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Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-14087

Kempf, W; Ostheeren-Michaelis, S; Paulli, M; Lucioni, M; Wechsler, J; Audring, H; Assaf, C; Rüdiger, T; Willemze, R; Meijer, C J L M; Berti, E; Cerroni, L; Santucci, M; Hallermann, C; Berneburg, M; Chimenti, S; Robson, A; Marschalko, M; Kazakov, D V; Petrella, T; Fraitag, S; Carlotti, A; Courville, P; Laeng, H; Knobler, R; Golling, P; Dummer, R; Burg, G (2008). Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC). Archives of Dermatology, 144(12):1609-1617.

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Abstract

BACKGROUND: Granulomatous cutaneous T-cell lymphomas (CTCLs) are rare and represent a diagnostic challenge. Only limited data on the clinicopathological and prognostic features of granulomatous CTCLs are available. We studied 19 patients with granulomatous CTCLs to further characterize the clinicopathological, therapeutic, and prognostic features. OBSERVATIONS: The group included 15 patients with granulomatous mycosis fungoides (GMF) and 4 with granulomatous slack skin (GSS) defined according to the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Patients with GMF and GSS displayed overlapping histologic features and differed only clinically by the development of bulky skin folds in GSS. Histologically, epidermotropism of lymphocytes was not a prominent feature and was absent in 9 of 19 cases (47%). Stable or progressive disease was observed in most patients despite various treatment modalities. Extracutaneous spread occurred in 5 of 19 patients (26%), second lymphoid neoplasms developed in 4 of 19 patients (21%), and 6 of 19 patients (32%) died of their disease. Disease-specific 5-year survival rate in GMF was 66%. CONCLUSIONS: There are clinical differences between GMF and GSS, but they show overlapping histologic findings and therefore cannot be discriminated by histologic examination alone. Development of hanging skin folds is restricted to the intertriginous body regions. Granulomatous CTCLs show a therapy-resistant, slowly progressive course. The prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
DDC:610 Medicine & health
Language:English
Date:2008
Deposited On:16 Feb 2009 14:12
Last Modified:23 Nov 2012 14:24
Publisher:American Medical Association
ISSN:0003-987X
Publisher DOI:10.1001/archdermatol.2008.46
PubMed ID:19075143

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