Granulomatous slack skin disease--disease features and response to pentostatin.
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.
Osuji
Nnenna
N
Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, UK.
Fearfield
Louise
L
Matutes
Estella
E
Wotherspoon
Andrew C
AC
Bunker
Christopher
C
Catovsky
Daniel
D
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
D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D015649:Pentostatin