FEBRILE ULCERONECROTIC MUCHAHABERMANN DISEASE
Keywords:Febrile ulceronecroticMucha-Habermann disease; pityriasislichenoidesetvarioliformisacuta; fatal outcome; T cell monoclonality.
Abstract We describe the case of a 64-years-old woman who was admitted for erysipelas of the face. Despite treatment, evolution was marked by the appearance of a necrotising ulcerative area in the centre of the erysipelas associated with local oedema and headache. A skin biopsy revealed a pityriasislichenoidesetvarioliformisacuta. Corticosteroids led to a rapid stabilisation of lesions, and after 6 months the patient shows only a small area of frontal hypopigmentation. The aetiology remains uncertain. There is no established standard treatment. We would like to draw attention of the medical and surgical specialists to this rare disease. Febrile UlceronecroticMucha-Habermann disease (FUMHD), first defined by Degos et al. in 1966, is a severe variant of pityriasislichenoides et varioliformisacuta (PLEVA). Characteristics of FUMHD include a fulminant course of the disease, painful ulceronecrotic erosions, fever and severe systemic manifestations. In current literature only 40 cases with an overall case fatality rate of 20% are described. Until today the knowledge about the etiology of this disease is only fragmentary, but an association with viral and lymphoproliferative diseases is being discussed.
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