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Baasan's syndrome
Synonyms:
Epidermolysis bullosa-skin absence, epidermolysa bullosa dystrophica (Bart type).
Associated persons:
Marianne Basan
Description:
Epidermolysis bullosa-skin absence, epidermolysa bullosa dystrophica (Bart type).
A form of ectodermal dysplasia. Features incluede coarse scalp hair, which is shed during the second decade of life, sparse eyebrow lashes and body hair, severe dental caries, decreased sweating and dry skin and mucous membranes, thick and short fingernails and toenails with longitudinal ridging, simian creases, tapering of fingertips and absence of fingerprint patterns. Both sexes affected. Autosomal dominant inheritance.
Bibliography:
- M. Basan
Ektodermale Dysplasie fehlendes Papillarmuscler. Nagelveränderungen und Vierfingerfurche.
Archiv für klinische und experimentelle Dermatologie, Berlin, November 1965, 222 (6): 546-557.
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