Document Detail


Familial syringoma: report of two cases with a published work review and the unique association with steatocystoma multiplex.
MedLine Citation:
PMID:  19335690     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Syringoma is a benign neoplasm of eccrine origin. Clinically, it manifests as small skin-colored to yellowish soft papules usually localized around the eyes and on the upper cheeks of middle-aged women. Familial cases have rarely been reported and may be inherited as an autosomal dominant trait or result from either germ line or somatic mutations. Syringoma can coexist with various conditions, notably Down syndrome. Herein, we report a family with multiple syringomas affecting members of three following generations and describe in detail a 36-year-old woman and her 17-year-old son. In the latter, steatocystoma multiplex, which is regarded as a benign cystic neoplasm of the folliculosebaceous unit or a nevoid malformation differentiated in the direction of the sebaceous duct, was associated. Acral distribution of steatocystoma multiplex and its presentation as subcutaneous nodules in this patient were unique.
Authors:
Angelo V Marzano; Roberta Fiorani; Valentina Girgenti; Carlo Crosti; Elvio Alessi
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  The Journal of dermatology     Volume:  36     ISSN:  1346-8138     ISO Abbreviation:  J. Dermatol.     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-04-01     Completed Date:  2009-08-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7600545     Medline TA:  J Dermatol     Country:  England    
Other Details:
Languages:  eng     Pagination:  154-8     Citation Subset:  IM    
Affiliation:
Institute of Dermatological Sciences, University of Milan, IRCCS Foundation, Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena, Milan, Italy.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Epidermal Cyst / complications*,  pathology
Female
Genes, Dominant
Humans
Male
Sweat Gland Neoplasms / complications*,  genetics*,  pathology
Syringoma / complications*,  genetics*,  pathology

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