate is an effective alternative&#46; In the very rare refractory cases&#44; bexarotene&#44;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> interferon alfa&#44;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> topical 5-fluorouracil&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> and photodynamic therapy<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> have been tried&#44; with varying success&#46;</p><p id="par0120" class="elsevierStylePara elsevierViewall">We conclude that LyP affects middle-aged patients in the form of outbreaks of papules on the extremities and trunk&#46; Most cases resolve spontaneously&#44; and the response to treatment is good&#46; The typical histologic finding is a wedge-shaped infiltrate of large CD30<span class="elsevierStyleSup">&#43;</span> cells and&#44; rarely&#44; CD56<span class="elsevierStyleSup">&#43;</span> cells&#46; Follow-up is necessary&#44; as the disease has been reported to be associated with other lymphoproliferative disorders&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0125" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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            0 => "Lymphomatoid papulosis"
            1 => "Findings histologic"
            2 => "Signs and symptoms"
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          "clase" => "keyword"
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            0 => "Papulosis linfomatoide"
            1 => "Hallazgos histol&#243;gicos"
            2 => "Presentaci&#243;n cl&#237;nica"
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        "titulo" => "Abstract"
        "resumen" => "<span class="elsevierStyleSectionTitle">Background</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Lymphomatoid papulosis &#40;LyP&#41; is a CD30<span class="elsevierStyleSup">&#43;</span> lymphoproliferative skin disease that has been described in association with Hodgkin lymphoma&#46; It has also been reported to progress to mycosis fungoides or cutaneous anaplastic large-cell lymphoma&#46;</p> <span class="elsevierStyleSectionTitle">Objective</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">To study the clinical and histologic features of LyP and response to treatment in a patient series&#46;</p> <span class="elsevierStyleSectionTitle">Materials and methods</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">For this retrospective&#44; descriptive&#44; observational study of patients with histologically confirmed LyP and sufficient follow-up data on record&#44; we extracted histologic findings on skin biopsy&#44; clinical presentation&#44; clinical course&#44; and response to treatments&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Eighteen patients &#40;10 male&#44; 8 female&#41; were identified&#46; Most biopsies &#40;14&#47;18&#44; 78&#37;&#41; showed a wedge-shaped lymphocytic infiltrate with CD30<span class="elsevierStyleSup">&#43;</span>&#44; CD3<span class="elsevierStyleSup">&#43;</span>&#44; and CD56<span class="elsevierStyleSup">&#8722;</span> cells&#46; A type A histologic pattern was present in the biopsies of 83&#37; of the patients&#46; The most common presentation &#40;83&#37;&#41; consisted of papules on the trunk&#59; for 62&#37; LyP resolved after a single episode&#46; Twelve percent of the patients developed mycosis fungoides &#40;mean follow-up&#44; 7 years&#41;&#59; no other associations were noted&#46;</p> <span class="elsevierStyleSectionTitle">Discussion</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Although few series of patients with LyP have been published in recent years&#44; the findings reported generally coincide with our observations&#46;</p> <span class="elsevierStyleSectionTitle">Conclusion</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">LyP is typically a CD30<span class="elsevierStyleSup">&#43;</span> lymphoproliferative disorder that usually runs a benign course and responds well to treatment&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Introducci&#243;n</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">La papulosis linfomatoide &#40;PL&#41; es una dermatosis que se engloba dentro de los procesos linfoproliferativos CD30 positivos de la piel&#46; Se ha descrito su asociaci&#243;n a linfoma de Hogking &#40;LH&#41;&#44; as&#237; como su progresi&#243;n a micosis fungoide &#40;MF&#41; y linfoma cut&#225;neo anapl&#225;sico de c&#233;lula grande &#40;LCACG&#41;&#46;</p> <span class="elsevierStyleSectionTitle">Objetivos</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Investigar los hallazgos cl&#237;nicos&#44; histol&#243;gicos y la respuesta al tratamiento en un grupo de pacientes con PL&#46;</p> <span class="elsevierStyleSectionTitle">Material y m&#233;todos</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Se llev&#243; a cabo un estudio retrospectivo&#44; descriptivo y observacional&#46; Se seleccionaron 18 pacientes con diagn&#243;stico histol&#243;gico confirmado de PL y con un adecuado seguimiento cl&#237;nico&#46; Se recopilaron los hallazgos histol&#243;gicos de las biopsias de piel&#44; la forma de presentaci&#243;n&#44; la evoluci&#243;n y la respuesta a los tratamientos utilizados&#46;</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Se reclutaron un total de 18 pacientes&#44; 10 varones y 8 mujeres&#46; La