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.
Periorificial skin lesions in the face of a toddler.
Extended periorificial dermatitis affecting the anal region and the legs
in a small infant.
Acral skin lesions on the toes of a small child.
Acrodermatitis Enteropathica; Zinc-Deficiency Syndrome;
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
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 ...