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and viruses such as hepatitis C &#40;splenic MZL&#41;&#46; Although the clinical behavior of these different types of lymphoma may differ&#44; they share similar morphologic features and have a similar immunophenotype&#58; CD19&#43;&#44; CD20&#43;&#44; CD79a&#43;&#44; CD5-&#44; CD10-&#44; CD23-&#44; bcl6-&#44; and bcl2&#43;&#46; More than 70&#37; of MZL&#44; including the primary cutaneous forms&#44; are of the MALT type&#44; whereas splenic and nodal forms occur less frequently and account for only 1&#37; of non-Hodgkin lymphomas&#46; With MALT lymphoma and nodal MZL&#44; there have been reports of cutaneous involvement&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> but this is rare in the case of splenic MZL&#46; We present a case of splenic MZL with epidermotropic skin involvement&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case Description</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 68-year-old man with a history of hypertension and benign prostatic hyperplasia in treatment with doxazosin presented with erythematous-violaceous infiltrated plaques and nodules of 1-year duration&#46; 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and myeloperoxidase &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a> and <a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; With a provisional diagnosis of MZL&#44; a staging study was performed&#46; The same monoclonal rearrangement of the heavy chain Ig gene was detected in the bone marrow biopsy and peripheral blood analysis&#46; A full biochemical analysis&#44; protein analysis&#44; and serology for <span class="elsevierStyleItalic">Borrelia burgdorferi</span> and for hepatitis virus B and C showed an increase in &#946;<span class="elsevierStyleInf">2</span>-microglobulin &#40;4&#46;02<span class="elsevierStyleHsp" style=""></span>mg&#47;L&#41; and past hepatitis B infection &#40;negative for hepatitis B surface antigen &#91;HBsAg&#93; and positive for anti-hepatitis B and anti-hepatitis B surface antibodies&#41;&#46; The remaining parameters were normal or negative&#46; Only mild superficial gastritis was detected in gastric endoscopy&#44; and no <span class="elsevierStyleItalic">Helicobacter pylori</span> were isolated from the biopsy sample&#46; The thoracoabdominal computed tomography showed massive splenomegalia&#46; With a possible diagnosis of splenic MZL&#44; splenectomy was performed&#46; Almost all the lesions disappeared and bicytopenia resolved after 1 month &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; Histologic study of the spleen showed a monomorphic micronodular proliferation with a immunophenotype similar to that of the cutaneous lesion except that IgD was negative&#46; Study by fluorescence in situ hybridization for <span class="elsevierStyleItalic">MALT1</span> translocation was negative and PCR of IgH showed monoclonality&#46; These findings confirmed the diagnosis of splenic MZL&#46; Four months later new lesions appeared and the patient received 6 cycles of chemotherapy in combination with rituximab &#40;R-CHOP &#91;rituximab&#44; cyclophosphamide&#44; doxorubicin&#44; vincristine&#44; and prednisone&#93;&#41; every 3 weeks&#46; The lesions disappeared after this treatment&#46; However&#44; 2 months later&#44; hepatitis developed due to reactivation of the hepatitis B virus and the patient died despite receiving treatment with lamivudine&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0015" class="elsevierStylePara elsevierViewall">Approximately 25&#37; of primary cutaneous lymphomas are B-cell lymphomas&#44; but in light of the lack of agreement on the classification of cutaneous B-cell lymphomas&#44; the true incidence of MZL is unknown&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> According to some authors&#44; MZL is the most frequent type of cutaneous B-cell lymphomas whereas others consider it to be relatively uncommon&#46; Given the differences in behavior and prognosis of different types of MZL&#44; it is important to determine when skin involvement in MZL is primary or secondary&#46; By definition&#44; a lymphoma is considered as a primary cutaneous malignancy if&#44; on diagnosis or during the staging study&#44; no other organ is found to be involved&#46; In our patient&#44; multiorgan involvement &#40;skin&#44; peripheral blood&#44; bone marrow&#44; and spleen&#41; of the MZL was demonstrated&#46; These characteristics are consistent with splenic MZL&#44; which has an indolent course and presents with splenomegalia &#40;almost 100&#37; of the time&#41;&#44; along with bone marrow involvement &#40;95&#37;&#41; and peripheral blood involvement &#40;75&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> At times&#44; autoimmune diseases may be associated with splenic MZL and can be the chief complaint&#46; They were not&#44; however&#44; reported in this patient at any time during the course of the disease&#46; In