Wu H T, Chen B H, Sheen J M, Chang T T, Ko S C
Department of Pediatrics, School of Medicine, Kaohsiung Medical College, Taiwan, Republic of China.
Kaohsiung J Med Sci. 1999 May;15(5):302-6.
The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in Taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. Skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. Brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.
继发于朗格汉斯细胞组织细胞增多症（LCH）的尿崩症发病率在不同报告中有所不同，范围从9.5%至50%，但在台湾文献中从未有过报道。因此，我们报告了一例病例，该患者多尿、多饮、体重减轻超过一年，头皮和躯干出现脂溢性皮炎样皮肤损害超过两年。她的体重和身长均低于第3百分位数。进行了禁水-加压素试验以区分肾性尿崩症和神经性尿崩症。皮肤活检显示为LCH图像，诊断为伴有完全性中枢性尿崩症的LCH。脑部MRI和其他实验室检查均在正常范围内。她接受了鼻腔去氨加压素治疗，并采用TPOG-H 94方案进行化疗。治疗3个月后，她的皮肤损害消失，每日尿量恢复到正常范围。
