PMID- 29263553 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 1998-3611 (Electronic) IS - 0019-5154 (Print) IS - 0019-5154 (Linking) VI - 62 IP - 6 DP - 2017 Nov-Dec TI - A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease which Presenting as Toxic Epidermal Necrolysis. PG - 675 LID - 10.4103/ijd.IJD_631_16 [doi] AB - Febrile ulceronecrotic Mucha-Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta, is an inflammatory dermatosis of unknown etiology manifested by ulcerative and necrotic lesions accompanied by systemic manifestations. The mortality rate of FUMHD is about 15%. It is reported here a case of FUMHD presenting as toxic epidermal necrolysis that resulted in multiple organ failure and death. FAU - Xing, Chenjing AU - Xing C AD - Department of Dermatology, Hangzhou Institute of Dermatology and Venereology, The Third People's Hospital of Hangzhou, Hangzhou, China. FAU - Shen, Hong AU - Shen H AD - Department of Dermatology, Hangzhou Institute of Dermatology and Venereology, The Third People's Hospital of Hangzhou, Hangzhou, China. FAU - Xu, Junzhu AU - Xu J AD - Department of Dermatology, Hangzhou Institute of Dermatology and Venereology, The Third People's Hospital of Hangzhou, Hangzhou, China. FAU - Liu, Zehu AU - Liu Z AD - Department of Dermatology, Hangzhou Institute of Dermatology and Venereology, The Third People's Hospital of Hangzhou, Hangzhou, China. FAU - Zhu, Jun AU - Zhu J AD - Department of Dermatology, Hangzhou Institute of Dermatology and Venereology, The Third People's Hospital of Hangzhou, Hangzhou, China. FAU - Xu, Aie AU - Xu A AD - Department of Dermatology, Hangzhou Institute of Dermatology and Venereology, The Third People's Hospital of Hangzhou, Hangzhou, China. LA - eng PT - Journal Article PL - India TA - Indian J Dermatol JT - Indian journal of dermatology JID - 0370750 PMC - PMC5724328 OTO - NOTNLM OT - Febrile ulceronecrotic Mucha-Habermann disease OT - skin OT - toxic epidermal necrolysis COIS- There are no conflicts of interest. What is new? The exact pathogenesis of FUMHD is unknown. Although various treatment options have been tried, treatment efficacy is difficult to determine because of the small number of reported cases. Systemic steroids and IVIG is considered to be effective in some reports, but in this case Our patient did not respond well to these treatment measures. More case reports and treatment experience are needed to help establish an ideal approach for its management. EDAT- 2017/12/22 06:00 MHDA- 2017/12/22 06:01 PMCR- 2017/11/01 CRDT- 2017/12/22 06:00 PHST- 2017/12/22 06:00 [entrez] PHST- 2017/12/22 06:00 [pubmed] PHST- 2017/12/22 06:01 [medline] PHST- 2017/11/01 00:00 [pmc-release] AID - IJD-62-675d [pii] AID - 10.4103/ijd.IJD_631_16 [doi] PST - ppublish SO - Indian J Dermatol. 2017 Nov-Dec;62(6):675. doi: 10.4103/ijd.IJD_631_16.