mayor&#237;a de las biopsias&#44; 14 de 18 &#40;78&#37;&#41; mostraban un infiltrado linfocitario en cu&#241;a&#44; CD30 positivo&#44; CD3 positivo y CD56 negativo&#46; El tipo histol&#243;gico m&#225;s frecuente fue el tipo A&#44; presente en un 83&#37; de las biopsias de los pacientes&#46; La forma cl&#237;nica de presentaci&#243;n m&#225;s frecuente fue en forma de p&#225;pulas en el tronco &#40;83&#37;&#41;&#46; Un 62&#37; de los pacientes sufri&#243; un &#250;nico brote autorresolutivo&#46; La media de seguimiento fue de 7 a&#241;os&#44; durante los cuales un 12&#37; de los pacientes desarroll&#243; una micosis fungoide&#44; sin encontrarse otras asociaciones&#46;</p> <span class="elsevierStyleSectionTitle">Discusi&#243;n</span><p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Existen pocas series de pacientes con PL publicadas en los &#250;ltimos a&#241;os&#59; sin embargo&#44; globalmente los hallazgos descritos en ellas coinciden con las de nuestro grupo de pacientes&#46;</p> <span class="elsevierStyleSectionTitle">Conclusiones</span><p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">La PL es un cuadro linfoproliferativo t&#237;picamente CD30 positivo que habitualmente tiene un curso benigno con buena respuesta a los tratamientos utilizados&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article&#58; Fern&#225;ndez-Guarino M&#44; et al&#46; Papulosis linfomatoide&#58; hallazgos cl&#237;nico-patol&#243;gicos en 18 pacientes&#46; Actas Dermosifiliogr&#46;2012&#59;103&#58;388-93&#46;</p>"
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          "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Typical image of a lymphomatoid papulosis lesion&#46; Papule with a central desquamative scab&#46;</p>"
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          "en" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">Intense CD30 positivity in type A lymphomatoid papulosis &#40;CD30&#44; original magnification &#215;30&#41;&#46;</p>"
        ]
      ]
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        "identificador" => "fig0020"
        "etiqueta" => "Figure 4"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr4.jpeg"
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            "Tamanyo" => 221054
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        "descripcion" => array:1 [
          "en" => "<p id="spar0080" class="elsevierStyleSimplePara elsevierViewall">CD3 positivity in the large cells of type A lymphomatoid papulosis &#40;CD30&#44; original magnification &#215;30&#41;&#46;</p>"
        ]
      ]
      4 => array:7 [
        "identificador" => "fig0025"
        "etiqueta" => "Figure 5"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
            "imagen" => "gr5.jpeg"
            "Alto" => 701
            "Ancho" => 935
            "Tamanyo" => 216804
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        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0085" class="elsevierStyleSimplePara elsevierViewall">Section of the only biopsy in which doubtful CD56 positivity was detected in some cells &#40;CD56&#44; original magnification &#215;30&#41;&#46;</p>"
        ]
      ]
      5 => array:7 [
        "identificador" => "tbl0005"
        "etiqueta" => "Table 1"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "tabla" => array:1 [
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Clinical Manifestations&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Site&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Histologic Pattern&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Form of Infiltrate&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Clinical Course&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Papules &#40;15&#47;18&#41; 83&#37;Crusted papules &#40;3&#47;18&#41; 17&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Extremities &#40;13&#47;18&#41; 72&#37;Trunk &#40;4&#47;18&#41; 22&#37;Extremities and trunk &#40;1&#47;18&#41; 6&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">A &#40;14&#47;18&#41; 83&#37;B &#40;1&#47;18&#41; 6&#37;C &#40;1-18&#41; 6&#37;Mixed &#40;1&#47;18&#41; 6&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Wedge-shaped &#40;14&#47;18&#41; 78&#37;Band-like &#40;2&#47;18&#41; 22&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Outbreak &#40;11&#47;18&#41; 62&#37;Recurrent outbreaks &#40;4&#47;18&#41; 22&#37;Chronic &#40;1&#47;18&#41; 6&#37;Mycosis fungoides &#40;2&#47;18&#41; 12&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab182207.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0090" class="elsevierStyleSimplePara elsevierViewall">Summary of Clinical and Histologic Findings&#46;</p>"
        ]
      ]
      6 => array:7 [
        "identificador" => "tbl0010"
        "etiqueta" => "Table 2"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "tabla" => array:1 [
