Random Medical and Weird I Find ALWAYS UPDATED * child warning

cheekymarls · #**771** ● 11-08-2014, 04:49 PM

Introduction
Aplasia cutis congenita (ACC) is a condition characterized by the absence of skin at birth. The defect can involve a small portion of the scalp or widespread areas. The most commonly involved site is the scalp. There is no racial or sex predilection. It is classified into nine groups based on the etiology and the clinical findings. Our patient suffered from isolated extensive ACC and was exposed in utero to both, azathioprine, and prednisolone. Azathioprine has been held responsible for an increase in congenital malformations although not specifically ACC and prednisolone is regarded safe in pregnancy.

Case Report
A new born baby boy was seen in the neonatal Intensive Care Unit (ICU) just after birth, who had almost complete absence of the skin except for some area around the nose (Figure-1) and on the buttocks (Figure-2) where skin had formed. The entire body was covered in a membrane which had numerous vessels visible on its surface. He had no nails or hair and nipples were not formed (Figure-3). He had micrognathia, his ears were malformed and his right testis was undescended. He was born with a very low birth weight 1.02 kg, fronto-occipital circumference was 26.5 cm (normal range 33- 38 cm) and crown heel length was 37 cm (normal range 48- 53cm). He was nursed in an incubator but died on the 4th day of birth of neonatal sepsis in spite of supportive management, including antibiotic therapy.

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He was born to non consanguineous parents by lower segment caesarian section at 38 weeks of gestation because the baby showed signs of intrauterine growth retardation. The mother had a history of two previous c-sections. Four other children were alive and healthy. There was no history of chicken pox or herpes zoster during pregnancy. The mother who was 26 years of age had a history of pemphigus vulgaris for the past 1 year, confirmed on histopathology and direct immunoflourescence. Her disease had been particularly difficult to control and she had been on tablet prednisolone 60 mg daily for the first 2 months of pregnancy. Gradual reduction was attempted but she developed fresh blisters when the dose was reduced below 30 mg daily, so 30mg/day had to be continued for the rest of her pregnancy. In addition, to prednisolone she was on azathioprine 150 mg daily (3mg/kg body weight) 6 weeks prior to and for the first 20 weeks of pregnancy. After delivery of the baby the dose of prednisolone was reduced gradually and her disease was controlled on 15 mg of prednisolone on alternate days