Twede James V, Turner Joyce T, Biesecker Leslie G, Darling Thomas N
Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814, USA.
J Am Acad Dermatol. 2005 May;52(5):834-8. doi: 10.1016/j.jaad.2004.12.047.
Proteus syndrome is a rare overgrowth disorder that is generally progressive, but the natural history of the skin lesions is not known.
Our purpose was to document the evolution of 4 common skin lesions in 16 patients with Proteus syndrome.
Most epidermal nevi and vascular malformations were reported to appear in the first month of life and had little tendency for expansion or development of additional lesions. Subcutaneous lipomas and cerebriform connective tissue nevi were commonly noted in the first year of life, but not in the first month. Most patients reported that subcutaneous lipomas and cerebriform connective tissue nevi progressively increased in size, and in most patients additional lesions developed at new locations. Of the 4 types of skin lesions, plantar cerebriform connective tissue nevi were most frequently cited as a source of symptoms.
Skin lesions of Proteus syndrome may not appear until later infancy or early childhood, making it difficult to diagnose in young children.
变形综合征是一种罕见的过度生长疾病，通常呈进行性发展，但皮肤病变的自然病程尚不清楚。
我们的目的是记录16例变形综合征患者4种常见皮肤病变的演变过程。
大多数表皮痣和血管畸形据报道在出生后的第一个月出现，且几乎没有扩展或出现额外病变的趋势。皮下脂肪瘤和脑回状结缔组织痣通常在出生后的第一年被发现，但在第一个月未出现。大多数患者报告皮下脂肪瘤和脑回状结缔组织痣的大小逐渐增加，并且大多数患者在新的部位出现了额外的病变。在这4种皮肤病变中，足底脑回状结缔组织痣最常被认为是症状的来源。
变形综合征的皮肤病变可能直到婴儿晚期或幼儿期才出现，这使得在幼儿中难以诊断。