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">CD30&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " colspan="2" align="center" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">CD3</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">CD56&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Tumor Cell&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Infiltrate Cell&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#43;&#43;&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&#37; &#40;6&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6&#37; &#40;1&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">50&#37; &#40;9&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#43;&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&#37; &#40;6&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&#37; &#40;6&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#43;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">24&#37; &#40;4&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&#37; &#40;6&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&#37; &#40;6&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#43;&#47;&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">12&#37; &#40;2&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6&#37; &#40;1&#47;17&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&#37; &#40;6&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">17&#37; &#40;3&#47;18&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">94&#37; &#40;16&#47;17&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab182205.png"
              ]
            ]
          ]
        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0095" class="elsevierStyleSimplePara elsevierViewall">Immunohistochemistry Findings&#46;</p>"
        ]
      ]
      7 => array:7 [
        "identificador" => "tbl0015"
        "etiqueta" => "Table 3"
        "tipo" => "MULTIMEDIATABLA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "tabla" => array:2 [
          "leyenda" => "<p id="spar0105" class="elsevierStyleSimplePara elsevierViewall">Abbreviation&#58; CALCL&#44; cutaneous anaplastic large-cell lymphoma&#46;</p>"
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">El Shabrawi-Caelen et al<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> &#40;n &#61; 78&#41;</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Bennek et al<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> 2000 &#40;n &#61; 118&#41;</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">B&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">C&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Mixed&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">B&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">C&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Mixed&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">13&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">8&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">79&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">5&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">9&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Wedge-shaped&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">CD30<span class="elsevierStyleSup">&#43;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t\ttop\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t">CD56<span class="elsevierStyleSup">&#43;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">40&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">50&#37;&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">CALCL&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
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                  \t\t\t\t">10 &#40;9&#37;&#41;</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Hodgkin lymphoma&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">0&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t">14&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
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                  \t\t\t\t">2 &#40;1&#46;7&#37;&#41;</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Mycosis fungoides&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
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                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">9&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
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                  \t\t\t\t">11 &#40;9&#46;3&#37;&#41;</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Papules&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
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                  \t\t\t\t">68&#37;</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Treatment&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="char" valign="\n
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                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">None&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " colspan="4" align="center" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">56&#37;</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Psoralen&#8211;UV-A&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
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Vol. 103. Núm. 5.
Páginas 388-393 (junio 2012)
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Vol. 103. Núm. 5.
Páginas 388-393 (junio 2012)
Original Article
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Lymphomatoid Papulosis: Clinical and Pathological Findings in 18 Patients
Papulosis linfomatoide: hallazgos clínico-patológicos en 18 pacientes
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14882
M. Fernández-Guarinoa,
Autor para correspondencia
montsefdez@msn.com

