title: 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.affiliation: Department of Dermatology, University Hospital of Zurich, Switzerland. cozzio@usz.ch
authors.forename: Antonio
authors.initials: A
authors.lastname: Cozzio
authors.forename: Jürg
authors.initials: J
authors.lastname: Hafner
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.forename: Andreas
authors.initials: A
authors.lastname: Häffner
authors.forename: Gabriele
authors.initials: G
authors.lastname: Palmedo
authors.forename: Sonja
authors.initials: S
authors.lastname: Michaelis
authors.forename: Michel
authors.initials: M
authors.lastname: Gilliet
authors.forename: Dieter
authors.initials: D
authors.lastname: Zimmermann
authors.forename: Günter
authors.initials: G
authors.lastname: Burg
mesh_terms: D000328:Adult; D000368:Aged; D002999:Clone Cells; D005260:Female; D005334:Fever / Q000209:etiology; D006801:Humans; D017514:Pityriasis Lichenoides / Q000276:immunology* / Q000473:pathology; D012867:Skin / Q000276:immunology* / Q000473:pathology; D013601:T-Lymphocytes