Mortality risk factors in febrile ulceronecrotic Mucha- Habermann disease: a systematic review of therapeutic outcomes and complications

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a variant of pityriasis lichenoides et varioliformis acuta (PLEVA). Although rare, the condition may progress to involve serious complications and even lead to fatal outcomes if diagnosis and appropriate treatment is delayed. A PubMed search following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRIMSA) guidelines was performed to find cases of FUMHD from the earliest records to October 2021. Treatments, complications, and patient outcomes were extracted from the literature and summarized, while a review of quality was also performed. A total of 63 publications with 68 patients were found. Successful treatment modalities for FUMHD included antibiotics, antivirals, systemic steroids, methotrexate (MTX), cyclophosphamide, cyclosporine (CYA), intravenous immunoglobulins (IVIG), pentoxifylline, and ultraviolet B phototherapy. Out of 68 patients, 55 patients had their condition fully resolved and 13 cases were fatal. Increased age, systemic involvement, and monoclonal T-cell receptor rearrangement were associated with worst prognosis, but mucosal involvement did not affect mortality risk. Overall, the publications had low risk of bias, but most lacked adequate follow-up periods. FUMHD is a diagnostic and therapeutic challenge due to the lack of clearly defined diagnostic criteria and optimum treatment. Further studies with larger patient populations and longer follow-up periods may lead to refinement of diagnostic criteria, establish an optimum treatment regimen, and better estimate the likelihood of recurrence.

Febrile ulceronecrotic Mucha-Habermann disease - a case and treatment review

“…In more than one case that failed initial systemic therapy, MTX showed excellent results [38,6]. Another recent systematic review that includes children and adults focused on mortality risk factors in FUMHD and therapeutic outcomes and complications [39].…”

“…Both pediatric and adult patients with a FUMHD mortality risk score 10 may benefit from an early transfer to an appropriate intensive care unit. Later, a systematic review found no association of mucosal involvement and mortality risk [39]. Also, monoclonal T cell receptor rearrangement was generally associated with a higher mortality risk [10].…”

“…Febrile ulceronecrotic Mucha-Habermann disease recurrences have been reported in literature, though are less severe than the primary episode. On a recent review, follow-up of 45 patients ranged from two weeks to 5 years, with recurrence being associated with the discontinuation of medication, more specifically MTX [39]. The authors suggest a followup of patients of at least 9 months to accurately represent the likelihood of recurrence.…”

“…The authors suggest a followup of patients of at least 9 months to accurately represent the likelihood of recurrence. Methotrexate is the most commonly prescribed medication for maintenance therapy in FUMDH [39].…”