Morbus Mucha‐Habermann unter dem Bild eines Kawasaki‐Syndroms

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

doi:10.1111/ddg.13989_g

Cited by 2 publications

(2 citation statements)

References 9 publications

(17 reference statements)

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“…We ruled out hematological diseases, such as leukemia or HLH, which would have required systemic steroid treatment. We initiated a treatment with immunoglobulins under the impression of a potential Kawasaki syndrome 45 or an unidentified infection. The multisystem inflammatory syndrome in children associated with SARS-CoV-2 48 was ruled out by repeated PCR and serology, as well as PCR of a skin biopsy.…”

Section: Case Reportmentioning

confidence: 99%

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Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

et al. 2021

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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare inflammatory dermatological disease. A case of a 13-year-old boy with FUMHD possibly triggered by mycoplasma infection is presented. Based on FUMHD cases identified in a MEDLINE literature search, demographic, treatment, and outcome data were analyzed. An FUMHD mortality risk score is proposed based on the likelihood ratios of risk factors for a fatal outcome. Our FUMHD case had marked leukopenia and thrombocytopenia at admission. He recovered without systemic immunosuppressive treatment. Literature review revealed 119 FUMHD cases. Overall lethality was 14/119 (12%, CI 6-17%), and lethality in children was lower (1/54, 2%, CI 0-6%) compared to adults (13/65, 20%, CI 11-31%). Risk factors for a fatal outcome (likelihood ratio; P) were sepsis (24.97, P < 0.001), adult vs. pediatric patient age (11.19; P = 0.001), systemic involvement (19.97, P < 0.001), and mucosal involvement (4.58; P = 0.032). The proposed FUMHD mortality risk score = Age/ 10 + 4 + 4 (if systemic involvement) + 1 (if mucosal involvement) was discriminative (sensitivity 93%, specificity 77%). In FUMHD, immune-suppressive treatment intensity should be balanced against the mortality risk, as infectious complications are a frequent cause of death.

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Section: Case Reportmentioning

confidence: 99%

“…21 Clinically, FUMHD may initially mimic viral illnesses or Kawasaki syndrome. 45 Stevens-Johnson syndrome 27 and malignant conditions involving clonal T-cell proliferation 1,4,6,29,40,47 are other potential differential diagnoses of FUMHD. Treatment options comprise topical or systemic modulation or suppression of the immune system.…”

Section: Introductionmentioning

confidence: 99%

Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

et al. 2021

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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

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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.

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Morbus Mucha‐Habermann unter dem Bild eines Kawasaki‐Syndroms

Cited by 2 publications

References 9 publications

Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

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

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