Sasaki T, Ono H, Nakajima H, Sugimoto J
Department of Dermatology, Yokohama City University School of Medicine, Japan.
J Dermatol. 1992 Apr;19(4):246-9. doi: 10.1111/j.1346-8138.1992.tb03217.x.
A female patient with acanthosis nigricans, insulin resistant diabetes, and generalized lipoatrophy is reported. The patient developed skin pigmentation and acanthosis nigricans around the age of 34. Arthralgia, muscle weakness, and peripheral neuropathy were also present when she first visited us at 36 years of age. Dermatomyositis, systemic sclerosis, and internal malignancy were ruled out, and the diagnosis of acanthosis nigricans and insulin resistant diabetes was made. Her diabetes gradually worsened and, since the age of 39, she has been treated with an oral anti-diabetic drug. Around the age of 47, generalized lipoatrophy became prominent. Insulin receptor studies ruled out insulin resistant diabetes type A and B. At this point, we diagnosed this patient as having lipoatrophic diabetes, which is a syndrome characterized by insulin resistant diabetes, acanthosis nigricans, generalized lipoatrophy, and other metabolic disturbances. The control of her diabetes has been poor, and diabetic neuropathy and lipoatrophy-induced painful skin lesions such as clavus and tylosis have been persistent. The present case indicates the importance of careful skin examinations in the diagnosis of this syndrome.
报告了一名患有黑棘皮病、胰岛素抵抗性糖尿病和全身性脂肪萎缩的女性患者。该患者在34岁左右出现皮肤色素沉着和黑棘皮病。36岁首次就诊时还存在关节痛、肌肉无力和周围神经病变。排除了皮肌炎、系统性硬化症和内部恶性肿瘤，诊断为黑棘皮病和胰岛素抵抗性糖尿病。她的糖尿病逐渐恶化，自39岁起接受口服抗糖尿病药物治疗。47岁左右，全身性脂肪萎缩变得明显。胰岛素受体研究排除了A型和B型胰岛素抵抗性糖尿病。此时，我们将该患者诊断为脂肪萎缩性糖尿病，这是一种以胰岛素抵抗性糖尿病、黑棘皮病、全身性脂肪萎缩和其他代谢紊乱为特征的综合征。她的糖尿病控制不佳，糖尿病性神经病变和脂肪萎缩引起的疼痛性皮肤病变如鸡眼和胼胝持续存在。本病例表明在该综合征诊断中仔细进行皮肤检查的重要性。
