Nazzaro V, Brusasco A, Gelmetti C, Ermacora E, Caputo R
Centro per le Malattie Cutanee Ereditarie, University of Milan, Italy.
Pediatr Dermatol. 1990 Sep;7(3):174-8. doi: 10.1111/j.1525-1470.1990.tb00276.x.
A 25-year-old woman who recently gave birth to a baby affected by classic lesions of incontinentia pigmenti (IP), had hypochromic, atrophic, and reticulated streaks on both lower limbs. Her personal history was unremarkable for IP, and physical examination revealed no other cutaneous signs of the disease. Immunohistochemical and electron microscopic studies were performed on biopsies obtained from both normal and hypochromic skin of the leg. Hypochromic skin showed epidermal atrophy and lack of adnexae, without significant melanocyte abnormality. As demonstrated by this patient, hypochromic reticulated streaks can represent the only cutaneous marker of IP in adulthood. Careful search for such skin lesions in the mother of a child with IP is essential in order to ascertain whether there is a risk of its occurrence in future offspring.
一名25岁的女性，其近期分娩的婴儿患有典型的色素失禁症（IP）病变，该女性双下肢有色素减退、萎缩性和网状条纹。她个人既往史中无IP相关异常，体格检查未发现该疾病的其他皮肤体征。对取自腿部正常皮肤和色素减退皮肤的活检组织进行了免疫组织化学和电子显微镜研究。色素减退皮肤显示表皮萎缩且附属器缺失，黑素细胞无明显异常。如该患者所示，色素减退性网状条纹可能是成年期IP唯一的皮肤标志物。仔细检查患有IP的儿童母亲身上的此类皮肤病变，对于确定未来后代是否有发病风险至关重要。
