Multiple symmetric lipomatosis with vulvar involvement: a rare case report associated with walking difficulty and urination disorder
Multiple symmetric lipomatosis with vulvar involvement: a rare case report associated with walking difficulty and urination disorder
复制标题
DOI：
10.1007/s00238-019-01573-3
复制
发表时间：
2019-09
影响因子：
0.5
通讯作者：
Maiko Kiyama;Koji Kanayama;Solji Roh;Y. Hattori;J. Oba;T. Iida;T. Ushiku;M. Okazaki
中科院分区：
文献类型：
--
作者：
Maiko Kiyama;Koji Kanayama;Solji Roh;Y. Hattori;J. Oba;T. Iida;T. Ushiku;M. Okazaki
多发性对称性脂肪增多症（MSL）-一种罕见的代谢性疾病，其特点是弥漫性，非包膜，无痛性脂肪沉积-仍然是一个具有挑战性的条件治疗。此外，MSL在女性的临床表现还没有得到充分的理解，由于男性占主导地位的疾病的患病率。在此，我们报告一个罕见的病例，MSL与外阴受累，并提供文献回顾，以增加对诊断和治疗的疾病的理解。一位46岁的日本女性，有20多年的长期饮酒史，表现为双侧大阴唇巨大增大，并主诉行走和排尿困难。磁共振成像显示非包膜脂肪沉积涉及外阴地区和直肠周围延伸。该患者接受了耻骨脂肪切除术，随后进行了会阴会阴成形术，这导致了良好的美学结局，患者满意度足够高。女性MSL的形态学表现与男性不同，外阴区域MSL的临床表现罕见，但由于可能出现严重后遗症（如行走困难、排尿障碍和性功能障碍）而不可忽视。在这种情况下，脂肪切除术后，外阴手术是指治疗的条件和后遗症，同时确保复发的机会少，更好的美学效果相比，其他治疗strategy.Level证据：V级，治疗，诊断，风险/预后研究。
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.