title: Granulomatous slack skin disease--disease features and response to pentostatin.
abstract: Granulomatous slack skin disease (GSSD) is a rare condition characterized clinically by redundant skin folds, which show a predilection towards flexural areas, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibres. The disease is often indolent, although rapid progression and transformation have been described. There is much debate as to whether this condition is a subset of mycosis fungoides or a separate disease entity in itself. We describe a case of GSSD with unique manifestations including granulomatous bone marrow involvement and hypercalcaemia. The patient has twice achieved a good response to pentostatin after failure of combination chemotherapy. This is the first report of the successful use of the purine analogue pentostatin in the management of GSSD.
authors.affiliation: Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, UK.
authors.forename: Nnenna
authors.initials: N
authors.lastname: Osuji
authors.forename: Louise
authors.initials: L
authors.lastname: Fearfield
authors.forename: Estella
authors.initials: E
authors.lastname: Matutes
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Wotherspoon
authors.forename: Christopher
authors.initials: C
authors.lastname: Bunker
authors.forename: Daniel
authors.initials: D
authors.lastname: Catovsky
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001853:Bone Marrow / Q000473:pathology; D006099:Granuloma / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D016410:Lymphoma, T-Cell, Cutaneous / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D015649:Pentostatin / Q000627:therapeutic use*; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000473:pathology
chemical_list: D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D015649:Pentostatin