Frias-Iniesta J, Sanchez-Pedreño P, Martinez-Escribano J A, Jimenez-Martinez A
Dermatology Department, Hospital Virgen de la Arrixaca, Murcia, Spain.
Br J Dermatol. 1997 Jun;136(6):935-8.
We report the case of a 20-year-old man, who was born with an intense erythema of the genital area, unresponsive to any treatment employed. When he was 9 months old, he presented with well-defined hyperkeratotic erythematous plaques around the mouth, eyes, nose, and perianal area, with similar plaques on the lateral aspect of the neck and axillae. At the same time the erythema of the genital area became hyperkeratotic. When he was 2 years old, he presented with a disabling palmoplantar keratoderma, initially focal, and later diffuse, also unresponsive to local or systemic treatments employed. The lesions have varied during the course of the disease without ever clearing completely. The axillary and inguinal plaques have shown spontaneous resolution on occasion. Six skin biopsies have been performed with no conclusive histological diagnosis of any of the typical disorders of keratinization. All treatments, topical and systemic, including etretinate and acitretin, have failed to improve the condition. We believe that this patient has Olmsted syndrome, a rare form of palmoplantar keratoderma with periorificial keratotic plaques.
我们报告了一例20岁男性病例，该患者出生时生殖器区域出现严重红斑，采用任何治疗方法均无反应。9个月大时，他在口周、眼周、鼻周及肛周出现边界清晰的角化过度性红斑斑块，颈部侧面和腋窝也有类似斑块。同时，生殖器区域的红斑出现角化过度。2岁时，他出现致残性掌跖角化病，起初为局限性，后来发展为弥漫性，对采用的局部或全身治疗均无反应。在疾病过程中，皮损有所变化，但从未完全消退。腋窝和腹股沟的斑块偶尔会自行消退。已进行了6次皮肤活检，但未对任何典型的角化异常疾病做出明确的组织学诊断。包括阿维A酯和阿维A在内的所有局部和全身治疗均未能改善病情。我们认为该患者患有奥姆斯特德综合征，这是一种罕见的掌跖角化病，伴有口周角化性斑块。
