steatocystoma multiplex

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Steatocystoma multiplex F. Peral Rubio, M.D. Department of Dermatology Hospital Universitario Virgen Macarena, Seville, Spain. www.dermatoblog.com

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Page 1: Steatocystoma multiplex

Steatocystoma multiplex

F. Peral Rubio, M.D.Department of Dermatology

Hospital Universitario Virgen Macarena, Seville, Spain.

www.dermatoblog.com

Page 2: Steatocystoma multiplex

Steatocystoma multiplex

A 30-year-old woman presents with multiple asymptomatic subcutaneous nodules, round-to-oval, well-defined, smooth-surfaced, yellow to skin-colored.

5-10 mm diameter firm papules scattered on the abdomen.

The lesions have been present for about 10 years.

Page 3: Steatocystoma multiplex
Page 4: Steatocystoma multiplex
Page 5: Steatocystoma multiplex

Steatocystoma multiplex

Biopsy specimens shows well-encapsulated dermal cysts with infolded walls lined by stratified squamous epithelium without a granular layer.

Sebaceous gland lobules are found within the cyst wall.

Page 6: Steatocystoma multiplex

Steatocystoma multiplex

Steatocystoma multiplex is an uncommon disorder of the pilosebaceous unit characterized by the development of numerous sebum-containing dermal cysts.

Although steatocystoma multiplex has been described as an autosomal dominant inherited disorder, most presenting cases are sporadic.

Page 7: Steatocystoma multiplex

Steatocystoma multiplex

Affected individuals often present with an increasing number of smooth flesh-to-yellow–colored cysts. The cysts are usually nontender and asymptomatic.

Lesions present as numerous flesh-to-yellow–colored dermal cysts ranging in size from 3 mm to 3 cm. Individual cysts range from elastic to firm and are often freely movable.

Page 8: Steatocystoma multiplex

Steatocystoma multiplex

The condition begins in adolescence or young adulthood and affects both sexes equally. It usually is present on the trunk and proximal extremities, but lesions may occur on the scrotum, thighs, forearms, and back.

Steatocystoma multiplex has been associated with pachyonychia congenita, acrokeratosis verruciformis, hypertrophic lichen planus, hypohidrosis , hidradenitis suppurativa, and natal teeth.

Page 9: Steatocystoma multiplex

Steatocystoma multiplex

Steatocystoma multiplex is a disorder of the pilosebaceous unit that occurs in either a sporadic or an autosomal dominant fashion.

Androgenic stimulation of the sebaceous gland, along with environmental factors and the site and type of the keratin mutation, influence the onset of the sebaceous cysts.

Page 10: Steatocystoma multiplex

Steatocystoma multiplex

F. Peral Rubio, M.D.Department of Dermatology

Hospital Universitario Virgen Macarena, Seville, Spain.

www.dermatoblog.com