CONRADI-HUNERMANN-SYNDROME WITH UNILATERAL DISTRIBUTION

Conradi-Hunermann syndrome is a type of chondrodysplasia punctata char acterized by skeletal, cutaneous, and ocular anomalies. Genetic hetero geneity and incomplete penetrance may explain the wide clinical spectr um. We report a 7-day-old girl, product of a preterm pregnancy and del ivery, with ichthyosiform erythoderma on the right half of the body at birth, as well as patchy cutaneous involvement of the contralateral s ide. On physical examination, we observed an ipsilateral shortening of the leg and a lenticular opacity of the right eye, Histopathologic st udy showed parakeratotic hyperkeratosis with prominent follicular invo lvement and atrophy of the granular and spinous layers. The skin erupt ion disappeared during the first 2 months, At 2 months of age, radiolo gic examination revealed stippled calcifications in the ribs, vertebra l, and paravertebral areas. The most important differential diagnosis was CHILD syndrome. We emphasize the importance of the histopathologic study in the differential diagnosis of both syndromes.