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

Cozzio, Antonio; Hafner, Jürg; Kempf, Werner; Häffner, Andreas C.; Palmedo, Gabriele; Michaelis, Sonja; Gilliet, Michel; Zimmermann, Dieter R.; Burg, Günter

doi:10.1016/j.jaad.2004.06.009

Cited by 58 publications

(100 citation statements)

References 25 publications

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“…In fact, until 2004 fatal outcomes were confined to persons older than 40 years. Cozzio et al [28] and Aytekin et al [30] reported cases in 2005 of 26- and 27-year-old females with fatal outcomes. Fatal outcomes were linked to pulmonary thromboembolism, sepsis, hypovolemic shock and thrombosis of superior mesenteric artery.…”

Section: Discussionmentioning

confidence: 99%

“…The clonal nature of FUMHD may represent an aggressive entity of cutaneous T cell lymphomas. Three cases with fatalities had monoclonal disease [27,28,29]: it has been suggested that T cell clonality may be related to disease severity.…”

Section: Discussionmentioning

confidence: 99%

“…Immunosuppressive monotherapy with methotrexate [6,8,11,13,15,19,25,28,29,36,40] or cyclosporine [31,35] or a combination of one of these with a high dose of glucocorticoids [6,8,11,13,25,28,29,36,41,43] has been used in -several cases. Antibiotics like erythro-mycin, acyclovir, ultraviolet photother-apy (PUVA) [11,19], IVIG [5,28,36,41,] photochemotherapy [41], 4,4-diaminodiphenyl sulfone [7,8,10,23,] and debridement with skin grafting [23] have also been used to treat FUMHD.…”

Section: Discussionmentioning

confidence: 99%

“…A febrile ulceronecrotic severe variant of PLEVA, called febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is more destructive and associated to large coalescent skin necrosis with a rapid progression [1]. 48 cases of PLEVA fulminans have been reported to date in the literature with 9 lethal forms (20%) (table 1) [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44]. Oral, genital and conjunctival mucosa can be affected [2,6,9,12,13].…”

Section: Introductionmentioning

confidence: 99%

“…Children tend to have more favorable outcomes and no deaths have been reported in children. In adults, the mortality was attributed to pulmonary thromboembolism [8,34,] pneumonia [4,12,32], cardiac arrest [3], sepsis [9,18,23,28], hypovolemic shock [27], and massive thrombosis of the superior mesenteric artery [34]. The pathogenesis of PLEVA fulminans has not fully been elucidated, although multiple hypotheses exist.…”

Section: Introductionmentioning

confidence: 99%

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Febrile Ulceronecrotic Mucha-Habermann Disease: Treatment with Infliximab and Intravenous Immunoglobulins and Review of the Literature

Meziane¹,

Caudron²,

Dhaille³

et al. 2012

Dermatology

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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta, characterized by an acute onset of ulceronecrotic papules, rapidly coalescing into large ulcers with necrotic crusts, associated with high fever and severe systemic symptoms. We report a case of a 65-year-old woman with a resistant form of FUMHD successfully treated with a tumor necrosis factor-α (TNFα) inhibitor (infliximab). After 1 year of treatment, because of the recurrence of lesions and -occurrence of severe sepsis, we decided to change the therapeutic procedure by introducing intravenous immunoglobulin witch induced a spectacular improvement. Only few cases of FUMHD treated with intravenous immunoglobulin have been reported to date. In our case, we describe the first utilization of TNFα inhibitors in the treatment of FUMHD: TNFα inhibitors may be useful, particularly in resistant cases. Further reports are required to confirm this potential therapeutic option.

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Section: Discussionmentioning

confidence: 99%