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Inherited vesiculobullous disorder characterized by intermittent simultaneous occurrence of diarrhea and bullous dermatitis (dry lesions surrounding the mouth, ears, nose, and eyes, but also affecting the fingers, feet, and knees) and failure to thrive in premature babies. In children, periorificial lesions of the face and anogenital region. Alopecia and absence of eyebrows, eyelashes, and thymus are common.

Danbolt-Closs Syndrome Periorificial skin lesions in the face of a toddler.

Danbolt-Closs Syndrome

Extended periorificial dermatitis affecting the anal region and the legs in a small infant.

Danbolt-Closs Syndrome

Acral skin lesions on the toes of a small child.

Acrodermatitis Enteropathica; Zinc-Deficiency Syndrome; Periorificial Dermatitis.

Named after Niels C.G. Danbolt and Karl P. Closs, two Norwegian physicians who described the disease in 1942, although the first description was given by Thore E. Brandt, a Swedish dermatologist, in 1936.

Exact incidence is not known, but estimates are about 2:1,000,000 live births. No racial or sexual predilection has been reported.

Results in decreased synthesis of picolinic acid that causes an impaired ability to utilize zinc from common food. The metabolic defect appears to be related to a cellular defect in zinc metabolism rather than in zinc absorption. However, oral or intravenous administration of zinc rapidly improves the condition. Fibroblasts of these patients contain 60% less zinc than in normal subjects. The immune system also is affected by zinc deficiency, which not only may result in decreased cellular and humoral immune response with increased susceptibility to infections but also may be involved in the association with connective tissue disorders. Zinc concentrations are decreased in the mucosa of the small intestine.

Vesiculobullous and/or pustular disorder caused by a disturbance of intraepidermal or dermal-epidermal adherence with subsequent accumulation of serous fluid within the cavities formed by tissue separation. The disorder develops during the first year of life as periorificial and acral vesiculobullous eruptions, which are followed by alopecia, ungual dystrophy, severe diarrhea, failure to thrive, cachexia, and psychological symptoms such as irritability or apathy. Laboratory tests reveal a significant decrease in zinc serum and tissue levels (caveat: special laboratory tubes may be required because many may contain zinc in the rubber cap or other parts) and a low level of alkaline phosphatases (because it is a zinc metalloenzyme). Be aware that hypoalbuminemia can result in falsely low zinc serum concentrations.

In addition to the inherited form of zinc deficiency, several clinical conditions may result in zinc deficiency, particularly during total parenteral nutrition (TPN) without zinc supplementation. In ...

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