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These findings confirmed a diagnosis of papular mycosis fungoides&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Laboratory tests disclosed mild hypercholesterolemia&#59; the remaining values&#44; including lactate dehydrogenase and &#223;2-microglobulin were within the reference range&#46; There were no palpable lymph nodes in the groin or axillas&#46; Chest radiograph and abdominal ultrasound findings were normal&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was treated with high-potency local topical corticosteroids &#40;clobetasol cream 0&#46;05&#37;&#41; once daily for 3 weeks&#46; Since his condition improved only slightly&#44; treatment with psoralen-UV-A &#40;PUVA&#41; &#40;18 sessions&#41; was started &#40;oral methoxsalen at 20<span class="elsevierStyleHsp" style=""></span>mg before PUVA&#41;&#46; The cutaneous symptoms disappeared almost completely with this treatment&#46; 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The clinical&#44; histopathological&#44; and immunohistochemical findings in our case are similar to those reported for previously published cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;7</span></a> All those authors<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a> concur that this presentation of mycosis fungoides is not very aggressive but also agree that patients should be followed up&#44; because in 1 case it has been reported to progress to plaque-phase mycosis fungoides &#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Prognosis is favorable in this subtype&#44; except in patients with previous symptoms of mycosis fungoides&#44; in whom it indicates disease progression&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The differential diagnosis can be made with lymphomatoid papulosis&#44; especially type B&#44;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5&#44;8</span></a> which usually manifests with ulcerated and crusted lesions that tend to resolve spontaneously&#46; It can also be made with pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica&#44; both of which usually yield intraepidermal neutrophils and necrotic keratinocytes<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> in the histopathology study&#46; Diagnosis can even be made with follicular mycosis fungoides&#44; which is characterized by infiltrate in the follicular epithelium&#44; a certain degree of syringotropism&#44; or both&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Neri et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> proposed a differential diagnosis with aggressive epidermotropic CD8 cytotoxic T-cell lymphoma&#44; after detecting an infiltrate composed of CD8<span class="elsevierStyleSup">&#43;</span>CD4<span class="elsevierStyleSup">&#8211;</span> cells in the case of papular mycosis fungoides they report&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The main therapeutic option for papular mycosis fungoides is PUVA&#44; which is generally successful&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5</span></a> Other options that have been applied in the initial stages<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> include coadjuvant topical retinoids&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> UV-B radiation&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> and topical corticosteroids in monotherapy&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Given the good response with PUVA in the present case and the evidence provided by previously published cases &#40;improvement in 8 out of 12 patients treated&#41;&#44; we propose PUVA as the most appropriate alternative&#44; although we do recognize that the small population could limit interpretation of our results&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Finally&#44; we would like to highlight the wide clinical variability of mycosis fungoides&#44; an entity that can go unnoticed and present in an unremarkable manner over several months as a papular eruption&#44; which is often asymptomatic&#46;</p></span>"
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Case and research letter
Papular Mycosis Fungoides on the Legs: A Case Report
Micosis fungoide papular en las piernas, a propósito de un caso
A. Santamarina-Albertosa,
Autor para correspondencia
alba.santamarina@gmail.com

Corresponding author.
