Pseudodendritic keratitis and systemic tyrosinemia.
Three children (6 eyes) with systemic tyrosinemia who presented with a diagnosis of herpes simplex keratitis are described. Two of the patients underwent extensive treatment for herpes simplex keratitis until their defects in tyrosine metabolism were discovered. All three children responded favorably to a low-tyrosine diet. These cases illustrate the typical corneal findings of the Richner-Hanhart syndrome (pseudodendritic keratitis, hyperkeratotic skin lesions of the palms and sole without mental retardation). The findings in these three patients were similar to those cases previously reported in the literature except none had mental retardation. The ophthalmologist confronted with a young child with bilateral dendritiform keratitis should request evaluation of the serum tyrosine levels.
D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications* / Q000178:diet therapy; D005260:Female; D006801:Humans; D007223:Infant; D007635:Keratitis, Dendritic / Q000178:diet therapy / Q000209:etiology*; D007645:Keratoderma, Palmoplantar / Q000150:complications* / Q000178:diet therapy; D008297:Male; D013577:Syndrome; D014443:Tyrosine / Q000097:blood / Q000378:metabolism*
D014443:Tyrosine