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

Details

Serval ID
serval:BIB_87C68F92E00C
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?
Journal
Journal of the American Academy of Dermatology
Author(s)
Cozzio A., Hafner J., Kempf W., Häffner A., Palmedo G., Michaelis S., Gilliet M., Zimmermann D., Burg G.
ISSN
1097-6787 (Electronic)
ISSN-L
0190-9622
Publication state
Published
Issued date
2004
Volume
51
Number
6
Pages
1014-1017
Language
english
Notes
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish
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.
Keywords
Adult, Aged, Clone Cells, Female, Fever/etiology, Humans, Pityriasis Lichenoides/immunology, Pityriasis Lichenoides/pathology, Skin/immunology, Skin/pathology, T-Lymphocytes
Pubmed
Web of science
Create date
19/11/2012 18:37
Last modification date
20/08/2019 14:47
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