Corresponding author.
, R. Carrillo-Gijónb, P. Jaén-Olasoloa
a Servicio de Dermatología, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain
b Servicio de Anatomía Patológica, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain
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Table 1. Summary of Clinical and Histologic Findings.
Table 2. Immunohistochemistry Findings.
Table 3. Summary of the Findings From the 2 Case Series of Patients With Lymphomatoid Papulosis Published Since 2000.
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Abstract
Background

Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative skin disease that has been described in association with Hodgkin lymphoma. It has also been reported to progress to mycosis fungoides or cutaneous anaplastic large-cell lymphoma.

Objective

To study the clinical and histologic features of LyP and response to treatment in a patient series.

Materials and methods

For this retrospective, descriptive, observational study of patients with histologically confirmed LyP and sufficient follow-up data on record, we extracted histologic findings on skin biopsy, clinical presentation, clinical course, and response to treatments.

Results

Eighteen patients (10 male, 8 female) were identified. Most biopsies (14/18, 78%) showed a wedge-shaped lymphocytic infiltrate with CD30+, CD3+, and CD56 cells. A type A histologic pattern was present in the biopsies of 83% of the patients. The most common presentation (83%) consisted of papules on the trunk; for 62% LyP resolved after a single episode. Twelve percent of the patients developed mycosis fungoides (mean follow-up, 7 years); no other associations were noted.

Discussion

Although few series of patients with LyP have been published in recent years, the findings reported generally coincide with our observations.

Conclusion

LyP is typically a CD30+ lymphoproliferative disorder that usually runs a benign course and responds well to treatment.

Keywords:
Lymphomatoid papulosis
Findings histologic
Signs and symptoms
Resumen
Introducción

La papulosis linfomatoide (PL) es una dermatosis que se engloba dentro de los procesos linfoproliferativos CD30 positivos de la piel. Se ha descrito su asociación a linfoma de Hogking (LH), así como su progresión a micosis fungoide (MF) y linfoma cutáneo anaplásico de célula grande (LCACG).

Objetivos

Investigar los hallazgos clínicos, histológicos y la respuesta al tratamiento en un grupo de pacientes con PL.

Material y métodos

Se llevó a cabo un estudio retrospectivo, descriptivo y observacional. Se seleccionaron 18 pacientes con diagnóstico histológico confirmado de PL y con un adecuado seguimiento clínico. Se recopilaron los hallazgos histológicos de las biopsias de piel, la forma de presentación, la evolución y la respuesta a los tratamientos utilizados.

Resultados

Se reclutaron un total de 18 pacientes, 10 varones y 8 mujeres. La mayoría de las biopsias, 14 de 18 (78%) mostraban un infiltrado linfocitario en cuña, CD30 positivo, CD3 positivo y CD56 negativo. El tipo histológico más frecuente fue el tipo A, presente en un 83% de las biopsias de los pacientes. La forma clínica de presentación más frecuente fue en forma de pápulas en el tronco (83%). Un 62% de los pacientes sufrió un único brote autorresolutivo. La media de seguimiento fue de 7 años, durante los cuales un 12% de los pacientes desarrolló una micosis fungoide, sin encontrarse otras asociaciones.

Discusión

Existen pocas series de pacientes con PL publicadas en los últimos años; sin embargo, globalmente los hallazgos descritos en ellas coinciden con las de nuestro grupo de pacientes.

Conclusiones

La PL es un cuadro linfoproliferativo típicamente CD30 positivo que habitualmente tiene un curso benigno con buena respuesta a los tratamientos utilizados.

Palabras clave:
Papulosis linfomatoide
Hallazgos histológicos
Presentación clínica
Texto completo
Introduction

Lymphomatoid papulosis (LyP) is a lymphoproliferative disorder that affects middle-aged patients in the form of recurrent outbreaks of papules or papulonecrotic lesions. It runs a benign course and usually resolves spontaneously in 4 to 6 weeks. However, as it can progress to other types of cutaneous T-cell lymphoma and is associated with Hodgkin lymphoma, patients should be monitored. Because the course of LyP is self-limiting and benign, treatment is often not prescribed or is restricted to topical corticosteroids until the lesions have resolved. When the lesions are more protracted or more extensive, they can be treated using psoralen–UV-A, low-dose methotrexate, or interferon alfa.1–5

LyP has traditionally been considered a primary cutaneous CD30+ lymphoproliferative disorder and is classified into 3 histologic patterns: type A (histiocytoid), which is characterized by the presence of large atypical lymphocytes accompanied by small lymphocytes, neutrophils, histiocytes, and eosinophils; type B (mycosis fungoides–like), which comprises a monomorphic infiltrate of small to medium-sized lymphocytes with cerebriform nuclei; and type C, which is formed by an infiltrate of large lymphocytes similar to that of type A, but in which the lymphocytes account for more than 50% of the infiltrate, thus mimicking cutaneous anaplastic large-cell lymphoma. Given the histologic overlap between LyP and other cutaneous lymphoproliferative disorders and the potential of this condition to evolve to or be associated with malignancy, the debate over whether it is benign, premalignant, or malignant remains unresolved. However, LyP is now widely accepted as a primary cutaneous lymphoma with a favorable prognosis. Given the absence of molecular or immunohistochemistry prognostic criteria, the correlation between clinical and histologic features and patient follow-up is essential for predicting disease course.1,6

Clonal rearrangement of T-cell receptors is observed in up to 60% of LyP lesions, with the same clone being found in separate lesions7,8; nevertheless, some authors have observed this clonality in small lymphocytes, thus preventing differentiation from reactive conditions.9 The multiple myeloma oncogene 1 marker was recently suggested to be a differentiating feature between LyP and cutaneous anaplastic large-cell lymphoma10; however, this role was rejected in later studies.11

The literature contains few LyP series and even fewer studies that contrast clinical data with histology and immunohistochemistry findings.

