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KMID : 0367419960390091315
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Journal of Korean Pediatric Society
1996 Volume.39 No. 9 p.1315 ~ p.1319
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Two Cases of Congenital Atrichial Associated with the Gastrointestinal Anomaly in Siblings
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정진경
백상호/김사영/오의탁/강홍자/김길서
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Abstract
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Congenital atrichia is an anomaly characterized by congenital absence of hair in varying degrees as well as the hypopigmentation & poor development of the abnormal residual hair on the scalp, eyebrows, eyelashes & body.
Two cases of congenital atrichia in siblings were reviewed.
The first cases was associated with congenital megacolon and skin biopsy of scalp (esp. parietal region) was non-specific. The second case was associated with bilateral inguinal hernia.
Congenital atrichia is inherited by autosomal dominant or recessive. Acording to the limited pedigree data, our cases were X-linked recessive inheritance.
We report these cases with a review of related literatures.
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