Ogino J, Saga K, Kagaya M, Kamada A, Kaneko R, Jimbow K
Department of Dermatology, Sapporo Medical University School of Medicine, Chyuo-ku, Minami 1, Nishi 16, Sapporo, Japan.
Br J Dermatol. 2004 Mar;150(3):589-93. doi: 10.1111/j.1365-2133.2004.05872.x.
Idiopathic acquired generalized anhidrosis is a very rare disease of unknown pathogenesis. We report a 25-year-old man with acquired generalized anhidrosis due to occlusion of the coiled ducts. He did not have sweat secretion over the entire surface of the body, including the palms and soles. Sweat-inducing stimuli provoked tingling pain on the skin. Pilocarpine iontophoresis on the forearm did not induce sweat secretion. Neurological examination did not reveal any abnormality in the central or peripheral nervous system. Skin biopsy showed that the coiled ducts were occluded by an amorphous eosinophilic substance. This amorphous eosinophilic substance was positive with periodic acid-Schiff (PAS) staining and was resistant to digestion by diastase. Electron microscopy demonstrated that the coiled ducts were completely occluded by an amorphous substance. The substance occluding the coiled ducts contained fibrous structures. These findings suggested that the acquired generalized anhidrosis in this patient was caused by occlusion of the coiled ducts by a PAS-positive substance probably derived from dark cell granules.
特发性获得性全身性无汗症是一种发病机制不明的罕见疾病。我们报告一名25岁男性，因蟠管阻塞导致获得性全身性无汗症。他全身包括手掌和脚底均无汗液分泌。发汗刺激可引起皮肤刺痛。前臂毛果芸香碱离子透入法未诱发出汗液分泌。神经学检查未发现中枢或周围神经系统有任何异常。皮肤活检显示蟠管被一种无定形嗜酸性物质阻塞。这种无定形嗜酸性物质经高碘酸-希夫（PAS）染色呈阳性，且对淀粉酶消化具有抗性。电子显微镜检查表明蟠管被一种无定形物质完全阻塞。阻塞蟠管的物质含有纤维结构。这些发现提示该患者的获得性全身性无汗症是由一种可能源自暗细胞颗粒的PAS阳性物质阻塞蟠管所致。