Objectives

Our objectives were to investigate histology and immunohistochemistry findings in skin biopsies from a group of patients with LyP and to describe the clinical presentation, course of the disease, association with other conditions, response to treatment, and potential correlations between these variables and histology findings.

Material and Methods

We performed a retrospective, descriptive, observational study of patients with a clinically and histologically confirmed diagnosis of LyP and with sufficient follow-up data on record. We selected patients using lymphomatoid papulosis as a search term in the histopathology database, which covers the period January 2000 to May 2010, and in the dermatology database, which covers the period January 1995 to May 2010. The databases yielded 44 and 24 patients, respectively. After ruling out patients who did not have an accurate diagnosis, a clinical history, or histology samples and those who were lost to follow-up, we identified 18 patients and 26 biopsy specimens.

We evaluated the following clinical variables: age at onset, sex, clinical presentation (type of lesions and site), laboratory results at diagnosis, the course of the lesions, treatments received, and the response to treatment. We also described associations with other lymphomas (both cutaneous and noncutaneous) and the latency period until these associations developed. As for histology findings, we studied type of LyP (A, B, C, or mixed), type of infiltrate (wedge-shaped or band-like), presence of epidermotropism, and positivity for CD30 (activated T-cell and B-cell marker and Hodgkin lymphoma cell marker), CD3 (T-cell marker), and CD56 (natural killer cell marker), as well as the intensity of these markers. We also determined the presence of clonal T-cell receptor rearrangement in patients who underwent testing. Immunohistochemistry markers were classified into 5 degrees of intensity (+++, ++, +, +–, and –) based on the consensus reached by 2 observers. These markers were tested using the following reagents:

  • -

    CD3: rabbit polyclonal antibody (Dako) IR503, Flex

  • -

    CD30: mouse polyclonal antibody (Dako) IR602, Flex

  • -

    CD56: synthetic monoclonal antibody diluted 1/25 (Master Diagnostics) Clone IB6

Results

The results are summarized in Tables 1 and 2. The study sample comprised 18 patients (10 men and 8 women) with a mean age of 42.7 years (range, 7-70 years). Onset was mainly in the form of papules (15 patients [83%]) and crusted papules (3 patients [17%]) (Fig. 1). The most frequent locations were the extremities (13 patients [72%]), trunk (4 patients [22%]), and both (1 patient [6%]).

Table 1.

Summary of Clinical and Histologic Findings.

Clinical Manifestations  Site  Histologic Pattern  Form of Infiltrate  Clinical Course 
Papules (15/18) 83%Crusted papules (3/18) 17%  Extremities (13/18) 72%Trunk (4/18) 22%Extremities and trunk (1/18) 6%  A (14/18) 83%B (1/18) 6%C (1-18) 6%Mixed (1/18) 6%  Wedge-shaped (14/18) 78%Band-like (2/18) 22%  Outbreak (11/18) 62%Recurrent outbreaks (4/18) 22%Chronic (1/18) 6%Mycosis fungoides (2/18) 12% 
Table 2.

Immunohistochemistry Findings.

  CD30  CD3CD56 
    Tumor Cell  Infiltrate Cell   
+++  33% (6/18)  6% (1/18)  50% (9/18)   
++  33% (6/18)  33% (6/18)  –   
24% (4/18)  33% (6/18)  33% (6/18)   
+/–  12% (2/18)  –  –  6% (1/17) 
–  –  33% (6/18)  17% (3/18)  94% (16/17) 
Figure 1.

Typical image of a lymphomatoid papulosis lesion. Papule with a central desquamative scab.

(0.1MB).

