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)

Werner Kempf, Sonja Ostheeren-Michaelis, Marco Paulli, Marco Lucioni, Janine Wechsler, Heike Audring, Chalid Assaf, Thomas Rüdiger, Rein Willemze, Chris J L M Meijer, Emilio Berti, Lorenzo Cerroni, Marco Santucci, Christian Hallermann, Mark Berneburg, Sergio Chimenti, Alistair Robson, Martà Marschalko, Dmitry V. Kazakov, Tony PetrellaSylvie Fraitag, Agnes Carlotti, Philippe Courville, Hubert Laeng, Robert Knobler, Philippa Golling, Reinhard Dummer, Günter Burg

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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.

Original languageEnglish
Pages (from-to)1609-1617
Number of pages9
JournalArchives of Dermatology
Volume144
Issue number12
DOIs
Publication statusPublished - Dec 2008

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ASJC Scopus subject areas

  • Dermatology

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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., ... 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. https://doi.org/10.1001/archdermatol.2008.46