our case&#44; cells from all involved areas had the same morphology and the same phenotype&#44; suggesting that they originated from the same clone&#46; In addition&#44; the clinical course&#44; in which the skin lesions all but disappeared and blood counts returned to normal after splenectomy&#44; is evidence in favor of diagnosis of splenic lymphoma with secondary skin involvement&#46; Skin involvement in MZL is exceptional and has only be reported occasionally&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;7</span></a> Chiang et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> reported a patient with multiple papules on the trunk and thighs&#46; Histology revealed MZL mimicking mycosis fungoides&#46; The staging study in that case showed bone marrow involvement and splenomegalia&#44; although histopathology of the spleen was not performed&#46; The patient responded well to narrow-band UV-B phototherapy&#46; In another study of a series of 85 cases of splenic MZL&#44; Mollejo et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> singled out 4 cases with a diffuse pattern of infiltration&#46; These lesions were negative for IgD and 2 of them were associated with epidermotropic skin involvement&#46; The authors considered these lymphomas to be a variant of splenic MZL&#46; They did not report the extent of skin involvement&#44; so we do not know whether it was as severe as in our patient&#46; Nevertheless&#44; there are certain similarities with our case&#44; such as intense involvement of the red pulp of the spleen&#44; negative IgD status &#40;splenic MZL usually expresses IgD&#41;&#44; and epidermotropic skin involvement&#46; Such epidermotropism is somewhat unusual and not reported in large series of primary cutaneous MZL&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> except for a study by Bathelier et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> who reported epidermotropism in 8&#37; of cases&#46; Of the 49 cases of MZL included in that series&#44; 29 were primary cutaneous forms and 20 had systemic involvement though none had involvement of the spleen&#46; The clinical features are not described&#44; and it is not specified whether the cases of epidermotropism corresponded to primary or secondary MZL&#46; In addition to this study and those of Chiang et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and Mollejo et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> &#40;which refer to secondary skin involvement in splenic MZL&#41;&#44; we could only find 1 other report of primary cutaneous MZL with epidermotropism&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">As is the case with cutaneous MZL&#44; splenic MZL is usually indolent and a watchful waiting approach can be adopted&#46; When treatment is indicated&#44; splenectomy has usually been considered&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> However&#44; new biologics such as rituximab &#40;anti-CD20 monoclonal antibody&#41; may also be administered although there is still some debate about its usefulness&#46; Some authors are in favor of treatment with this drug&#44; with or without associated chemotherapy&#44; depending on the status of the patient&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> The use of rituximab is&#44; however&#44; not without its risks&#46; One of these is reactivation of hepatitis B&#44; which can be fatal&#44; as was the case in our patient&#46; This complication may occur during or after treatment&#46; Cases have been reported up to 12 months after discontinuation&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> The first patient with this complication was reported in 1999&#44; and new cases were subsequently reported&#44; almost always when rituximab was administered in combination with chemotherapy although there have also been reports after monotherapy&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">14&#44;15</span></a> This makes it necessary to screen for hepatotoxic viruses&#44; not only when patients are treated with rituximab but also in the case of treatment with other biologics widely used at present in dermatology such as anti-tumor necrosis factor agents&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> In patients with prior hepatitis B infection &#40;HBsAg-&#41;&#44; viral DNA should be monitored to rule out reactivation&#46; Prophylaxis should be given to patients positive for HBsAg as it has been found that no patients who received prophylactic treatment have died even in the presence of hepatitis B reactivation &#40;compared to historical controls&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> Nevertheless&#44; there is no consensus regarding the prophylactic agent of choice &#40;lamivudine&#44; entecavir&#44; or tenofovir&#41; or the duration of treatment&#44; that is&#44; whether it should be longer because of the possibility of late reactivation or shorter because of the development of resistance with prolonged treatment&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In summary&#44; we present a rare case of MZL with skin involvement secondary to splenic MZL&#46; Although histology does not generally allow differentiation between primary MZL and secondary MZL&#44; we highlight that epidermotropism is common when cutaneous infiltration occurs in splenic MZL occurs but rare in primary cutaneous MZL&#46; Finally&#44; we would also like to emphasize the need to screen for the hepatitis virus before using immunosuppressive drugs&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare no conflicts of interest&#46;</p></span></span>"
