Granulomatous slack skin: report of a case associated with Hodgkin's disease and a review of the literature

Abstract

Summary We report a case of granulomatous slack skin (GSS) associated with Hodgkin's disease, and review the literature on this entity. GSS, a variant of cutaneous T‐cell lymphoma, clinically presents with erythematous patches in the flexures, which gradually transform into bulky, pendulous areas of skin. Histology shows an elastolytic granulomatous infiltrate, with atypical lymphoid cells, and occasional epidermotropism. As far as we are aware, 10 cases of GSS, including our patient, have been reported in detail. The male: female ratio of these cases is 9:1, and the age range 15–51 years. Five cases were associated with Hodgkin's disease, one with small lymphocytic lymphoma, and one developed cutaneous T‐cell lymphoma. The axillae, abdomen and groins were the most frequently affected areas. No definitive management for GSS has been established. Surgery has been performed in localized forms, and systemic treatments have included corticosteroids, dapsone, chlorambucil, nitrogen mustard, and radiotherapy. Our patient was treated with chemotherapy for his Hodgkin's disease, and this resulted in complete remission of the lymphoma. Subsequent maintenance therapy with interferon‐alpha produced good control of the cutaneous lesions.

Acquired disorders of elastic tissue: Part II. decreased elastic tissue

“…131,134 Systemic involvement has been reported rarely, occurring in the lymph nodes, spleen, and submucosa of the bronchial tree. 127,131,140,141 Differential diagnosis. The differential diagnosis of granulomatous slack skin includes middermal elastolysis, acquired cutis laxa, and granulomatous cutaneous T-cell lymphoma.…”

Granulomatous slack skin: report of a case with response to electron beam therapy

“…Some authors claimed that GSS was a benign disorder, and a variant of cutaneous T-cell lymphoma [4]. In contrary, some authors suggested that GSS was a precursor of malign lymphoma, especially Hodgkin's disease [7,10,14,15]. However, in the literature, 58% of cases had not been associated with any lymphoproliferative malignancy.…”

Cútis laxa granulomatosa: relato de caso

“…Ulcerações ocorrem eventualmente. 6,7 O caso aqui descrito apresentava localização cutânea preferencial e esplenomegalia, sem evidência de doença em outros sítios anatômicos.…”

“…Os achados mais proeminentes dessa doença incluem a perda dos tecidos reticular e elástico da derme e células gigantes multinucleadas com fagocitose das fibras elásticas. 1,2,6,9 Nas lesões tardias, observa-se infiltrado linfocitário, localizado na derme e no tecido celular subcutâ-neo. 1 Células linfóides com epidermotropismo também são observadas, e microabscessos de Pautrier podem ser encontrados.…”

“…2,10 Essa alteração cromossômica está associada com a patogênese do linfoma de Hodgkin (LH), assim como dos linfomas não Hodgkin, leucemias e sarcoma de Ewing. 6,8 Rearranjos para os genes TCR β 6,9 e TCR δ 7 nas lesões cutâneas da CLG têm sido encontrados, confirmando a possibilidade de doença clonal T. A clonalidade não 3 O uso do interferon α nessa paciente apenas melhorou o quadro de hiperemia cutânea. Outras modalidades de tratamento tópico ou sistêmico podem ser empregadas, com mono ou poliquimioterapia, uso de corticosterói-des, azatioprina e psoraleno-UVA.…”