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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]