History: This 4 month old female was seen because of a diaper rash and irritability of approximately 8 weeks duration. She was the product of an uneventful pregnancy and normal delivery. The mother indicated that the patient had a rash that started in the diaper area and subsequently spread to the thighs, ankles, wrists, and the perioral area. The dermatitis was associated with loose, watery stools, irritability, and a 200 gm weight loss over the month preceding presentation. She had abeen at the 50 th percentile of the growth curve for weight and length until approximately 2 mos prior to presentation, at which time she started to drop to the 5 th percentile. She had been breast fed q 2-3 hrs ad lib. The rash was unresponsive to treatment with Mycolog and to Westcort creams prescribed by the family physician.
Physical Examination revealed an erythematous slightly scaly eruption shown in Figures A-D. The face and extremeties were markedly edematous (Figures C, D).
Laboratory studies revealed a hypochromic, microcytic anemia ( Hgb 6.0 mg/dL, Hct 18%) with a reticulocyte count of 2.2. CBC and urinalysis were normal. Liver function tests were normal except for a decreased total protein of 2.8 g/dL and an albumin of 1.5 g/dL. The PT was 13.8 s (normal 10.5-12.5) and PTT 26.8 s (normal 23.5-29). The serum vitamin A and E levels were normal. The sweat chloride was 96 mmol/L and on repeat 88 mmol/L. Chest X-ray showed hyperexpansion without infiltrate and a normal cardiac shadow. Pulmonary function testis revealed hyperinflation with normal flows pre- and post bronchodilators.
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