Characterized by alopecia, macular degeneration,
growth retardation, cleft hand, ectrodactyly syndactyly, and ectodermal
Alopecia with Macular Degeneration and Growth Retardation.
One case report exists. Autosomal
recessive inheritance most probable.
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.
No significant anesthetic considerations
specific to this condition.
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
Ohdo S, Hirayama K, Terawaki T: Association of ectodermal dysplasia,
ectrodactyly, and macular dystrophy: The EEM syndrome. J Med Genet
Senecky Y, Halpern GJ, Inbar D, et al: Ectodermal dysplasia, ectrodactyly and macular dystrophy
(EEM syndrome) in siblings. Am J Med Genet