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Journal of the American Academy of Dermatology
Volume 2, n° 4
pages 309-317 (avril 1980)
Doi : 10.1016/S0190-9622(80)80043-0
Clinical and laboratory studies

Lamellar ichthyosis, dwarfism, mental retardation, and hair shaft abnormalities : A link between the ichthyosis-associated and BIDS syndromes

Joseph L. Jorizzo, M.D., Robert G. Crounse, M.D. , Clayton E. Wheeler, M.D.
 From the Department of Dermatology, University of North Carolina, School of Medicine, USA 

1Reprint requests to: Dr. Robert G. Crounse, Department of Dermatology, University of North Carolina, School of Medicine, Chapel Hill, NC 27514.

There is an expanding list of syndromes that combine ichthyosis with neuroectodermal and mesodermal defects. We present the case of a young woman with the following features: lamellar ichthyosis, dwarfism, mental retardation, nail and dental abnormalities, unusual facies, poor sexual maturation, punctate cataracts, and hair shaft abnormalities. There were no neurologic or auditory defects. Hair examination revealed pili torti, trichoschisis, bright and dark bands with polarizing microscopy, and a low hair sulfur content. The patient did not have aminoaciduria or immunoglobulin abnormalities. We believe that this case is a link between the ichthyosis-associated syndromes and the group of syndromes that have in common abnormal hair with clean transverse fractures (trichoschisis) and low sulfur content. A more complete examination of the hair in other patients with ichthyosis and associated abnormalities will probably reveal more patients with this new syndrome.

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