Acquired acrodermatitis enteropathica: case report of an atypical presentation

Abstract

Acrodermatitis enteropathica (ADE) is a rare genetic or acquired disorder of hypozincemia. It can be caused by impaired intestinal absorption of zinc or by poor consumption of the mineral. It is characterized by skin lesions on acral and periorificial areas and may be associated to alopecia, diarrhea and increased frequency of infections. We present an atypical presentation of ADE in a 33-year-old women with a history of mental retardation and psoriasis that presented with lesions on the periorificial areas and extremities, and low plasma zinc levels.

Acrodermatitis dysmetabolica with concomitant acquired acrodermatitis enteropathica in a patient with maple syrup urine disease

“… 4 , 6 AD and AE share the histopathologic feature of keratinocyte pallor in the upper epidermis. 4 , 7 , 8 Although biopsy can be useful, it is nonspecific and can be seen with other nutritional deficiencies, such as necrolytic migratory erythema and pellagra. Therefore, diagnosis heavily relies on clinical history, physical examination, and laboratory studies.…”

A Case of Zinc Deficiency Histologically Showing Spongiform Pustules of Kogoj

“…In cases of chronic zinc deficiency, there are psoriasis-like histopathological changes to the epidermis (4). However, only one case of neutrophilic microabscesses in the stratum corneum related to zinc deficiency has been reported so far (5). Interestingly, plasma zinc may be decreased in generalized pustular psoriasis (6) or erosive pustular dermatitis of the leg (7).…”

Bullous acrodermatitis enteropathica with interface dermatitis

“…The most relevant clue to ADE diagnosis is keratinocyte pallor in the upper part of the epidermis, which has also been found in other nutritional deficiencies 2,3 . Other characteristics of ADE include confluent parakeratosis, psoriaform hyperplasia, dermal edema, neutrophilic microabscesses, and perivascular lymphohistiocytic infiltration 3,4 . Bullous ADE has been characterized histopathologically by coalesced intraepidermal vesiculation on a background of eosinophilic necrotic keratinocytes with scant spongiosis, and a predominantly lymphoneutrophilic infiltrate within the vesicles 5,6 .…”