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

Détails

ID Serval
serval:BIB_87C68F92E00C
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?
Périodique
Journal of the American Academy of Dermatology
Auteur(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
Statut éditorial
Publié
Date de publication
2004
Volume
51
Numéro
6
Pages
1014-1017
Langue
anglais
Notes
Publication types: Case Reports ; Journal ArticlePublication Status: ppublish
Résumé
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.
Mots-clé
Adult, Aged, Clone Cells, Female, Fever/etiology, Humans, Pityriasis Lichenoides/immunology, Pityriasis Lichenoides/pathology, Skin/immunology, Skin/pathology, T-Lymphocytes
Pubmed
Web of science
Création de la notice
19/11/2012 19:37
Dernière modification de la notice
03/03/2018 19:03
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