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            0 => "Marginal zone lymphoma"
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            0 => "Linfoma B de la zona marginal"
            1 => "Linfoma cut&#225;neo de la zona marginal"
            2 => "Linfoma espl&#233;nico"
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Marginal zone B-cell lymphoma &#40;MZL&#41; is subclassified into extranodal MZL of mucosa-associated lymphoid tissue &#40;including cutaneous lymphomas&#41;&#44; splenic MZL&#44; and nodal MZL&#46; We report the case of a 68-year-old man with erythematous-violaceous plaques and nodules&#46; Skin biopsy showed an epidermotropic lymphocytic infiltration and cytology and immunohistochemistry were consistent with MZL&#46; The workup revealed disease in the peripheral blood and bone marrow and massive splenomegaly&#46; Splenectomy confirmed the diagnosis of splenic MZL and led to resolution of the skin lesions&#46; Cutaneous recurrence was treated successfully with chemotherapy and rituximab but caused fatal hepatitis due to hepatitis B virus reactivation&#46; Skin involvement by splenic MZL is uncommon&#59; this form of the disease can present epidermotropism&#44; a very rare finding in primary cutaneous MZL&#46; Treatment consists of splenectomy&#44; which may be associated with chemotherapy and&#47;or rituximab&#59; this treatment may lead to reactivation of latent hepatitis B infection and screening for hepatitis should therefore be performed prior to starting therapy&#46;</p>"
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El linfoma B de la zona marginal &#40;LZM&#41; se subdivide en LZM extraganglionar tipo MALT &#40;incluye los cut&#225;neos&#41;&#44; espl&#233;nico y ganglionar&#46; Presentamos el caso de un var&#243;n de 68 a&#241;os con placas y n&#243;dulos eritematoviol&#225;ceos&#46; La biopsia cut&#225;nea mostr&#243; una infiltraci&#243;n linfocitaria epidermotropa con citolog&#237;a e inmunohistoqu&#237;mica compatibles con LZM&#46; El estudio de extensi&#243;n mostr&#243; afectaci&#243;n de sangre perif&#233;rica&#44; m&#233;dula &#243;sea y esplenomegalia masiva&#46; La esplenectom&#237;a confirm&#243; el diagn&#243;stico de LZM espl&#233;nico con desaparici&#243;n de las lesiones cut&#225;neas&#46; Tras la recidiva cut&#225;nea se instaur&#243; quimioterapia&#47;rituximab con desaparici&#243;n de la misma&#44; pero produci&#233;ndose una hepatitis letal por reactivaci&#243;n de virus de hepatitis B&#46; La afectaci&#243;n cut&#225;nea por un LZM espl&#233;nico es un hallazgo infrecuente&#46; Dicha afectaci&#243;n puede presentar epidermotropismo&#44; hallazgo excepcional en los LZM cut&#225;neos primarios&#46; El tratamiento consiste en esplenectom&#237;a y&#47;o quimioterapia y&#47;o rituximab que pueden reactivar una hepatitis B inactiva&#44; por lo que es preciso el <span class="elsevierStyleItalic">screening</span> de hepatitis antes de iniciarlos&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; G&#243;mez-de la Fuente E&#44; et al&#46; Linforma B espl&#233;nico de la zona marginal con afectaci&#243;n cut&#225;nea epidermotropa&#46; Actas Dermosifiliogr&#46;2012&#59;103&#58;427-31&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Infiltrated plaques and nodules&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A&#44; Cutaneous biopsy with epidermotropic tumoral infiltration of the entire dermis&#46; Hematoxylin-eosin&#44; original magnification x40&#46; B&#44; Detail of the same slide&#44; original magnification x200&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A&#44; Immunostaining with CD20&#46; B&#44; Immunostaining with bcl-20&#46; Original magnification&#44; x20 in both cases&#46; Involvement of the entire epidermis and epidermotropism can be observed&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Almost complete disappearance of lesions 1 month after splenectomy&#46;</p>"
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      "titulo" => "References"
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Case Report
Splenic Marginal Zone B-Cell Lymphoma with Epidermotropic Skin Involvement
Linfoma B esplénico de la zona marginal con afectación cutánea epidermotropa
E. Gómez-de la Fuentea,
Autor para correspondencia
egomezf@fhalcorcon.es

Corresponding author.
