Metabolic Disorders

Phenylketonuria is an inherited disorder caused by inability to metabolize phenylala-nine secondary to a defect in the enzyme phenylalanine hydroxylase. Affected individuals have fair complexion and blond hair. If untreated, seizures and mental retardation result. Approximately 25% of these individuals have an eczematous-like rash associated with their disease.

Acrodermatits enteropathica is a lethal autosomal recessive disorder with clinical symptoms resulting from profound zinc deficiency secondary to an undefined defect in zinc absorption. The condition is characterized by dermatitis, failure to thrive, diarrhea, alopecia, nail dystrophy, severe gastrointestinal disturbances and frequent infections. Dermatitis lesions are vesciculobullous and are distributed in a symmetrical pattern in the acral and perioral regions. Treatment of choice is elemental zinc replacement.

Celiac disease is a malabsorption disorder secondary to sensitivity to gliadin, the alcohol-soluble portion of gluten found in cereal grains. An eczematous dermatitis, dermatitis herpetiformis, has been reported to occur in some patients. Dermatitis herpetiformis is a highly pruritic skin rash that is characterized by a chronic papulovescicular eruption on the extensor surfaces and buttocks. This disorder is associated with celiac disease in up to 85% of patients. Treatment for celiac disease is life-long dietary avoidance of gluten-containing foods.

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