Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA patients only very rarely have systemic signs; the cutaneous lesions are usually asymptomatic, but may be pruritic and may heal with scarring. FUMHD often starts out as classic PLEVA, but goes on to develop widespread ulceronecrotic lesions and is associated with a high mortality rate. Whether Pityriasis lichenoides chronica (PLC) and PLEVA form a spectrum rather than single entities of clonal lymphoproliferative diseases has been discussed. Recently, it has been proposed that FUMHD, too, is a clonal lymphoproliferative disorder. Here, we report two cases of FUMHD with monoclonal T-cell population, as detected by Southern blot analysis. We propose that clonal FUMHD represents a cutaneous T-cell lymphoma entity.

• Authors: Antonio Cozzio, Jürg Hafner, Werner Kempf, Andreas Häffner, Gabriele Palmedo, Sonja Michaelis, Michel Gilliet, Dieter Zimmermann, Günter Burg
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 51 Issue 6 Pg. 1014-7 (Dec 2004) ISSN: 1097-6787 [Electronic] United States
• PMID: 15583604 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Aged
• Clone Cells
• Female
• Fever (etiology)
• Humans
• Pityriasis Lichenoides (immunology, pathology)
• Skin (immunology, pathology)
• T-Lymphocytes