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Widespread Absence of the Skin in a Newborn

A n~wb?rn male infant presented to the New York Hospital In March 1996 for evaluation of widespread absence of the skin. This full-term, 3000-g infant was born to a 29-year-old gravida 1, para 0 mother. The infant was the product of a twin gestation (monochorionic, diarnni otic); demise of the second fetus occurred at approximately 10 weeks' gestation. The mother had had appropriate pre natal care. There was no history of maternal illness during the pregnancy, and no medication or drug ingestion was reported. Maternal serologies were negative for hepatitis and syphilis, and there was no history of herpes simplex infection. Prenatal testing was significant for a low alphafetoprotein level by amniocentesis and a normal fetal ultrasound. Delivery of the viable twin was via caesarean section for a breech presentation. APGAR scores were 9 and 10 at 1 and 5 minutes. No gross placental abnormalities or infarcts were noted at birth. Immediately after birth, extensive areas of absent skin were noted and the patient was transferred to The New York Hospital for further management. A degenerated second fetus was delivered. On physical examination, the skin of the scalp was intact and mucous membranes were normal. There were extensive symmetric areas of atrophic fibrotic translucent membranes in a wide H-shaped distribution across the anterior mid-trunk and flanks, with a similar contiguous thinner band extending around the back and under the umbilicus (Fig. 1). The tissue in these areas had a translucent parchment paper-like consistency, and dermal vasculature was prominently visualized (Fig. 2). Smaller stellate atrophic plaques were present symmetrically on the inner elbows and anterior thighs extending to the lateral buttocks (Fig. 3). Granulation tissue was evident at the base of the plaques on the arms and th ighs. There were no blisters, hyperpigmentation, or cutis marmorata. The anterior and posterior fontanels were open, and the palate was intact. There was no evidence of spinal dysraphism, and the limbs were without deformity. Hair and nails were normal. No