TY - JOUR
T1 - Familial syringoma
T2 - Report of two cases with a published work review and the unique association with steatocystoma multiplex
AU - Marzano, Angelo V.
AU - Fiorani, Roberta
AU - Girgenti, Valentina
AU - Crosti, Carlo
AU - Alessi, Elvio
PY - 2009/3
Y1 - 2009/3
N2 - 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.
AB - 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.
KW - Benign adnexal skin tumors
KW - Familial syringoma
KW - Multiple syringomas
KW - Steatocystoma
KW - Steatocystoma multiplex
UR - http://www.scopus.com/inward/record.url?scp=62749191470&partnerID=8YFLogxK
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U2 - 10.1111/j.1346-8138.2009.00613.x
DO - 10.1111/j.1346-8138.2009.00613.x
M3 - Article
C2 - 19335690
AN - SCOPUS:62749191470
VL - 36
SP - 154
EP - 158
JO - Journal of Dermatology
JF - Journal of Dermatology
SN - 0385-2407
IS - 3
ER -