Multiple symmetric lipomatosis with vulvar involvement: a rare case report associated with walking difficulty and urination disorder

Multiple symmetric lipomatosis (MSL)—a rare metabolic condition characterized by diffuse, nonencapsulated, painless fat deposits—remains a challenging condition to treat. In addition, the clinical manifestation of MSL in women has not been fully understood due to the male-dominated prevalence of the disease. Here, we report a rare case of MSL with vulvar involvement and provide a review of the literature to increase the understanding of both the diagnosis and treatment of the disease. A 46-year-old Japanese woman, who had a history of chronic alcohol consumption for over 20 years, presented with bilateral giant enlargement of the labia major and complained of difficulty in walking and urination. Magnetic resonance imaging revealed nonencapsulated fat deposits involving the vulvar region and extending around the rectum. The patient underwent pubic lipectomy followed by episioperineoplasty, which resulted in a good esthetic outcome with sufficient patient satisfaction. The morphological presentation of MSL in women is different from that in men, and clinical expression of MSL in the vulvar region is rare but cannot be overlooked owing to the potential of serious sequelae such as walking difficulty, urination disorder, and sexual dysfunction. In such cases, lipectomy followed by episioperineoplasty is indicated for the treatment of the condition and its sequelae, while ensuring less chance of relapse and better esthetic outcome compared with other treatment strategies. Level of evidence: Level V, therapeutic, diagnostic, risk/prognostic study.