Febrile Ulceronecrotic Mucha–Habermann Disease: Two Cases with Excellent Response to Methotrexate

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA), featuring large, ulcerative, necrotic skin plaques, high fever, and other systemic symptoms, is a rare disorder of unknown etiology. No randomized controlled trials have established treatment guidelines and multiple modalities are often employed, making it difficult to assess the efficacy of any single agent. We report two cases of this condition in which treatment with methotrexate plus antibiotic treatment for superinfection led to rapid improvement.

A fatal case of febrile ulceronecrotic Mucha-Habermann disease in a child

“…In this respect, methotrexate, which is a less-aggressive immunosuppressive agent, seems to be the most successful therapy in the reported cases. 2 , 13 , 14 …”

A Diagnostically Challenging Case of Febrile Ulceronecrotic Mucha-Habermann Disease in an Adult Female Successfully Treated with Methotrexate and Cyclosporine

“…Early symptoms of FUMHD can vary between patients and as evidenced by our patient, it may take repeat skin biopsies until the correct diagnosis can be reached. Unfortunately, this delay in diagnosis and treatment may jeopardize patients’ chances of recovery, as early intervention has been suggested to improve prognosis [2, 4, 6-8]. To better define this disease, Nofal et al [2] proposed that acute onset of generalized ulceronecrotic papules and plaques associated with fever and histopathological findings consistent with PLEVA is needed to make a diagnosis of FUMHD.…”

“…Various treatments attempted for FUMHD include immunosuppressants, antibiotics, antivirals, phototherapy, intravenous immunoglobulin, and dapsone, but individual treatment efficacy is difficult to determine and largely based on anecdotal evidence from case reports [2, 5]. In the literature, methotrexate is increasingly presented as a favorable treatment modality, although at least four fatalities have occurred despite methotrexate therapy [2, 8]. Given its relative success, methotrexate has been suggested as first-line treatment for FUMHD, but reports have cautioned that FUMHD can recur upon discontinuation of methotrexate, and long-term methotrexate use has been linked to serious side effects including hepatoxicity and hematopoietic suppression [9, 10].…”

A fatal case of febrile ulceronecrotic mucha-habermann disease which presenting as toxic epidermal necrolysis

“…Methotrexate, among the recovered cases described so far, seems to be the most successful therapy. [ 9 10 ] Because of liver dysfunction, we missed the opportunity to use methotrexate. Early intervention with methotrexate may be particularly useful; however, the treatment of FUMHD is still a challenge, and its optimum treatment remains to be determined.…”