Untreated Acrodermatitis Enteropathica in Adults

Abstract

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.

Evidence supporting zinc as an important antioxidant for skin

“…Smell and taste dysfunction, mood changes, and cognitive impairment are subsequent neuropsychiatric manifestations of acute zinc loss 9,14 . Indeed some adults with acrodermatitis enteropathica have been misdiagnosed with psychiatric illnesses described as “schizoid” and “depressive” 15,16 . Likewise, zinc‐deficient infants are irritable and difficult to console.…”

Acrodermatitis enteropathica

“…The latter patients need not represent a disease variant; their plasma zinc concentrations may reflect maintenance of the relatively small plasma zinc pool by release of the element during turnover of peripheral tissues. This possibility is illustrated by an untreated case whose plasma zinc concentration returned to normal following a laparotomy (Olholm-Larsen, 1978).…”

Acrodermatitis enteropathica - lifelong follow-up and zinc monitoring

“…In such cases chronic zinc deficiency may go unrecognized as the dermatological manifestations may be mild. The consequences of their long‐term zinc deficiency may, however, include significant neurological and neuropsychiatric sequelae 2, 3 …”

“…We would certainly be concerned to maintain her serum zinc within the normal range during a pregnancy as there is evidence to suggest that zinc deficiency may be teratogenic 5 . It is important to maintain her zinc level within the normal range, in any case, because chronic zinc deficiency in adults may contribute to disturbances of the central nervous system such as cerebellar disease, parkinsonism and cortical atrophy 2, 3 …”

“…SIR, Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder, recently mapped to chromosomal region 8q24. 3,1 in which there is impaired absorption of zinc from the small intestine. Very little information is available on the long-term prognosis.…”