In the literature six cases of acrodermatitis enteropathica (AE) are described in which the patients survived to adult age. In a few of these cases the symptoms disappeared at the onset of puberty, while in the rest the disease had a fluctuating course with long symptom-free intervals and an uncharacteristic symptomatology. Two cases are reported. In case 1 there remained only characteristic skin changes on the feet at adult age, in case 2 both skin and intestinal symptoms disappeared at adult age. For long periods this patient was invalidated through depressions, a symptom which is characteristic of children suffering from an active AE. The same patient developed parkinsonism at a relatively young age. In both cases a very low serum zinc level essentially supported the diagnosis. It is possible that AE in adults is underdiagnosed because of an uncharacteristic symptomatology.
A 36-year-old woman developed vitiligo in areas with active dermatitis herpetiformis. Both diseases have been associated with autoimmune disorders. However, in the present case the vitiligo is believed to be an isomorphic reaction to the dermatitis herpetiformis lesions.
Three cases of acrodermatitis enteropathica (a.e.) from two nonrelated families are described. Two siblings had characteristic symptoms of a.e. in childhood. Both survived to adulthood without treatment, at which time the clinical picture became uncharacteristic of a.e. Even so, the serum zinc levels confirmed the diagnosis in both cases. The third case showed classic symptoms of a.e.; the patient had a greatly reduced serum zinc level and responded at once to treatment with zinc sulphate. Heterozygous carriers of the gene for a.e. have often had slightly reduced serum zinc levels. The value of this test could probably be improved by correcting the normal range of serum zinc for factors known to influence this, such as the patient's age and serum albumin level. The normal range ought also to be corrected for diurnal and postprandial variations of serum zinc.
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