The results of a complete blood count and biochemistry (including lactate dehydrogenase) at diagnosis were normal for all patients, as were the results for β2-microglobulin in the 5 patients who were tested for it. Mean follow-up after diagnosis was 7 years (range, 2-22 years). The majority of patients (11 patients, 62%) had a single outbreak that resolved without treatment or with topical treatment, 4 patients (22%) experienced recurrent outbreaks, 2 patients (12%) developed associated mycosis fungoides or had both types of cutaneous T-cell lymphoma as their presenting complaint, and 1 patient continued to be affected by the chronic form, with no response to treatment. Patients who experienced recurrent outbreaks over the years responded well to psoralen–UV-A during each outbreak. In the patient with chronic disease and the patient with mycosis fungoides presenting simultaneously with LyP, psoralen–UV-A combined with methotrexate partially controlled the lesions.

As biopsy had been repeated in some cases during follow-up, 26 specimens were available for the 18 patients. Wedge-shaped infiltrate was more common than band-like infiltrate (78% vs 22%). The most common histologic pattern was type A (15 patients [83%]) (Fig. 2); types B, C, and mixed AB were found in 1 patient each. All the samples were positive for CD30; in most cases this positivity was moderate or intense (Fig. 3). Positivity for CD3 was more intense in the cells of the infiltrate than in the tumor cells (Fig. 4), which were also positive for CD3, although less intensely. Interestingly, among the cells accompanying the tumor cells, one group of cells was positive for CD3 while the other was not, as if the cell populations were different (even though both had similar morphology). None of the cells were positive for CD56, although in 1 case this finding was doubtful (Fig. 5). Epidermotropism was only found in type B and mixed AB. T-cell receptor rearrangement was not detected in the 9 patients who underwent testing. Curiously, findings in repeat biopsy specimens did not vary (3 patients and 7 specimens).

Figure 2.

Typical image of type A lymphomatoid papulosis. Note the characteristic large-cell, wedge-shaped infiltrate (hematoxylin-eosin, original magnification ×10).

(0.26MB).
Figure 3.

Intense CD30 positivity in type A lymphomatoid papulosis (CD30, original magnification ×30).

(0.18MB).
Figure 4.

CD3 positivity in the large cells of type A lymphomatoid papulosis (CD30, original magnification ×30).

(0.21MB).
Figure 5.

Section of the only biopsy in which doubtful CD56 positivity was detected in some cells (CD56, original magnification ×30).

(0.21MB).

Table 1 summarizes the clinical characteristics of the patients and histologic patterns of LyP; no apparent correlation was found between the clinical variables studied and the histologic type. The small sample size prevented us from applying statistical tests with sufficient power to be able to analyze the variables described.

Discussion

The epidemiologic and clinical findings for our group of patients are consistent with those published in the literature,2,3 that is, LyP is a lymphoproliferative disease that is slightly more common in men and has a mean age at onset of 40 to 45 years. In most cases, onset takes the form of papules or crusted papules on the limbs; however, several forms have been reported (eg, vesicular, agminated, and ulcerative-necrotic).1

A review of the literature from the last 10 years revealed only 2 series that analyze clinical and histologic findings and their association with other diseases in patients with LyP (Table 3)2,3. In addition, the Spanish medical literature includes a recently published series comprising 9 pediatric patients with LyP.4 Data on the histology of the lesions reported by El Shabrawi-Caelen et al3 in 2004 are consistent with our results, namely, a clear predominance of type A (75% of patients). Types B and C were less common, with a frequency of 4% and 13%, respectively. Again, these findings were consistent with those of our series, in which each type affected 6% of patients. Most infiltrates were wedge-shaped, except for those of patients with type B disease, which, given their resemblance to the infiltrates occurring in mycosis fungoides, tended to be band-like. Similarly, in 2000, Bennek et al2 reported on a series of 118 patients, most of whom were type A (79%). The frequency of the other types was 5% for type B, 7% for type C, and 9% for the mixed type. The authors provided no immunohistochemistry findings.

Table 3.

Summary of the Findings From the 2 Case Series of Patients With Lymphomatoid Papulosis Published Since 2000.

