Case for Diagnosis
Treatment-Resistant Acral Papules
Pápulas acrales resistentes al tratamiento
J. Castiñeiras González
, P. Sánchez Sambucety, M.Á. Rodríguez Prieto
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Complejo Asistencial Universitario de León, León, Spain
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The lesions had not improved after treatment with oral and topical corticosteroids and antifungal agents.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The physical examination revealed multiple erythematous-desquamative papules on and between the toes of her left foot (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No further lesions were identified on the rest of her skin. No pathological locoregional enlarged lymph nodes were present.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy revealed a superficial band-like lymphoid infiltrate and a deep infiltrate surrounding the glands and eccrine ducts. Changes resulting from syringometaplasia and marked epidermotropism were also visible (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a> A-C). Immunohistochemistry was intensely positive for CD3 and CD4 (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a> D), weakly positive for CD8, and negative for CD30. T-cell receptor gene rearrangement was monoclonal.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The remaining additional tests revealed the following: glucose, 91 mg/dL; urea, 36 mg/dL; creatinine, 0.59 mg/dL; aspartate aminotransferase, 11 IU/L; alanine aminotransferase, 10 IU/L; alkaline phosphatase, 72 IU/L; γ-glutamyl transferase, 12 IU/L; total proteins, 7.7 g/L; lactate dehydrogenase, 185 U/L; chloride, 100 mmol/L; sodium, 139 mmol/L; potassium, 4.5 mmol/L; β<span class="elsevierStyleInf">2</span>-microglobulin, 1.65 mg/L; leukocytes, 9900/μL (normal formula); hemoglobin, 13.6 g/dL; mean corpuscular volume, 91.9 fL; and platelets, 174 000/μL. Computed tomography of the thorax, abdomen, and pelvis revealed normal findings.</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What is your diagnosis?</span></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Syringotropic mycosis fungoides.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Treatment was started with topical psoralen–UV-A (8-methoxypsoralen 0.1% o/w emulsion) 3 times per week in combination with clobetasol propionate cream 0.05% once daily. After 3 months of treatment, the lesions resolved, except in those areas that were less exposed to sunlight, such as the interdigital spaces (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Mycosis fungoides is the most common primary cutaneous lymphoma and a classic mimicker of other skin conditions. While multiple clinical, pathological, and clinicopathological variants have been described, the only ones recognized in the latest classification of cutaneous lymphomas of the World Health Organization (WHO) (2016) are folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin syndrome. Syringotropic mycosis fungoides is a very infrequent clinicopathological variant; in other words, it has distinctive clinical and histopathologic characteristics<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>. Given that this condition can be confused with other chronic skin conditions, diagnosis is challenging and requires a high degree of suspicion.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Clinically, mycosis fungoides can be generalized or localized, with a predilection for palms and soles, in the form of patches and/or erythematous-desquamative papules and plaques. Owing to adnexal involvement, these can occur with alopecia, anhidrosis, or follicular accentuation, similar to the folliculotropic variant, from which it may be clinically indistinguishable, except for the anatomical distribution of the lesions. Moreover, classic mycosis fungoides lesions can appear on other areas of the body.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Histologically, the condition is characterized by a superficial band-like infiltrate of atypical lymphocytes, as well as a deep infiltrate, which is more prominent around the eccrine glands. Concomitant involvement of the follicle is observed in 75% of cases<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a>. The characteristics of the infiltrate, together with the finding of syringometaplasia, help to differentiate histologically with respect to the folliculotropic variant<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a>. Similarly, other classic characteristics of mycosis fungoides are often present, for example, epidermotropism. However, the clinical-histopathological correlation is essential when attempting to confirm the diagnosis of this uncommon presentation.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The differential diagnosis should include other clinical pictures such as dyshidrotic eczema, contact dermatitis, and perniosis, in which the differentiating characteristic, in addition to the absence of epidermis and syringotropism, is the presence of lymphocytic vasculitis. Furthermore, it is important to differentiate this condition from the abovementioned folliculotropic MF, since the syringotropic version has an indolent course with a better prognosis. The condition known as syringolymphoid hyperplasia is currently considered a type of syringotropic mycosis fungoides<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a>.</p><p id="par0060" class="elsevierStylePara elsevierViewall">In addition to traditional approaches to mycosis fungoides, various therapies have been proposed for the solitary, or localized, forms of syringotropic mycosis fungoides, including psoralen–UV-A and local radiotherapy, with generally favorable responses reported<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a>. Owing to the depth of the tumor infiltrate, treatments targeting the skin may not be effective<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a>.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Clinical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Diagnosis" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Clinical Course and Treatment" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Comment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Conflicts of Interest" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Castiñeiras González J, Sánchez Sambucety P, Rodríguez Prieto MÁ. Pápulas acrales resistentes al tratamiento. Actas Dermosifiliogr. 2022;113:83–84.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 372 "Ancho" => 830 "Tamanyo" => 38738 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 409 "Ancho" => 830 "Tamanyo" => 137716 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin × 4. B and C, Hematoxylin-eosin × 20. D, CD4 immunohistochemistry × 4.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 457 "Ancho" => 901 "Tamanyo" => 47527 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Variantes clínico-patológicas de micosis fungoide" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "H. Muñoz González" 1 => "A.M. Molina Ruiz" 2 => "L. 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