A case of febrile ulceronecrotic Mucha-Habermann disease requiring debridement of necrotic skin and epidermal autograft

Abstract

We report a case of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) in a 21-year-old man. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse ulcerations associated with high fever and systemic symptoms. It is sometimes lethal especially in elderly patients. In the present case, intense generalized maculopapular erythematous plaques with central necrosis developed progressively in association with a high fever. Initial treatment with systemic betamethasone had been unsuccessful and the skin lesions, which covered about 50% of the body surface, became severely ulcerated. Although the development of new lesions had ceased spontaneously, widespread ulceration of the skin remained. Debridement of the necrotic skin and skin grafting using cultured epidermal autografts and meshed allografts of cadaver skin led to prompt reepithelization.

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

“…Yanaba et al [23] observed a predominantly CD8+ lymphocytes infiltrate around the dermis and epidermis which might suggest a cytotoxic attack of lymphocytes to altered epidermal antigens.Some authors have suggested that PLEVA is a benign clonal disorder of activated T cell lymphocytes. Dereure et al [46] demonstrated that 13 of 20 (65%) PLEVA biopsy specimens had a dominant T cell clone.…”

“…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.…”

“…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.…”

“…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].…”

PUVA-Therapie einer schwer verlaufenden Pityriasis lichenoides et varioliformis acuta

“…Diese seltene Dermatose gehört in die Gruppe der erythematosquamösen Erkrankungen und tritt pleomorph auf: papulosquamöse, hämorrhagisch-vesikulöse, papulonekrotische und selten ulzeronekrotische Verläufe wurden beschrieben [10,12]. Das Manifestationsalter liegt in den ersten Lebensdekaden [10].…”