Ben Ghorbel I, Houman M H, B'chir S, Chamakhi S, Miled M
Service de médecine interne, hôpital La Rabta, 1007 Tunis, Tunisie.
Rev Med Interne. 2001 May;22(5):469-74. doi: 10.1016/s0248-8663(01)00373-3.
Langerhans' cell histiocytosis is a rare disorder of unknown etiology characterized by a wide clinical spectrum and varied behavior. Diabetes insipidus is a relatively common feature in Langerhans' cell histiocytosis. The presence of both diabetes insipidus and mellitus associated with histiocytosis in an adult is rare. To our knowledge, only three previous cases have been reported.
We report the clinical presentation, pathologic findings and clinical progress in an adult female who had disseminated Langerhans' cell histiocytosis (hypothalamic infiltration, multifocal bone involvement) associated with both diabetes insipidus and mellitus.
The pathogenesis of diabetes mellitus in such an association will be discussed.
朗格汉斯细胞组织细胞增多症是一种病因不明的罕见疾病,具有广泛的临床谱和多样的表现。尿崩症是朗格汉斯细胞组织细胞增多症中相对常见的特征。成人同时出现尿崩症和与组织细胞增多症相关的糖尿病较为罕见。据我们所知,此前仅报道过3例。
我们报告了一名成年女性的临床表现、病理结果及临床病程,该患者患有播散性朗格汉斯细胞组织细胞增多症(下丘脑浸润、多灶性骨受累),同时伴有尿崩症和糖尿病。
将讨论这种关联中糖尿病的发病机制。