Harlequin baby--a rare case of keratinization disorder

Nepali, N.; Makaju, R.K.; Sharma, N.; Bedi, T.R.S.; Chawla, C.D.

Nepal Medical College Journal Nmcj 7(2): 148-149

2005


ISSN/ISBN: 2676-1319
PMID: 16519086
Document Number: 594521
Harlequin icthyosis is a very rare inborn error of epidermal keratinization with autosomal recessive inheritance. Abnormal lipid metabolism in mitochondria with defective lamellar body formation is the main defect leading to hyperkeratosis. Prenatal diagnosis can be done by invasive procedures such as fetal skin biopsy and also by ultrasonography.

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