Bu T S, Kim Y K, Whang K U
Department of Dermatology, College of Medicine, Soonchunhyang University, Seoul, Korea.
J Dermatol. 1997 Apr;24(4):266-9. doi: 10.1111/j.1346-8138.1997.tb02787.x.
Dermatopathia pigmentosa reticularis (DPR) is a very rare disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. Many other dermatologic findings have been associated with this triad, including adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and nonscarring blisters on the dorsa of the hands and feet. The mode of inheritance is unclear but may be autosomally dominant. To our knowledge, only 11 cases have been reported in the world, and none has previously been described in the Orient. We present a Korean patient with the typical features of the DPR triad, along with adermatoglyphia, hypohidrosis, and nonscarring blisters on the dorsa of the feet.
网状色素沉着性皮病(DPR)是一种非常罕见的疾病,具有全身性网状色素沉着、非瘢痕性脱发和甲营养不良三联征。许多其他皮肤表现也与该三联征相关,包括无指纹症、少汗或多汗、掌跖角化过度以及手足背部的非瘢痕性水疱。其遗传方式尚不清楚,但可能为常染色体显性遗传。据我们所知,全世界仅报道过11例,此前在东方地区尚无相关病例描述。我们报告了一名具有DPR三联征典型特征的韩国患者,同时伴有无指纹症、少汗症以及足背部的非瘢痕性水疱。