Steatocystoma Multiplex Limited to the Vulva: Report of a Very Rare Case Successfully Treated by a Simple Surgical Method

Aim: Steatocystoma multiplex (SM) is a rare malformation characterized by multiple cutaneous cystic lesions containing oily substance. SM has autosomal dominant mode of inheritance, though there are a significant number of sporadic cases reported as well. Characteristically, the lesions manifest around the time of puberty and are found on the sternal areas, face, trunk and scrotum. SM limited to the vulva is a very rare condition as there are only a five cases in the literature.

Case: A 32-year-old woman presented with a 1 year-history of nodules, gradually increasing in size and number, on the labia majora. Similar lesions were not present on other parts of her body and family members. On physical examination, yellowish papules and nodules ranging in size from 2-15mm without punctum were present on the labia majora. Biopsy performed at the time revealed histological changes with steatocystoma multiplex. After a mini incision, evacuation of the cyst followed by the removal of the cyst wall was performed without complication. The lesions healed rapidly without scarring. The patient was followed by yearly controls. Recurrence was not observed until the fourth year of surgery. Smaller lesions were observed on previously untreated areas of vulva which were removed again successfully.

Discussion: SM treatment is difficult. In addition to medical treatments including antibiotic and anti-inflammatory drugs and isotretinoin, there are different surgical treatments including total excision and grafting, cryotherapy and CO2 laser with limited success.

Conclusion: This is a very rare case of SM limited to the vulva, simply and successfully treated by a simple surgical method, emphasizing the importance of cyst wall removal to achieve long time remission.