Poulin Y, Perry H O, Muller S A
J Am Acad Dermatol. 1984 Apr;10(4):600-10. doi: 10.1016/s0190-9622(84)80264-9.
We are reporting the third case of the Olmsted syndrome, an entity that has received little attention in the medical literature. The syndrome consists of congenital diffuse, sharply marginated keratoderma of the palms and soles, onychodystrophy , constrictions of digits, and periorificial keratoses. Our patient, in addition, had congenital universal alopecia, absence of a premolar tooth, leukokeratosis of oral tissue, and hyperlaxity of the joints. The differential diagnosis includes hidrotic ectodermal dysplasia of the Clouston type, pachyonychia congenita, acrodermatitis enteropathica, keratoma hereditarium mutilans of Vohwinkel , mal de Meleda , and other forms of palmoplantar keratodermas . This patient had originally been reported as having acrodermatitis enteropathica, a condition with which the Olmsted syndrome can easily be confused.
我们报告第3例奥姆斯特德综合征，这一病症在医学文献中很少受到关注。该综合征包括先天性弥漫性、边界清晰的掌跖角化病、甲营养不良、指（趾）缩窄和口周角化病。此外，我们的患者有先天性全身性脱发、一颗前磨牙缺失、口腔组织白色角化病和关节过度松弛。鉴别诊断包括克劳斯顿型汗孔性外胚层发育不良、先天性厚甲症、肠病性肢端皮炎、沃温克尔遗传性残毁性角皮瘤、梅勒达病以及其他形式的掌跖角化病。该患者最初被报告为患有肠病性肢端皮炎，奥姆斯特德综合征很容易与之混淆。
