Brandt syndrome, Dan bolt-Cross syndrome, Congenital zinc deficiency
Acrodermatitis enteropathica is due to malabsorption of zinc by the intestinal cells. The exact cause is not known but it may relate to mutations in a gene (SLC39A4) that codes the zinc transporter protein, ZIP4. It is considered that the missing protein may be responsible for decreased zinc uptake and abnormal zinc metabolism.
Without treatment, the disease is lethal and affected individuals may die within a few years. There is no cure for the situation. Treatment includes lifelong dietary zinc supplementation in the range of greater than 1-2 mg/kg of bodyweight per day..
Acrodermatitis enteropathica is an autosomal recessive metabolic confusion affecting the uptake of zinc, characterized by periorificial (around the natural orifices) and acral (in the limbs) dermatitis, alopecia (loss of hair), and diarrhea.