Tight, shiny membrane encases newborn’s skin
On physical examination in the delivery room, the neonate was vigorous with normal vital signs. Apgar scores were 8 and 9 at 1 and 5 minutes of life. Detailed examination on admission to the neonatal intensive care unit (NICU) showed that in addition to the skin findings, she also had ectropion, eclabium, malformed ears, and contractures of the extremities (Figure 2). The patient’s abdominal and cardiac exams were normal. A clinical diagnosis of ichthyosis with a collodion membrane was made.
The patient was placed in an isolette with a relatively high humidity because of concern for transepidermal water loss. An umbilical venous catheter (UVC) was inserted for administration of fluid and medication as needed. A post-UVC insertion abdominal x-ray done to confirm position of the UVC revealed a double “bubble” appearance indicating a clinical diagnosis of duodenal atresia (Figure 3). An immediate consultation with Pediatric Surgery was done for evaluation and repair of the duodenal atresia. Dermatology was consulted at day 1 of life to help with skin management.
Generalized scaling and thickening of the skin can be caused by a variety of disorders (Table). These could be congenital or acquired. Congenital Ichthyosis could be associated with collodion membrane as seen in this patient.1 These conditions include lamellar ichthyosis (LI), harlequin ichthyosis, congenital ichthyosiform erythroderma, Sjögren-Larssen syndrome, Netherton syndrome, and epidermolytic hyperkeratosis.2,3
Other causes of diffuse scaling include nutritional deficiencies that are usually acquired such as acrodermatitis enteropathica, which is associated with zinc deficiency; hypohidrotic ectodermal dysplasia; metabolic disease such as Gaucher disease; neutral lipid storage disease; adverse effects of some medications; and asteatotic dermatitis and eczema.4,5
Unlike generalized scaling of the skin that has varying causes, the radiographic finding of double bubble appearance is pathognomonic of duodenal atresia, which requires prompt surgical intervention.