Acrodermatitis chronica atrophicans

General: Rare familial skin disorder; autosomal recessive; both sexes equally affected; occurs in infants; not present at birth but develops during first few weeks; zinc deficiency; there are reports of improvement following treatment with zinc suggesting an abnormality in the zinc-binding factor.

Ocular: Scarring of the conjunctiva; recurrent iridocyclitis; keratomalacia; cataracts; photophobia; blepharitis; punctal stenosis; corneal opacification.

Clinical: Vesiculobullous eruption around body orifices, skin of knees, elbows, and paronychial areas; complete alopecia; erythematous psoriasiform plaques.

Feldberg R, et al. Keratomalacia in acrodermatitis enteropathica (AE). Metab Pediatr Ophthalmol 1981; 5:207-211.

Matta CS, et al. Eye manifestations in acrodermatitis enteropathica. Arch Ophthalmol 1975; 93:140-l42.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: https://www.ncbi.nlm.nih.gov/omim/.

Ohlsson A. Acrodermatitis enteropathica: reversibility of cerebral atrophy with zinc therapy. Acta Paediatr Scand 1981; 70:269-273.

Racz P, et al. Bilateral cataract in acrodermatitis enteropathica. J Pediatr Ophthalmol Strabismus 1979; 16:180-l82.

Whitmore PV. Skin and mucous membrane disorders. In: Duane TD, ed. Clinical Ophthalmology, vol. V. Philadelphia: JB Lippincott, 1982:6-7.