ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (37)
Steatocystoma multiplex in Adult
Other Resources UpToDate PubMed

Steatocystoma multiplex in Adult

Contributors: Tyler Werbel MD, Susan Burgin MD
Other Resources UpToDate PubMed

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 and Alagille syndrome. The development of steatocystomas is thought to be associated with the mutation in keratin 17 on chromosome 17q21.2. To date, 18 distinct mutations of KRT17 associated with steatocystoma multiplex have been identified. 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, lesions 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. When multiple lesions become inflamed, it has been proposed that this variant be termed steatocystoma multiplex suppurativa.

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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:01/27/2021
Last Updated:01/27/2021
Copyright © 2021 VisualDx®. All rights reserved.
Steatocystoma multiplex in Adult
Steatocystoma multiplex : Axilla, Neck, 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.
Copyright © 2021 VisualDx®. All rights reserved.