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Steatocystoma multiplex - Skin
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Steatocystoma multiplex - Skin

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Contributors: Chris G. Adigun MD, Jeffrey D. Bernhard MD, Belinda Tan MD, PhD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Steatocystoma multiplex, also known as sebocystomatosis and epidermal polycystic disease, is characterized by multiple, epithelial-lined, sebum-filled dermal cysts that are distinguished by the presence of sebaceous glands within the cyst walls. These lesions may occur sporadically or in an autosomal dominant pattern of inheritance. Steatocystoma multiplex may also occur with syndromes such as pachyonychia congenita type II and Alagille syndrome. In pachyonychia congenita type II, mutations are present in keratins 6b and 17; the development of steatocystomas is thought to be associated with the mutation in keratin 17. Solitary lesions that occur sporadically are termed steatocystoma simplex.

Steatocystoma multiplex tends to present during adolescence or young adulthood. Lesions typically occur on the trunk, upper arms, scrotum, axillae, or chest but in severe cases may be generalized. Lesions are asymptomatic skin-colored or yellowish dermal papules or nodules. When the lesion is incised, oily material may be expressed. Individual cystic lesions may become infected, leading to sinus formation and scarring.

Long-term management is difficult, and spontaneous resolution is not expected to occur.

For more information, see OMIM.

Codes

ICD10CM:
L72.2 – Steatocystoma multiplex

SNOMEDCT:
109433009 – Steatocystoma multiplex

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Last Updated: 03/29/2017
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Steatocystoma multiplex - Skin
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Steatocystoma multiplex : Axilla, Scrotum, Trunk, Upper arm, Groin, Smooth papules, Smooth nodules, Cysts
Clinical image of Steatocystoma multiplex
Many whitish-yellow and brown, smooth papules on the lower chest and abdomen.
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