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Staphylococcal-scalded skin syndrome:evaluation,diagnosis,and management

World Journal of Pediatrics 2018年第2期14卷 116-120页

作者： Alexander K.C.Leung Benjamin Barankin Kin Fon Leong Department of Pediatrics The Alberta Children's HospitalThe University of CalgaryCalgary#200233-16th Avenue NWCalgaryAB T2M 0H5Canada Toronto Dermatology Centre TorontoONCanada The Pediatric Institute Kuala Lumpur General HospitalKuala LumpurMalaysia

Background Staphylococcal-scalded skin syndrome(SSSS),also known as Ritter disease,is a potentially life-threatening disorder and a pediatric *** diagnosis and treatment is imperative to reduce the morbidity and mortality of this *** purpose of this article is to familiarize physicians with the evaluation,diagnosis,and treatment of *** sources A PubMed search was completed in Clinical Queries using the key terms"Staphylococcal scalded skin syn-drome"and"Ritter disease".Results SSSS is caused by toxigenic strains of Staphylococcus *** of the amino-terminal extracellular domain of desmoglein 1 by staphylococcal exfoliative toxins results in disruption of keratinocytes adhesion and cleavage within the stratum granulosum which leads to bulla *** diagnosis is mainly clinical,based on the findings of tender erythroderma,bullae,and desquamation with a scalded appearance especially in friction zones,periorificial scabs/crusting,positive Nikolsky sign,and absence of mucosal *** empiric treatment with intravenous anti-staphylococcal antibiotic such as nafcillin,oxacillin,or flucloxacillin is essential until cultures are available to guide *** or cefuroxime may be used should the patient have penicillin *** the patient is not improving,critically ill,or in com-munities where the prevalence of methicillin-resistant *** is high,vancomycin should be *** A high index of suspicion is essential for an accurate diagnosis to be made and treatment promptly initiated.

关键词： blisters Desquamation Erythroderma Exfoliative toxins Staphylococcus aureus

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Homozygous Nonsense Mutation in SDR9C7 in a Chinese Patient With Autosomal Recessive Congenital Ichthyosis

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International Journal of Dermatology and Venereology 2023年第1期6卷 52-54页

作者： Xin Huang Zhi-Ming Chen Yong Yang Genetic Skin Disease Center Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIsHospital for Skin Diseases(Institute of Dermatology)Chinese Academy of Medical Sciences and Peking Union Medical CollegeNanjingJiangsu 210042China

Introduction:Autosomal recessive congenital ichthyosis(ARCI)is a heterogeneous group of cornification *** date,14 genes have been found to be related to *** presentation:A 23-year-old woman developed generalized erythroderma and scales over her trunk and limbs shortly after birth,followed by recurrent blisters and nail deformities.A diagnosis of ARCI was made based on her clinical manifestations,family history,and genetic analysis,which revealed a homozygous mutation inSDR9C7(c.187C>T,p.Q63X).Discussion:Most genes responsible for ARCI are associated with epidermal lipid metabolism,which contributes to the cutaneous ***9C7,which encodes short-chain dehydrogenase/reductase family 9C member 7,has also been recently found to play vital roles in this process by regulating ceramide binding to the epidermal cornified cell *** patients clinically suspected to have ARCI,recurrent onychomycosis is a strong indication that they carry aSDR9C7 gene ***:Remarkable phenotypic and genotypic heterogeneity exists among patients with *** analysis is an effective tool in diagnosing this and other hereditary *** patient developed recurrent onychomycosis,a typical presentation of ARCI caused bySDR9C7 mutation,and the unusual blisters further expand the clinical phenotypic spectrum of ARCI.

关键词： autosomal recessive congenital ichthyosis SDR9C7 onychomycosis blisters

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