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acrodermatitis enteropathica
acrodermatitis enteropathica[‚a·krō‚dər·mə¦tī·təs ‚en·tə·rə′pa·thə·kə] (medicine) An often fatal inherited disease involving inefficient intestinal absorption of zinc; readily treated by adding zinc to the diet. acrodermatitis enteropathica
acrodermatitis [ak″ro-der″mah-ti´tis] inflammation of the skin of the limbs, especially the hands or feet.chronic atropic acrodermatitis (acrodermatitis chro´nica atro´phicans) 1. chronic inflammation of the skin of the extremities, leading to atrophy.2. a diffuse chronic skin disease usually confined to the limbs, seen mainly in women in Northern, Central, and Eastern Europe, and characterized initially by an erythematous, edematous, pruritic phase followed by sclerosis and atrophy. It is caused by infection with Borrelia burgdorferi. See also lyme disease.acrodermatitis conti´nua (continuous acrodermatitis) chronic inflammation of the skin of the limbs, in some cases becoming generalized.enteropathic acrodermatitis (acrodermatitis enteropa´thica) a hereditary disorder of infancy due to defective zinc uptake, characterized by dermatitis with vesicles and pustules, usually around the mouth or anus and on the head, elbows, knees, hands, and feet; there are also gastrointestinal disturbances such as diarrhea, as well as total alopecia.acrodermatitis per´stans acrodermatitis continua.ac·ro·der·ma·ti·tis en·ter·o·path·'i·ca [MIM*201100] a progressive hereditary defect of zinc metabolism in young children (onset, 3 weeks-18 months); often manifests first as a blistering, oozing, and crusting eruption on an extremity or around one of the orifices of the body, followed by loss of hair and by diarrhea or other gastrointestinal disturbances; relieved by lifelong oral zinc supplementation; autosomal recessive trait.acrodermatitis enteropathica An autosomal recessive MIM 201100 condition characterised by dermatitis—affecting the cheeks, elbows, knees, periorbital and perianal regions, accompanied by hair loss on the scalp, eyebrows and lashes—and diarrhoea. AE is associated with short stature, anaemia, skin hyperpigmentation, hepatosplenomegaly, hypogonadism, poor wound healing, immune deficiency, recurrent bacterial and fungal infections due to immune defect.
Lab
Reduced serum zinc due to impaired uptake.
Management
Oral zinc.acrodermatitis enteropathica Congenital zinc deficiency An AR condition characterized by dermatitis and diarrhea; the dermatitis affects the cheeks, elbows, knees, periorbital and perianal regions, and is accompanied by hair loss on the scalp, eyebrows and lashes; AE is associated with short stature, anemia, skin hyperpigmentation, hepatosplenomegaly, hypogonadism, poor wound healing, immune deficiency, recurrent bacterial and fungal infections due to immune defect Lab ↓ serum zinc due to impaired uptake Management Oral zincac·ro·der·ma·ti·tis en·ter·o·path·i·ca (ak'rō-dĕr'mă-tī'tis en'tĕr-ō-path'i-kă) A progressive hereditary defect of zinc metabolism in young children (onset, 3 weeks-18 months); often manifests first as a blistering, oozing, and crusting eruption on an extremity or around one of the orifices of the body, followed by loss of hair and by diarrhea or other gastrointestinal disturbances; relieved by lifelong oral zinc supplementation; autosomal recessive trait. acrodermatitis enteropathica A rare genetic skin inflammation (DERMATITIS), affecting mainly the extremities, with reddening, ulceration and pustule formation. The condition is caused by the inability to absorb zinc from the diet and can be treated by zinc supplements.ac·ro·der·ma·ti·tis en·ter·o·path·i·ca (ak'rō-dĕr'mă-tī'tis en'tĕr-ō-path'i-kă) [MIM*201100] Progressive hereditary defect of zinc metabolism in young children; often manifests first as a blistering, oozing, and crusting eruption on a limb or around an orifice; relieved by lifelong oral zinc supplementation. AcronymsSeeAE |