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February 1969

Picture of the Month

Author Affiliations

Boston; Uppsala, Sweden
From the Department of Pediatrics, University Hospital, Uppsala, Sweden.

Am J Dis Child. 1969;117(2):201-202. doi:10.1001/archpedi.1969.02100030203015

Denouement and Discussion 

Acrodermatitis Enteropathica  (Danbolt-Closs Syndrome)

Manifestations  Acrodermatitis enteropathica usually manifests itself in infancy, shortly after the child has been weaned or has been started on supplementary formula. It is characterized by a symmetrical, erythematous, and vesiculopustular dermatitis localized to the extremities and areas around the body orifices, diarrhea, and alopecia. Additional manifestations include blepharitis, conjunctivitis, corneal opacities, photophobia, stomatitis, glossitis, hoarsness, paronychiae with dystrophy of the nails, failure to thrive, apathy, irritability, and emotional disturbances.Without treatment the disease may be progressive. Short remissions are followed by relapses which are increasingly severe and end fatally. Spontaneous healing has been reported in relation to onset of puberty.

Genetics  Acrodermatitis enteropathica appears to be inherited as an autosomal recessive trait.

Treatment  Derivatives of halogen-substituted 8-hydroxy-quinolines (Diodoquin, Entero-Vioform, or Sterosan) are the drugs of choice. Initially a high dosage is often needed for effective treatment, but it is important to determine

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