Febrile ulceronecrotic Mucha‐Habermann disease mimicking Kawasaki disease

Weins, Andreas Benedikt; Theiler, Martin; Bogatu, Bettina; Kerl, Karin; Pleimes, Marc; Pachlopnik-Schmid, Jana; Weibel, Lisa

doi:10.1111/ddg.13989

Cited by 2 publications

(2 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Several case reports demonstrate the potential for initial misdiagnosis as other conditions, including: Stevens-Johnson Syndrome (SJS), 8 presumed pustular psoriasis, 47 Kawasaki disease, 62 and most commonly chickenpox. 9 , 10 Although FUMHD can be managed with similar treatments to other conditions such as SJS and Kawasaki disease, accurate diagnosis allows for more targeted management which may improve treatment outcomes.…”

Section: Discussionmentioning

confidence: 99%

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

Tasouli-Drakou

Nguyen²,

Guinn³

et al. 2022

Dermatol Reports

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

Tasouli-Drakou

Nguyen²,

Guinn³

et al. 2022

Dermatol Reports

View full text Add to dashboard Cite

show abstract

“…FUMHD can occur in individuals of almost any age. To the best of our knowledge, over 100 cases of FUMHD have been published in the literature, with the youngest patient being a 9-month-old boy and the oldest being an 82-year-old woman ( 2 , 3 ). A previous systemic review demonstrated distinct outcomes between paediatric and adult populations with FUMHD.…”

mentioning

confidence: 99%

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

Liu

Zhong

Wang³

et al. 2023

Acta Derm Venereol

View full text Add to dashboard Cite

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) that is characterized by a large ulceronecrotic appearance with high fever and a variety of systemic symptoms. We report here a case of FUMHD in a 17-year-old male Chinese patient who was treated successfully with a combination therapy of methotrexate, methylprednisolone, and intravenous immunoglobulin. In addition, a literature review was conducted to summarize the key characteristics of paediatric FUMHD cases.

show abstract

Febrile ulceronecrotic Mucha‐Habermann disease mimicking Kawasaki disease

Cited by 2 publications

References 10 publications

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

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

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

Contact Info

Product

Resources

About