24 year old woman presents to clinic with multiple cream colored papules on her chest. She said she first noticed them at around age 13. She reports that her mother has similar bumps. We performed a biopsy and a yellow oily substance came out of the papule when punctured.
What is your diagnosis?
Steatocystoma multiplex is a relatively rare, autodominant, inherited disorder. There is a defect in the keratin 17 gene. Keratin 17 is a protein found in nailbeds, hair follicles and sebaceous glands. Cysts develop at adolescence. Dermatopathologically, the papules seem to be malformations of the sebaceous gland duct. Steatocstomas can occur singularly (simplex) or in large numbers, as in this case, (multiplex). They can occur anywhere on the body and are found in both men and women. Other sub-types have been described including localized, generalized, facial, acral and suppurative.
In the case of Steatocystoma multiplex the treatment is both difficult and unsatisfying due to the very large number of cysts to be managed. Surgical excision , laser ablation, electrocautery and cryotherapy have all been reported to have been used with some success. Oral isotretinoin and oral antibiotics may cause a temporary diminution in the number and size of the cysts, but both are usually not a durable, long term solution.
My approach is to treat the lesions that are symptomatic and encourage my patients to tolerate the lesions that are not.
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