Case Study

This is 4 month old boy, born at 30 weeks gestation due to premature rupture of membranes. He presents with a 2 month history of well-demarcated, erosive, vivid red plaques on face and buttocks. The mother described the appearance of "blisters".

Diagnosis:
Acrodermatitis Enteropathica

Discussion
This is a classic example of a rare but fascinating disease. The clinical presentation of prematurity, exclusive breast feeding, and striking clinical signs and symptoms should suggest the diagnosis. Laboratory information later revealed a markedly decreased serum zinc level at 0.11microgram/ml (range is 0.66 - 1.10 microgram/ml) and decreased alkaline phosphatase in the infant.

The biopsy showed typical changes of the disease, with broad areas of parakeratosis with a diminished granular layer associated with pallor in the superficial keratinocytes. These changes are more typical of early lesions. With progression of the disease, confluenct necrosis with subcorneal or intraepidermal clefts may occur.

Zinc supplementation has led to complete resolution of the lesions.

Acknowledgments
My thanks to Dr. Linda Golkar, Pediatric Dermatologist at Loma Linda University, for the clinical photos and history.

References:
The Doctor's Doctor-Acrodermatitis Enteropathica