Febrile Ulceronecrotic Mucha-Habermann Disease: A Case Report and a Systematic Review

The characteristics and treatments of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) are not well-understood. We reported a FUMHD case, and searched Medline, Embase, Pubmed, Scopus, and Web of Science from inception to June 16, 2021, to perform a systematic review to synthesize its characteristics and treatments. Seventy-eight reports, including 84 people were eligible. Most of them were male (62/83, 74.7%), with high fever state (50/80, 62.5% had a high fever of 39°C or above), and with more positive skin bacterial cultures (31/41, 75.6%). Adults were associated with a higher risk of death (OR = 12.976, 95% CI: 1.049, 160.504, p = 0.046), but not positive blood bacterial cultures (p = 0.102). Systematic corticosteroids combination with other immunosuppressants (methotrexate or cyclosporine) were associated with significantly more effective cases (26/31 = 83.9%, χ 2 = 4.065, p = 0.044). Furthermore, no significant differences between the low-dose and high-dose systematic corticosteroid groups were detected in treatment validation (p > 0.05). Overall, FUMHD was associated with male patients, high fever, and positive skin bacterial cultures. Early combination therapy with lower doses of corticosteroids and methotrexate or cyclosporine may be an optimal choice for the treatment of FUMHD.