Steatocystoma multiplex

Steatocystoma multiplex is an unusual inherited disorder in which numerous sebum-containing cysts develop at puberty. These are generally on the chest and may also occur on the abdomen, upper arms and face. In some cases cysts may develop all over the body.

Steatocystoma multiplex

Clinical features

As the tendency to develop cysts is inherited in an autosomal dominant fashion, one parent can be expected to also have steatocystoma multiplex. Both males and females may be affected.

The cysts are mostly small (2-20 mm) but they may be several centimetres in diameter. They tend to be soft to firm, and contain an oily yellow liquid. Sometimes they contain one or more hairs. They may become inflamed and heal with scarring, like acne nodules.

The cysts are thought to come from an abnormal lining of the passageway to the oil glands (sebaceous duct).

Steatocystoma multiplex may be associated with pachyonychia congenita type 2 and with natal teeth (MIM184510).

What is the cause of steatocystoma multiplex?

Steatocystoma mulcitiplex is thought to be due to mutations in the keratin 17 gene (K17). This gene is responsible for a keratin protein found in the nail bed, hair follicles and sebaceous glands.

Treatment of steatocystoma multiplex

• Individual cysts can be removed surgically. In most cases, small incisions (cuts into the skin) allow the cyst and its contents to be extracted through the opening. If it is tethered to the underlying skin, excision biopsy may be necessary.
• Inflammation can be reduced with oral antibiotics
• Oral isotretinoin is not curative but may temporarily shrink the cysts and reduce inflammation.