Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and Review of the Literature

Abstract

Febrile ulceronecrotic Mucha-Habermann disease is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by the sudden onset of ulceronecrotic skin lesions and associated with high fever and systemic symptoms. We report here a case of a 20-year-old woman in whom the disease started as pityriasis lichenoides et varioliformis acuta and evolved to febrile ulceronecrotic Mucha-Habermann disease. Almost 90% of the body surface was involved, together with high fever and malaise. Steroids alone proved to be an insufficient therapeutic procedure. The remission achieved was attributed to the use of methotrexate. To our knowledge, only 39 cases of febrile ulceronecrotic Mucha-Habermann disease have been reported in the literature to date.

Febrile ulceronecrotic Mucha–Habermann disease during pregnancy: A case report

“…FUMHD occurs more frequently in children, adolescents or young adults than in older subjects. 2 Up to 15% mortality rate that increased with the age of patients has been reported. 3 In the cases with fatal outcome, death was attributed to pulmonary thromboembolism, pneumonia, cardiac arrest, sepsis, hypovolemic shock and massive thrombosis of the superior mesenteric artery.…”

“…3 In the cases with fatal outcome, death was attributed to pulmonary thromboembolism, pneumonia, cardiac arrest, sepsis, hypovolemic shock and massive thrombosis of the superior mesenteric artery. 2 Although the exact mechanism of PL remains unknown, infectious agents or T-cell dyscrasia leading to an inflammatory reaction or immune complex-mediated hypersensitivity vasculitis are the three major hypotheses considered in the aetiopathogenesis of PL. 4 As is known, there is an increased susceptibility to urinary tract infections in pregnancy.…”

A case of febrile ulceronecrotic Mucha‐Habermann disease responsive to systemic corticosteroids

“…This is a case of FUMHD that was resolved with systemic corticosteroids. FUMHD is a rare, severe subtype of pityriasis lichenoides et varioliformis acuta (PLEVA), a cutaneous inflammatory disorder that commonly affects young males 1 . Most patients do not have mucosal involvement but it can be seen in some cases.…”