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August 2001

Linear Atrophy, Telangiectases, and Soft Nodules Along the Lines of Blaschko With Skeletal Abnormalities

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Arch Dermatol. 2001;137(8):1095-1100. doi:

A 5-year-old girl presented with disseminated atrophic and hyperpigmented skin lesions with scattered telangiectases and soft, yellow-red nodules that she had had since birth. The skin lesions were distributed in a linear and serpiginous pattern along the lines of Blaschko on her limbs and trunk (Figure 1). Some lesions were superficially eroded and crusted, but blisters were not present. The patient had a miniature and asymmetrical face, with large ears. A 2 × 2-cm plaque of scarring alopecia that also had been present since birth was noted on her scalp. Her teeth were irregular and malformed. Her nail plates were irregular and dystrophic. She was of short stature and had syndactyly and polydactyly in her fingers and toes. She had undergone corrective surgery for the polydactyly of her toes 4 years earlier (Figure 2). Her intelligence was normal, and her ophthalmologic examination revealed no abnormalities other than astigmatism of both eyes. Her parents and sister were healthy, without any skin lesions.

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