Two Cases of Congenital Atrichia Associated with the Gastrointestinal Anomaly in Siblings

ABSTRACT

Congenital atrichia is an anomaly characterized by congenital absence of hair in varying degrees as well as the ypopigmentation & poor development of the abnormal residual hair on the scalp, eyebrows, eyelashes & body. Two cases of congenital atrichia in siblings were reviewed. The first case 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.