PMID- 34287852 OWN - NLM STAT- MEDLINE DCOM- 20220317 LR - 20220317 IS - 1365-4632 (Electronic) IS - 0011-9059 (Linking) VI - 61 IP - 4 DP - 2022 Apr TI - Mucha-Habermann disease: a pediatric case report and proposal of a risk score. PG - 401-409 LID - 10.1111/ijd.15770 [doi] AB - 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. CI - (c) 2021 The Authors. International Journal of Dermatology published by Wiley Periodicals LLC on behalf of the International Society of Dermatology. FAU - Blohm, Martin Ernst AU - Blohm ME AUID- ORCID: 0000-0003-3873-0634 AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. AD - Division of Neonatology and Paediatric Intensive Care, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Ebenebe, Chinedu Ulrich AU - Ebenebe CU AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. AD - Division of Neonatology and Paediatric Intensive Care, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Rau, Cornelius AU - Rau C AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. AD - Division of Neonatology and Paediatric Intensive Care, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Escherich, Carolin AU - Escherich C AD - Department of Paediatric Oncology, Haematology and Clinical Immunology, University Hospital Dusseldorf, Dusseldorf, Germany. FAU - Johannsen, Jessika AU - Johannsen J AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Escherich, Gabriele AU - Escherich G AD - Department of Paediatric Haematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Driemeyer, Joenna AU - Driemeyer J AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Nagel, Philipp Daniel AU - Nagel PD AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. AD - Division of Neonatology and Paediatric Intensive Care, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Kobbe, Robin AU - Kobbe R AD - Division of Infectious Diseases, First Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Lutgehetmann, Marc AU - Lutgehetmann M AD - Institute of Medical Microbiology, Virology and Hygiene, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Lennartz, Maximilian AU - Lennartz M AD - Institute of Pathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Booken, Nina AU - Booken N AD - Department of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Schneider, Stefan Werner AU - Schneider SW AD - Department of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Singer, Dominique AU - Singer D AD - Department of Paediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. AD - Division of Neonatology and Paediatric Intensive Care, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20210720 PL - England TA - Int J Dermatol JT - International journal of dermatology JID - 0243704 RN - 0 (Immunosuppressive Agents) SB - IM MH - Adolescent MH - Adult MH - Child MH - *Herpes Simplex MH - Humans MH - Immunosuppressive Agents MH - Male MH - Middle Aged MH - *Pityriasis Lichenoides/complications MH - Risk Factors MH - *Thrombocytopenia MH - Young Adult EDAT- 2021/07/22 06:00 MHDA- 2022/03/18 06:00 CRDT- 2021/07/21 13:09 PHST- 2021/03/31 00:00 [revised] PHST- 2020/12/01 00:00 [received] PHST- 2021/06/17 00:00 [accepted] PHST- 2021/07/22 06:00 [pubmed] PHST- 2022/03/18 06:00 [medline] PHST- 2021/07/21 13:09 [entrez] AID - 10.1111/ijd.15770 [doi] PST - ppublish SO - Int J Dermatol. 2022 Apr;61(4):401-409. doi: 10.1111/ijd.15770. Epub 2021 Jul 20.