28.16.22 Granulomatous Slack Skin Syndrome (GSS) is a rare form of cutaneous T-cell lymphoma.
Clinical features:
(1) The patient initially develops erythematous or violaceous skin plaques
(2) Over time the plaques evolve into sagging of skin (skin redundancy), usually in flexural areas such as axilla and inguinal region.
(3) variable lymphadenopathy
(4) variable splenomegaly
The condition can occur at any age.
A biopsy of affected skin shows:
(1) presence of a monoclonal T-cell infiltrate around blood vessels and/or in papillary dermis. T cells show cerebriform nuclei. Pautrier’s microabscesses may be present.
(2) presence of multinucleated giant cells and macrophages
Some patients also have Hodgkin’s disease.
The response to therapy tends to be limited with the disease showing indolent progression.
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