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Acrodermatitis enteropathica-like rash and enterocolitis


European Journal of Dermatology. Volume 8, Number 6, 445-6, September 1998, Votre diagnostic ?

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Author(s) : Florence GRANEL, Annick BARBAUD, Sophie REICHERT, Jean-Luc SCHMUTZ

Summary : A premature, 1-month-old infant, with neonatal enterocolitis and receiving parenteral nutrition, developed an erosive, erythematous rash, with multiple blisters spreading to the face (Fig. 1), genital region and thighs (Fig. 2). In addition to the rash, the baby had a fever and was irritable. The baby's white blood cell count was 15,800/mm3, and the C-reactive protein level was 85 g/dl. Bacterial culture of the blood isolated Staphylococcus aureus which was methicilline resistant, and a mycologic culture of urine and stool isolated Candida parapsilosis. Bacterial cultures of the central and umbilical catheter, the throat and gastric liquid were negative. A skin biopsy was taken and histological examination revealed epidermal hyperkeratosis with parakeratosis and a dermal infiltrate of mononuclear cells (Fig. 3). Direct immunofluorescence of the skin biopsy was negative. The rash failed to respond to topical antibacterial cream (fusidic acid) and systemic antibacterial treatments (vancomycin, amikacin). The antifungal fluconazole was added intravenously and oral nutritional supplements were begun. What is your diagnosis?

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