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Characterized by alopecia, macular degeneration, growth retardation, cleft hand, ectrodactyly syndactyly, and ectodermal dysplasia.

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Alopecia with Macular Degeneration and Growth Retardation.

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One case report exists. Autosomal recessive inheritance most probable.

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Repeated loss of scalp and body hair with some regrowth (hypotrichosis) started at the age of 20 years. Ring-shaped (“bull's-eye”) degeneration of the retinal pigmentary epithelium of both eyes with poor visual acuity was noted. Short stature (proportional dwarfism) but otherwise normal musculoskeletal examination. Intelligence was normal.

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No significant anesthetic considerations specific to this condition.

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Ectodermal Dysplasia, Ectrodactyly, and Macular Dystrophy (EEM) Syndrome: Main features are syndactyly and other abnormalities of the fingers and toes, sparse head hair from birth, bilateral macular degeneration, and small widely spaced teeth.

Kuster W, Majewski F, Hammerstein W: Alopecia, macular degeneration, and growth retardation: A new syndrome? Am J Med Genet 28:477, 1987.  [PubMed: 3425621]
Ohdo S, Hirayama K, Terawaki T: Association of ectodermal dysplasia, ectrodactyly, and macular dystrophy: The EEM syndrome. J Med Genet 20:52, 1983.  [PubMed: 6302256]
Senecky Y, Halpern GJ, Inbar D, et al: Ectodermal dysplasia, ectrodactyly and macular dystrophy (EEM syndrome) in siblings. Am J Med Genet 101:195, 2001.  [PubMed: 11424132]

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