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Restrictive dermopathy is one of the lethal forms of autosomal recessive skin dysplasia. It was first described by Witt et al. in 1986as a syndrome characterized by rigid or thick skin involving the entire body, dysmorphic face, arthrogryposis multiplex and pulmonary hypoplasia. In this work, we report on 2 sisters from consecutive pregnancies affected with restrictive dermopathy. The first baby was stillborn at 32 weeks of gestation to a 29 years old gravida 3, para 0 mother. The birth weight was 1.8 kg and the crown-heel length was 36cm. The second baby was born at 31 weeks of gestation with 1.1kg in weight and 28.0 cm in crown-heel length by cesarean section and died of possible sepsis at 4 days after birth. They had characteristic facies with pinched nose, low set ears, open mouth and micrognathia. The skin of the entire body was taut with prominent scale. Ankylosis was observed at hip, knee, ankle, shoulder, elbow, wrist and interphalangeal joints. Histopathologic examination of the representative skin revealed normal epidermis, dense bands of dermal collagen fibers oriented parallel to epidermis. Elastic fibers were absent in the dermis. We present two cases of restrictive dermopathy that affect 2 sisters from consecutive pregnancies. The neonatal historyand morphologic findings of this disorder are typical. It is characterized by intrauterine growth retardation, tight and rigid skin, dysmorphic face, arthrogryposis and early neonatal death.
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