Familial syringoma: report of two cases with a published work review and the unique association with steatocystoma multiplex.

J Dermatol

Institute of Dermatological Sciences, University of Milan, IRCCS Foundation, Ospedale Maggiore Policlinico, Mangiagalli and Regina Elena, Milan, Italy.

Published: March 2009

AI Article Synopsis

  • Syringoma is a benign skin tumor originating from sweat glands, typically appearing as small, soft papules near the eyes and cheeks, commonly in middle-aged women.
  • Familial cases of syringoma are rare but can be inherited or caused by genetic mutations and may also be associated with other conditions like Down syndrome.
  • The report describes a family across three generations with multiple syringomas, highlighting a 36-year-old woman and her 17-year-old son, who also has a related condition called steatocystoma multiplex, characterized by unique subcutaneous nodules.

Article Abstract

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.

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http://dx.doi.org/10.1111/j.1346-8138.2009.00613.xDOI Listing

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