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December 1986

Apocrine Hidrocystomas of the Lids, Hypodontia, Palmar-Plantar Hyperkeratosis, and Onychodystrophy A New Variant of Ectodermal Dysplasia

Author Affiliations

From the Departments of Ophthalmology (Drs Font, Schanzer, and Lewis) and Dermatology (Dr Stone), Baylor College of Medicine, Houston.

Arch Ophthalmol. 1986;104(12):1811-1813. doi:10.1001/archopht.1986.01050240085045

• A fourth case of a newly recognized variant of ectodermal dysplasia is reported. This syndrome is characterized by bilateral apocrine hidrocystomas of the eyelid margins, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy. To our knowledge, this syndrome, with its striking ocular manifestations, has not been previously documented in the ophthalmic literature.

Schopf E, Schulz H-J, Passarge E:  Syndrome of cystic eyelids, palmoplantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait . Birth Defects 1971;8:219-221.
Burket JM, Burket BJ, Burket DA:  Eyelid cysts, hypodontia and hypotrichosis . J Am Acad Dermatol 1984;10:922-925.Article
Freire-Maia N, Pinheiro M:  So-called anhidrotic ectodermal dysplasia . Int J Dermatol 1980;19:455-456.Article
Freire-Maia N:  Ectodermal dysplasias . Hum Hered 1971;21;309-312.Article
Freire-Maia N:  Ectodermal dysplasias revisited . Acta Genet Med Gemellol 1977;26:121-131.
McKusick VA: Mendelian Inheritance in Man: Catalogs of Autosomal Dominant, Autosomal Recessive, and X-Linked Phenotypes , ed 6. Baltimore, The Johns Hopkins University Press, 1983, p 795.