, L.B. Villalónb, L. Calzado-Villarreala, F. Pinedo-Moraledac, J.L. López-Estebaranza
a Servicios de Dermatología, Hospital Universitario Fundación Alcorcón, Madrid, Spain
b Servicios de Hematología, Hospital Universitario Fundación Alcorcón, Madrid, Spain
c Servicios de Anatomía Patológica, Hospital Universitario Fundación Alcorcón, Madrid, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Marginal-zone B-cell lymphoma &#40;MZL&#41; is a neoplasm that originates in lymphocytes of the marginal zone&#44; which is a compartment of lymphoid follicles that is highly developed in lymphoid organs with a high influx of antigens&#46; The World Health Organization classification subdivides MZL into mucosa-associated lymphoid tissue &#40;MALT&#41; lymphoma and splenic or nodal lymphoma&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The etiopathogenic process has been linked to chronic antigenic stimulus by self-antigens or bacteria such as <span class="elsevierStyleItalic">Borrelia burgdorferi</span> &#40;cutaneous MZL&#41; and <span class="elsevierStyleItalic">Helicobacter pylori</span> &#40;gastric MZL&#41; and viruses such as hepatitis C &#40;splenic MZL&#41;&#46; Although the clinical behavior of these different types of lymphoma may differ&#44; they share similar morphologic features and have a similar immunophenotype&#58; CD19&#43;&#44; CD20&#43;&#44; CD79a&#43;&#44; CD5-&#44; CD10-&#44; CD23-&#44; bcl6-&#44; and bcl2&#43;&#46; More than 70&#37; of MZL&#44; including the primary cutaneous forms&#44; are of the MALT type&#44; whereas splenic and nodal forms occur less frequently and account for only 1&#37; of non-Hodgkin lymphomas&#46; With MALT lymphoma and nodal MZL&#44; there have been reports of cutaneous involvement&#44;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> but this is rare in the case of splenic MZL&#46; We present a case of splenic MZL with epidermotropic skin involvement&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case Description</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 68-year-old man with a history of hypertension and benign prostatic hyperplasia in treatment with doxazosin presented with erythematous-violaceous infiltrated plaques and nodules of 1-year duration&#46; The lesions first appeared on the abdomen and then spread to the trunk and the roots of the limbs &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; He had no fever or other symptoms&#44; except noninflammatory arthralgia&#46; Lymph nodes were not palpable in the physical examination&#46; His blood counts showed cytopenia &#40;leukocytes 2000&#44; neutrophils 700&#44; and platelets 88&#160;000&#41;&#46; With a suspected diagnosis of a lymphoproliferative process&#44; a skin biopsy was taken&#46; This showed epidermotropic infiltration of the papillary and deep dermis&#46; Intermediate-size lymphocytes with a centroid or monocytoid appearance were present in perivascular and interstitial areas between collagen bundles and in the basal layer of the epidermis&#46; Immunohistochemical staining was positive for CD20&#44; CD79a&#44; bcl2&#44; and immunoglobulin &#40;Ig&#41; D&#44; and negative for CD3&#44; CD5&#44; CD10&#44; bcl6&#44; CD23&#44; cyclin D1&#44; multiple myeloma oncogene 1&#44; and myeloperoxidase &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a> and <a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; With a provisional diagnosis of MZL&#44; a staging study was performed&#46; The same monoclonal rearrangement of the heavy chain Ig gene was detected in the bone marrow biopsy and peripheral blood analysis&#46; A full biochemical analysis&#44; protein analysis&#44; and serology for <span class="elsevierStyleItalic">Borrelia burgdorferi</span> and for hepatitis virus B and C showed an increase in &#946;<span class="elsevierStyleInf">2</span>-microglobulin &#40;4&#46;02<span class="elsevierStyleHsp" style=""></span>mg&#47;L&#41; and past hepatitis B infection &#40;negative for hepatitis B surface antigen &#91;HBsAg&#93; and positive for anti-hepatitis B and anti-hepatitis B surface antibodies&#41;&#46; The remaining parameters were normal or negative&#46; Only mild superficial gastritis was detected in gastric endoscopy&#44; and no <span class="elsevierStyleItalic">Helicobacter pylori</span> were isolated from the biopsy sample&#46; The thoracoabdominal computed tomography showed massive splenomegalia&#46; With a possible diagnosis of splenic MZL&#44; splenectomy was performed&#46; Almost all the lesions disappeared and bicytopenia resolved after 1 month &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; Histologic study of the spleen showed a monomorphic micronodular proliferation with a immunophenotype similar to that of the cutaneous lesion except that IgD was negative&#46; Study by fluorescence in situ hybridization for <span class="elsevierStyleItalic">MALT1</span> translocation was negative and PCR of IgH showed monoclonality&#46; These findings confirmed the diagnosis of splenic MZL&#46; Four months later new lesions appeared and the patient