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| Fig. 40.4 Acrodermatitis enteropathica. A, Periorificial dermatitis in a young patient with acrodermatitis enteropathica. B, Acral dermatitis in a young patient with characteristic involvement around the nails. (Courtesy of James E. Fitzpatrick, MD.) |
Acrodermatitis enteropathica is a rare autosomal recessive disorder of intestinal zinc absorption. Zinc is normally incorporated into multiple types of enzymes present in all body tissues but is concentrated five- to sixfold in the epidermis. Deficiency of this trace element results in dramatic findings. The classic triad consists of acral dermatitis, alopecia, and diarrhea. Growth failure, anemia, impaired wound healing, and mental and emotional disturbances are also seen. The periorificial and acral dermatitis (Fig. 40-4) was considered to be pathognomonic in the past, but more recent reports have described similar cutaneous eruptions in essential fatty acid deficiency, biotin deficiency, and infants treated for organic acidurias. Acrodermatitis enteropathica develops in days to weeks after birth in bottle-fed infants and shortly after weaning in breastfed infants. Uncommonly, signs and symptoms are first noted at puberty.
Maverakis E, Fung MA, Lynch PJ, et al: Acrodermatitis enteropathica and an overview of zinc metabolism,
J Am Acad Dermatol 56:116–124, 2007.
