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Steatocystoma multiplex


Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Steatocystoma multiplex represents a benign hamartomatous malformation of the pilosebaceous unit. Although probably genetically heterogeneous, steatocystoma multiplex often demonstrates an autosomal-dominant pattern of inheritance. Some pedigrees have demonstrable keratin 17 abnormalities. Clinically, 2–15-mm firm, white, and skin-colored dermal cysts are seen on the trunk, proximal limbs, axilla, face, and scalp. Rare variants may be seen on vulva and palms. An oily white liquid can be expressed from many cysts. The adolescent age of onset suggests a hormonal basis. These are true sebaceous cysts: they contain sebum, and sebaceous gland lobules are present in their walls. Overlap with eruptive vellus hair cysts and an association with pachyonychia congenita type II have been reported. Some cases may be associated with persistent primary dentition.

Management Strategy

The cysts persist indefinitely. Although usually a minor cosmetic problem, they can be highly disfiguring. Paradoxically, those patients who would benefit most from treatment are sometimes regarded as being unsuitable for surgery because they have too many cysts to excise. The surgical technique described later is quick and can be used on large numbers of lesions in one session. It produces good cosmetic results.

Lesions can become inflamed due to rupture of the cyst wall, with leakage of the contents into the dermis, or because of secondary bacterial infection. Suppuration and scarring may follow. The clinical picture then resembles cystic acne and is called steatocystoma multiplex suppurativum. Oral isotretinoin is an effective treatment for inflammatory lesions but not for non-inflamed cysts. This suggests it operates by a direct antiinflammatory effect rather than by reducing the sebum excretion rate. Alternatively, inflamed cysts can be treated with incision and drainage , intralesional triamcinolone , tetracycline 1 g/day, or minocycline 100–200 mg/day.

Topical treatment is largely ineffective because it does not penetrate to reach the cyst wall.

Specific Investigations

  • Skin biopsy if diagnosis is in doubt

  • Keratin gene testing

A novel mutation (p. Arg94Gly) of keratin 17 in a Chinese family with steatocystoma multiplex

Zang D, Zhou C, He M, et al. Eur J Dermatol 2011; 21: 142–4.

Steatocystoma multiplex, oligodontia and partial persistent primary dentition associated with a novel keratin 17 mutation

Gass JK, Wilson NJ, Smith FJ, et al. Br J Dermatol 2009; 161: 1396–8.

First-Line Therapies

Lesions not inflamed

  • Surgical incision and extraction of cyst wall

  • D

Inflamed lesions

  • Isotretinoin

  • D

  • Antibiotics

  • E

  • Incision and drainage

  • E

Steatocystoma multiplex suppurativum: treatment with isotretinoin

Schwartz JL, Goldsmith LA. Cutis 1984; 34: 149–53.

The treatment of steatocystoma multiplex suppurativum with isotretinoin

Statham BN, Cunliffe WJ. Br J Dermatol 1984; 111: 246.

Isotretinoin in the treatment of steatocystoma multiplex: a possible adverse reaction

Rosen BL, Broadkin RH. Cutis 1986; 115: 115–20.

Steatocystoma multiplex treated with isotretinoin: a delayed response

Mortiz DL, Silverman RA. Cutis 1988; 42: 437–9.

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