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Ichthyosis Congenita: Deafness. Presented by Dr. P. J. Koblenzer.
The patient is a 1½-year-old physically and mentally defective Negro boy. Family history shows no known cases of ichthyosis.Soon after birth, the child's skin was noted to be thickened and extremely dry. Sparse hair was present at birth. Since that time the skin has become irregularly thickened particularly on the palms and soles. He frequently develops cracks about the mouth and perianal area which tend to become irritated and infected. He is persistently malodorous. The skin in the diaper area becomes macerated and sheds in large flakes. He has had intermittent low-grade fevers since birth. He has never been known to sweat.There was, on examination, a generalized thickening of the skin, particularly of the palms and soles with marked follicular plugging particularly of these extremities. There was no scalp or eyebrow hair. A few distorted eyelashes
PHILADELPHIA DERMATOLOGICAL SOCIETY. Arch Dermatol. 1965;92(6):754–760. doi:10.1001/archderm.1965.01600180146032
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