, R. Muñoz-Martíneza, T. Alvarez-Gagob, A. Miranda-Romeroa
a Servicio de Dermatología, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
b Servicio de Anatomía Patológica, Hospital Clínico Universitario de Valladolid, Valladolid, Spain
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and the results revealed lymphocyte aggregates in the superficial dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A&#41; interspersed with infiltrate-free areas&#46; The infiltrate was composed of atypical small-to-medium lymphocytes&#44; with an irregular nucleus&#46; There was marked epidermotropism &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>B and C&#41;&#44; and isolated areas of the basement membrane had been destroyed&#46; Immunohistochemistry &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>D&#41; revealed a particularly intense infiltrate composed of CD3<span class="elsevierStyleSup">&#43;</span> and CD4<span class="elsevierStyleSup">&#43;</span> cells&#44; with less intense staining of CD8<span class="elsevierStyleSup">&#43;</span> cells and CD30<span class="elsevierStyleSup">&#8211;</span> cells&#46; The hair follicles and eccrine glands were not involved&#44; and no dermal mucin deposits were found&#46; Polymerase chain reaction assay of gene rearrangement in the T-cell receptor from the skin biopsy revealed monoclonal T-cell proliferation&#46; These findings confirmed a diagnosis of papular mycosis fungoides&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Laboratory tests disclosed mild hypercholesterolemia&#59; the remaining values&#44; including lactate dehydrogenase and &#223;2-microglobulin were within the reference range&#46; There were no palpable lymph nodes in the groin or axillas&#46; Chest radiograph and abdominal ultrasound findings were normal&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was treated with high-potency local topical corticosteroids &#40;clobetasol cream 0&#46;05&#37;&#41; once daily for 3 weeks&#46; Since his condition improved only slightly&#44; treatment with psoralen-UV-A &#40;PUVA&#41; &#40;18 sessions&#41; was started &#40;oral methoxsalen at 20<span class="elsevierStyleHsp" style=""></span>mg before PUVA&#41;&#46; The cutaneous symptoms disappeared almost completely with this treatment&#46; Today&#44; 18 months later&#44; the patient is free of lesions and other signs of mycosis fungoides&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Mycosis fungoides is considered to be the most common form of cutaneous T-cell lymphoma&#46; It tends to takes the form of blotches that appear on areas of the body usually not exposed to sunlight&#46; However&#44; the clinical manifestations of mycosis fungoides vary considerably&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> and up to 50 variants have been described&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> One such variant is papular mycosis fungoides&#44; an uncommon entity first described by Kodama et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> in 2005&#59; only 11 cases have been published in the literature&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Mycosis fungoides usually presents as an asymptomatic persistent eruption of reddish papules generally affecting the trunk in middle-aged patients&#46; The clinical&#44; histopathological&#44; and immunohistochemical findings in our case are similar to those reported for previously published cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;7</span></a> All those authors<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a> concur that this presentation of mycosis fungoides is not very aggressive but also agree that patients should be followed up&#44; because in 1 case it has been reported to progress to plaque-phase mycosis fungoides &#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Prognosis is favorable in this subtype&#44; except in patients with previous symptoms of mycosis fungoides&#44; in whom it indicates disease progression&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The differential diagnosis can be made with lymphomatoid papulosis&#44; especially type B&#44;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5&#44;8</span></a> which usually manifests with ulcerated and crusted lesions that tend to resolve spontaneously&#46; It can also be made with pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica&#44; both of which usually yield intraepidermal neutrophils and necrotic keratinocytes<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> in the histopathology study&#46; Diagnosis can even be made with follicular mycosis fungoides&#44; which is characterized by infiltrate in the follicular epithelium&#44; a certain degree of syringotropism&#44; or both&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> Neri et al&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> proposed a differential diagnosis with aggressive epidermotropic CD8 cytotoxic T-cell lymphoma&#44; after detecting an infiltrate composed of CD8<span class="elsevierStyleSup">&#43;</span>CD4<span class="elsevierStyleSup">&#8211;</span> cells in the case of papular mycosis fungoides they report&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The main therapeutic option for papular mycosis fungoides is PUVA&#44; which is generally successful&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5</span></a> Other options that have been applied in the initial stages<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> include coadjuvant topical retinoids&#44;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> UV-B radiation&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> and topical corticosteroids in monotherapy&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Given the good response with PUVA in the present case and the evidence provided by previously published cases &#40;improvement in 8 out of 12 patients treated&#41;&#44; 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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A&#44; Focal lymphocytic infiltrates in the superficial dermis interspersed with infiltrate-free hypodermis and dermis &#40;hematoxylin-eosin &#91;H-E&#93;&#44; original magnification &#215;2&#41;&#46; B&#44; Detail of well-defined lymphocytic infiltrate in the dermis &#40;H-E&#44; original magnification &#215;10&#41;&#46; C&#44; Small and medium atypical lymphocytes and visible epidermotropism &#40;H-E&#44; original magnification &#215;40&#41;&#46; D&#44; CD3<span class="elsevierStyleSup">&#43;</span> cells are visible throughout the infiltrate &#40;CD3&#44; original magnification&#44; &#215;10&#41;&#46;</p>"
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ISSN: 15782190
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