Acrodermatitis enteropathica with immune deficiency

A male infant presented with alopecia, eczematoid dermatitis, chronic diarrhea, and failure to thrive. The usual causes of chronic malabsorption were ruled out. Acrodermatitis enteropathica was diagnosed. A trial of oral Diodoquin and breast milk as the only source of nutrition resulted in rapid amelioration of symptoms. The patient acquired secondary diarrhea and secondary infections with enteropathogenic Escherichia coli , Candida, and Pseudomonas aeruginosa and subsequently died of sepsis. Studies of serum and tears revealed a marked deficiency of immunoglobulins. Necropsy demonstrated a generalized defect in his immunopoietic tissues. There were low levels of the essential fatty acids in serum. The observations in this case suggest that acrodermatitis enteropathica may in some instances be the clinical expression of a primary immune disorder.