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Steatocystoma multiplex

  • Author(s): Naik, Narayan S., MD
  • et al.
Main Content

Steatocystoma multiplex
Narayan S. Naik, M.D.1
Dermatology Online Journal 6(1): 10

1. Department of Dermatology, New York University

PATIENT: 22-year-old woman


DURATION: Two years


DISTRIBUTION Neck, chest, and abdomen


HISTORY

The patient presented with a two-year history of multiple lesions over her neck, chest, and abdomen.

The lesions are asymptomatic and have not changed since their appearance. There has been no treatment. She has a history of forming keloids at sites of trauma.

She is in good health and takes no medications. No other family members have similar lesions or any other history of skin disease.


Figure 1Figure 2

PHYSICAL EXAMINATION

Multiple, 3-to-5-mm, smooth, firm, flesh-colored-to-yellow nodules were present over the anterior neck, chest, and upper abdomen. No puncta were noted in any of the lesions. Hyperpigmented, smooth, shiny plaques also were present on the upper chest.


LABORATORY DATA

None


HISTOPATHOLOGY

There is a cyst lined by thin squamous epithelium with sebaceous gland lobules arising from its wall.


DIAGNOSIS

Steatocystoma multiplex


COMMENT

Steatocystoma multiplex is an uncommon, inherited disorder that is characterized by multiple, asymptomatic, variably-sized dermal cysts. The condition is transmitted in an autosomal dominant fashion, although there may be sporadic cases have been documented. Solitary lesions of steatocystoma simplex have no hereditary tendency.

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. The lesions lack surface puncta but may exude a creamy or oily fluid when punctured. The condition has been associated with pachyonychia congenita, acrokeratosis verruciformis, hypertrophic lichen planus, hypohidrosis , hidradenitis suppurativa, and natal teeth.

Histologically, steatocystomas are mid-dermal cysts lined by an eosinophilic, undulating epithelial lining. Flattened, sebaceous lobules are usually present close to or within the cyst wall. Lanugo hairs may be present in the cyst cavity. The cyst lining is thought to be of sebaceous duct origin.

A relationship between steatocystomas and vellus hair cysts has been reported. [1] Steatocystomas share many characteristics with eruptive vellus hair cysts, which include age of onset, genetic mode of transmission, clinical appearance, and distribution. Hybrid lesions with histologic features of both conditions have been described.[2] Both conditions have been reported to occur in close proximity to each other in the same patient. It has been suggested that the two conditions may lie along a spectrum of the same disease process and alternatively that the two conditions are distinct based on the expression of different keratins. Lesions of steatocystoma multiplex express keratins 10 and 17 in contrast to eruptive vellus hair cysts which express only keratin 17.[3]

The condition poses no threat to a patient's health but is frequently a cosmetic problem.[4] There are few satisfactory treatment options. Needle aspiration may decrease the size of lesions for a period of months. The number and extent of lesions usually precludes surgical excision. Inflamed lesions can be injected with intralesional glucocorticoids or incised and drained. Isotretinoin usually does not eradicate the condition but may be used to decrease the size of suppurative lesions.

References

1.Ahn SK; Chung J; Lee WS; Lee SH; Choi EH. Hybrid cysts showing alternate combination of eruptive vellus hair cyst, steatocystoma multiplex, and epidermoid cyst, and an association among the three conditions. Am J Dermatopathol 1996;18:645 PubMed.

2. Hurlimann AF; Panizzon RG; Burg G. Eruptive vellus hair cyst and steatocystoma multiplex: hybrid cysts. Dermatology 1996;192:64 PubMed.

3. Tomkova H; Fujimoto W; Arata J. Expression of keratins (K 10 and K 17) in steatocystoma multiplex, eruptive vellus hair cysts, and epidermoid and trichilemmal cysts. Am J Dermatopathol 1997;19:250 PubMed.

4. Patrizi A; Neri I; Guerrini V; Costa AM; Passarini B. Persistent milia, steatocystoma multiplex and eruptive vellus hair cysts: variable expression of multiple pilosebaceous ducts within an affected family. Dermatology 1998;196:392 PubMed.

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