  El Shabrawi-Caelen et al3 (n = 78)Bennek et al2 2000 (n = 118)
  Mixed  Mixed 
75%  4%  13%  8%  79%  5%  7%  9% 
Wedge-shaped  90%  50%  100%  –  –  –  –  – 
CD30+  100%  75%  100%  –  –  –  –  – 
CD56+  40%  50%  50%  –  –  –  –  – 
CALCL  0%  0%  0%  0%  10 (9%)
Hodgkin lymphoma  3%  0%  9%  14%  2 (1.7%)
Mycosis fungoides  3%  0%  9%  9%  11 (9.3%)
Papules  –  –  –  –  68%
Treatment  –  –  –  –         
None          56%
Psoralen–UV-A          35%

Abbreviation: CALCL, cutaneous anaplastic large-cell lymphoma.

It is important to note that it is not possible to differentiate LyP from other CD30+ lymphoproliferative disorders on immunohistochemistry findings. Although positivity demonstrates the presence of the disease, some cases are negative. The reasons why LyP is CD30 are not clear and have been reported mainly for type B disease.12 One possible explanation is that manifestations assumed to be compatible with LyP were really those subsequently reported for papular mycosis fungoides and, therefore, indicative of a CD30 disorder.13

CD3 is a pan–T-cell marker and, as such, positive in almost all cases of LyP. In our series, we found that it was more intensely positive in cells of the infiltrate accompanying large cells, thus suggesting the existence of 2 T-cell populations. However, as this finding was not described in the literature reviewed, further studies are warranted to confirm it.

We analyzed CD56, because 50% of the LyP biopsy specimens in the series by El Shabrawi-Caelen et al3 were positive for this marker. In our study, however, we found only 1 case of doubtful CD56 positivity. The literature review showed that positivity for CD56 in LyP remains open to debate and generally tends to be described as very rare.14 The reasons for such a discordant finding between 2 series published in the last 6 years remain unclear, although one explanation could be the different dilutions of reagent used: 1:20 in the study by El Shabrawi-Caelen et al in 2004, 1:40 in 3 cases of CD56+ LyP in the study by Flann et al14, and 1:25 in our series.

T-cell receptor testing in patients with LyP is useful for differentiating between lesions involving clonal rearrangement and those of a reactive nature. However, positivity or negativity of the T-cell receptor is merely orientative in clinical practice. Moreover, the role of clonal rearrangement in LyP remains unclear: in 2003, Gellrich et al9 reported clonality in small CD30 cells in the accompanying infiltrate, whereas in 2002, Steinhoff et al7 reported clonality in large CD30+ cells.

Classification of LyP into 3 histologic patterns, both in our study and in the literature reviewed,2,3 is more a convention than of relevance to symptoms, prognosis, and response to treatment. No apparent association exists between the histologic pattern and the variables studied.

Symptoms are self-limiting in most cases (approximately 60% to 70% according to the literature). However, the risk of progression from LyP to mycosis fungoides has been reported in 10% to 12% of cases, an association with Hodgkin lymphoma in 9% of cases, and an association with cutaneous anaplastic large-cell lymphoma in 9% of cases (Table 3). We detected progression to mycosis fungoides in only 12% of our patients; neither of the other associations was observed. A diagnosis of LyP after a diagnosis of mycosis fungoides must be made with caution, since it may reveal a transformation to large cell disease, thus necessitating a radical change in the treatment and prognosis of the initial mycosis fungoides. Knowledge of these associations in patients with LyP requires us to perform periodic follow-up and 6-monthly monitoring with laboratory tests (including lactate dehydrogenase) and chest x-ray.5

Most cases of LyP present with papular lesions, are self-limiting, and do not require treatment. More persistent outbreaks usually respond well to psoralen–UV-A, as was the case in 35% of the patients in the series by Bennek et al2 in 2000 and in 22% of our patients. Low-dose methotrexate is an effective alternative. In the very rare refractory cases, bexarotene,15 interferon alfa,16 topical 5-fluorouracil,17 and photodynamic therapy18 have been tried, with varying success.

We conclude that LyP affects middle-aged patients in the form of outbreaks of papules on the extremities and trunk. Most cases resolve spontaneously, and the response to treatment is good. The typical histologic finding is a wedge-shaped infiltrate of large CD30+ cells and, rarely, CD56+ cells. Follow-up is necessary, as the disease has been reported to be associated with other lymphoproliferative disorders.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

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Please cite this article: Fernández-Guarino M, et al. Papulosis linfomatoide: hallazgos clínico-patológicos en 18 pacientes. Actas Dermosifiliogr.2012;103:388-93.

Copyright © 2011. Elsevier España, S.L. and AEDV
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