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

Abstract

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, <i>p</i> = 0.046), but not positive blood bacterial cultures (<i>p</i> = 0.102). Systematic corticosteroids combination with other immunosuppressants (methotrexate or cyclosporine) were associated with significantly more effective cases (26/31 = 83.9%, χ<sup>2</sup> = 4.065, <i>p</i> = 0.044). Furthermore, no significant differences between the low-dose and high-dose systematic corticosteroid groups were detected in treatment validation (<i>p</i> > 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.

Rapid recovery in a child with febrile ulceronecrotic Mucha‐Habermann disease following intravenous immunoglobulin administration

“…A good response following administration of IVIg was observed in 43.8% of cases and a fatal outcome despite therapy was reported in two cases. 5 After a single low-dose IVIg infusion, our child improved significantly, and the fever remitted immediately. Thus, IVIg could serve as an adjunctive treatment modality in managing this lifethreatening condition in combination with other systemic agents.…”

“…IVIg has accumulating evidence in treating various dermatological conditions safely. 5 The proposed mechanisms of IVIg include inhibition of autoreactive T cells and synergistic action with steroids, causing suppression of lymphocyte activation. There are a few case reports in the literature (Table 1) where IVIg has been used as an adjunct for disease control.…”

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

“…This life-threatening variant of PLEVA is also known as PLEVA fulminans. Febrile ulceronecrotic Mucha-Habermann disease is distinguishable from the two most common types of pityriasis, PLEVA and pityriasis chronica [2]. It is characterized by a sudden onset of multiple generalized ulceronecrotic papules which rapidly coalesce into large ulcers associated with fever [3,4].…”

A Diagnostically Challenging Case of De Novo Febrile Ulceronecrotic Mucha-Habermann Disease with Fatal Pulmonary Involvement: A Case Report

“…On the other hand, non-infectious triggers including drugs such as levamisole-adulterated, cocaine, tegafur, and multivitamins were also reported in the literature. The co-existence of cutaneous T-cell lymphoma in patients with FUMHD suggests that increased monoclonality or polycolnality in T cells is a predisposing factor for this disease [5][6][7][8][9][10][11][12]. All of these different theories drove dermatologists to use multiple management modalities [4].…”