Steatocystoma Multiplex

Sep 05, 2007 13:16

This is the initial and sole diagnosis by Dr. Lipana and Dr. Datuin, two of the beautiful dermatologists of PGH. It couldn't be cured. The cysts will keep on coming. Should one grow large enough to hurt any of my organs, they will be surgically removed and it'll leave scars. To my friends who are wondering, it's not contagious. Relax. We can still mingle. Haha. And hopefully, it wouldn't affect my lifestyle. There is, however, a cosmetic problem. Recently, I also found out that a close friend has it. Nice to know I'm not alone. The chance of getting it is one in 10,000. We both got lucky in this sick lottery. Here's what I got off the net:

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.
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