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Acrodermatitis Enteropathica

Definition


Definition of Acrodermatitis Enteropathica
Acrodermatitis enteropathica is an autosomal recessive metabolic disorder affecting the uptake of zinc, characterized by periorificial (around the natural orifices) and sacral (in the limbs) dermatitis, alopecia (loss of hair), and diarrhoea.

Symptoms


Symptoms of Acrodermatitis Enteropathica
Features of acrodermatitis enteropathica start appearing in the first few months of life, as the infant discontinues breast milk. There are erythematous patches and plaques of dry, scaly skin. The lesions may appear eczematous, or may evolve further into crusted vesicles, bullas or pustules. The lesions are frequent around the mouth and anus, and also in hands, feet and scalp. There may be supportive inflammation of the nail fold surrounding the nail plate - known as paronychia. Alopecia - loss of hair from scalp, eyebrows and eyelashes may occur. The skin lesions may be secondarily infected by bacteria such as Staphylococcus aurous or fungi like Candida albicans. These skin lesions are accompanied by diarrhoea.

Causes


Causes of Acrodermatitis Enteropathica
Similar features may be present in acquired zinc deficiency. This disease also is related to deficiency of zinc due to congenital causes.

Other names for acrodermatitis enteropathica include:

  1. Brandt syndrome
  2. Danbolt-Cross syndrome
  3. Congenital zinc deficiency

Diagnosis


Diagnosis of Acrodermatitis Enteropathica
Consult with your doctor.

Prognosis


Prognosis of Acrodermatitis Enteropathica
Consult with your doctor.

Treatment


Treatment of Acrodermatitis Enteropathica
Without treatment, the disease is fatal and affected individuals may die within a few years. There is no cure for the condition. Treatment includes lifelong dietary zinc supplementation in the range of greater than 1-2 mg/kg of bodyweight per day.

Prevention


Prevention of Acrodermatitis Enteropathica
Consult with your doctor.


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