received 6 cycles of chemotherapy in combination with rituximab &#40;R-CHOP &#91;rituximab&#44; cyclophosphamide&#44; doxorubicin&#44; vincristine&#44; and prednisone&#93;&#41; every 3 weeks&#46; The lesions disappeared after this treatment&#46; However&#44; 2 months later&#44; hepatitis developed due to reactivation of the hepatitis B virus and the patient died despite receiving treatment with lamivudine&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0015" class="elsevierStylePara elsevierViewall">Approximately 25&#37; of primary cutaneous lymphomas are B-cell lymphomas&#44; but in light of the lack of agreement on the classification of cutaneous B-cell lymphomas&#44; the true incidence of MZL is unknown&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> According to some authors&#44; MZL is the most frequent type of cutaneous B-cell lymphomas whereas others consider it to be relatively uncommon&#46; Given the differences in behavior and prognosis of different types of MZL&#44; it is important to determine when skin involvement in MZL is primary or secondary&#46; By definition&#44; a lymphoma is considered as a primary cutaneous malignancy if&#44; on diagnosis or during the staging study&#44; no other organ is found to be involved&#46; In our patient&#44; multiorgan involvement &#40;skin&#44; peripheral blood&#44; bone marrow&#44; and spleen&#41; of the MZL was demonstrated&#46; These characteristics are consistent with splenic MZL&#44; which has an indolent course and presents with splenomegalia &#40;almost 100&#37; of the time&#41;&#44; along with bone marrow involvement &#40;95&#37;&#41; and peripheral blood involvement &#40;75&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> At times&#44; autoimmune diseases may be associated with splenic MZL and can be the chief complaint&#46; They were not&#44; however&#44; reported in this patient at any time during the course of the disease&#46; In our case&#44; cells from all involved areas had the same morphology and the same phenotype&#44; suggesting that they originated from the same clone&#46; In addition&#44; the clinical course&#44; in which the skin lesions all but disappeared and blood counts returned to normal after splenectomy&#44; is evidence in favor of diagnosis of splenic lymphoma with secondary skin involvement&#46; Skin involvement in MZL is exceptional and has only be reported occasionally&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;7</span></a> Chiang et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> reported a patient with multiple papules on the trunk and thighs&#46; Histology revealed MZL mimicking mycosis fungoides&#46; The staging study in that case showed bone marrow involvement and splenomegalia&#44; although histopathology of the spleen was not performed&#46; The patient responded well to narrow-band UV-B phototherapy&#46; In another study of a series of 85 cases of splenic MZL&#44; Mollejo et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> singled out 4 cases with a diffuse pattern of infiltration&#46; These lesions were negative for IgD and 2 of them were associated with epidermotropic skin involvement&#46; The authors considered these lymphomas to be a variant of splenic MZL&#46; They did not report the extent of skin involvement&#44; so we do not know whether it was as severe as in our patient&#46; Nevertheless&#44; there are certain similarities with our case&#44; such as intense involvement of the red pulp of the spleen&#44; negative IgD status &#40;splenic MZL usually expresses IgD&#41;&#44; and epidermotropic skin involvement&#46; Such epidermotropism is somewhat unusual and not reported in large series of primary cutaneous MZL&#44;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;9</span></a> except for a study by Bathelier et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> who reported epidermotropism in 8&#37; of cases&#46; Of the 49 cases of MZL included in that series&#44; 29 were primary cutaneous forms and 20 had systemic involvement though none had involvement of the spleen&#46; The clinical features are not described&#44; and it is not specified whether the cases of epidermotropism corresponded to primary or secondary MZL&#46; In addition to this study and those of Chiang et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> and Mollejo et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> &#40;which refer to secondary skin involvement in splenic MZL&#41;&#44; we could only find 1 other report of primary cutaneous MZL with epidermotropism&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">As is the case with cutaneous MZL&#44; splenic MZL is usually indolent and a watchful waiting approach can be adopted&#46; When treatment is indicated&#44; splenectomy has usually been considered&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> However&#44; new biologics such as rituximab &#40;anti-CD20 monoclonal antibody&#41; may also be administered although there is still some debate about its usefulness&#46; Some authors are in favor of treatment