Granulomatous slack skin presenting as diffuse poikiloderma and necrotic ulcers, with features of granulomatous vasculitis and response to oral prednisone, acitretin, and oral psoralen plus ultraviolet light therapy—A case report

نویسندگان

  • Maria Isabel Beatriz Lim Puno
  • Ma. Teresa Ela Dimagiba
  • Ma. Jasmin Jacinto Jamora
  • Filomena Legarda Montinola
  • Patricia Ty Tinio
چکیده

GSS: granulomatous slack skin PUVA: psoralen plus ultraviolet A therapy INTRODUCTION Granulomatous slack skin (GSS), a rare variant of mycosis fungoides, is characterized clinically by bulky, pendulous skin folds on flexural areas and histologically by elastolytic granulomatous infiltrates of clonal T cells. The monoclonal rearrangement of the T-cell receptor b and g genes can be demonstrated by polymerase chain reaction in most cases. The clinical course of GSS is slow and complex, often becoming a diagnostic challenge especially in its early stages. GSS may be confused with leprosy or cutaneous tuberculosis especially if occurring in endemic areas like the Philippines. The prognosis of GSS is influenced by the development of lymphoproliferative concomitant diseases such as Hodgkin disease, non-Hodgkin lymphoma, and acute myelogenous leukemia as evidenced in 50% of reported cases. We report a case of a 43-year-old Filipino man with GSS initially diagnosed as cutaneous tuberculosis. To date, there are only about 50 cases of GSS reported in literature with features of large vessel involvement reported in only 4 cases and necrobiotic changes reported in 1 case.

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عنوان ژورنال:

دوره 3  شماره 

صفحات  -

تاریخ انتشار 2017