with this drug&#44; with or without associated chemotherapy&#44; depending on the status of the patient&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> The use of rituximab is&#44; however&#44; not without its risks&#46; One of these is reactivation of hepatitis B&#44; which can be fatal&#44; as was the case in our patient&#46; This complication may occur during or after treatment&#46; Cases have been reported up to 12 months after discontinuation&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> The first patient with this complication was reported in 1999&#44; and new cases were subsequently reported&#44; almost always when rituximab was administered in combination with chemotherapy although there have also been reports after monotherapy&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">14&#44;15</span></a> This makes it necessary to screen for hepatotoxic viruses&#44; not only when patients are treated with rituximab but also in the case of treatment with other biologics widely used at present in dermatology such as anti-tumor necrosis factor agents&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> In patients with prior hepatitis B infection &#40;HBsAg-&#41;&#44; viral DNA should be monitored to rule out reactivation&#46; Prophylaxis should be given to patients positive for HBsAg as it has been found that no patients who received prophylactic treatment have died even in the presence of hepatitis B reactivation &#40;compared to historical controls&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> Nevertheless&#44; there is no consensus regarding the prophylactic agent of choice &#40;lamivudine&#44; entecavir&#44; or tenofovir&#41; or the duration of treatment&#44; that is&#44; whether it should be longer because of the possibility of late reactivation or shorter because of the development of resistance with prolonged treatment&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">In summary&#44; we present a rare case of MZL with skin involvement secondary to splenic MZL&#46; Although histology does not generally allow differentiation between primary MZL and secondary MZL&#44; we highlight that epidermotropism is common when cutaneous infiltration occurs in splenic MZL occurs but rare in primary cutaneous MZL&#46; Finally&#44; we would also like to emphasize the need to screen for the hepatitis virus before using immunosuppressive drugs&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors declare no conflicts of interest&#46;</p></span></span>"
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El linfoma B de la zona marginal &#40;LZM&#41; se subdivide en LZM extraganglionar tipo MALT &#40;incluye los cut&#225;neos&#41;&#44; espl&#233;nico y ganglionar&#46; Presentamos el caso de un var&#243;n de 68 a&#241;os con placas y n&#243;dulos eritematoviol&#225;ceos&#46; La biopsia cut&#225;nea mostr&#243; una infiltraci&#243;n linfocitaria epidermotropa con citolog&#237;a e inmunohistoqu&#237;mica compatibles con LZM&#46; El estudio de extensi&#243;n mostr&#243; afectaci&#243;n de sangre perif&#233;rica&#44; m&#233;dula &#243;sea y esplenomegalia masiva&#46; La esplenectom&#237;a confirm&#243; el diagn&#243;stico de LZM espl&#233;nico con desaparici&#243;n de las lesiones cut&#225;neas&#46; Tras la recidiva cut&#225;nea se instaur&#243; quimioterapia&#47;rituximab con desaparici&#243;n de la misma&#44; pero produci&#233;ndose una hepatitis letal por reactivaci&#243;n de virus de hepatitis B&#46; La afectaci&#243;n cut&#225;nea por un LZM espl&#233;nico es un hallazgo infrecuente&#46; Dicha afectaci&#243;n puede presentar epidermotropismo&#44; hallazgo excepcional en los LZM cut&#225;neos primarios&#46; El tratamiento consiste en esplenectom&#237;a y&#47;o quimioterapia y&#47;o rituximab que pueden reactivar una hepatitis B inactiva&#44; por lo que es preciso el <span class="elsevierStyleItalic">screening</span> de hepatitis antes de iniciarlos&#46;</p>"
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        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; G&#243;mez-de la Fuente E&#44; et al&#46; Linforma B espl&#233;nico de la zona marginal con afectaci&#243;n cut&#225;nea epidermotropa&#46; Actas Dermosifiliogr&#46;2012&#59;103&#58;427-31&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">A&#44; Cutaneous biopsy with epidermotropic tumoral infiltration of the entire dermis&#46; Hematoxylin-eosin&#44; original magnification x40&#46; B&#44; Detail of the same slide&#44; original magnification x200&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">A&#44; Immunostaining with CD20&#46; B&#44; Immunostaining with bcl-20&#46; Original magnification&#44; x20 in both cases&#46; Involvement of the entire epidermis and epidermotropism can be observed&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Almost complete disappearance of lesions 1 month after splenectomy&#46;</p>"
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Información del artículo
ISSN: 15782190
Idioma original: Inglés
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