Acrodermatitis enteropathica
- autosomal
recessive disorder of zinc
transport
- Features:
periorificial and acral dermatitis, alopecia, and diarrhea.
- presents 1-2 months after birth if formula-fed, or
1-2 months after stopping breastfeeding
- In contrast to
Wiscott-Aldrich, the eruption of acrodermatitis enteropathica consists of erythematous, crusted patches around
the mouth and eyes, on
the distal extremities, and in the
perineum. Also distinguish from
Histiocytosis rash.
- Labs*:
- Plasma zinc levels are low, and characteristic
histopathologic
findings are seen on skin biopsy.
- Determining hair, urine, and parotid saliva
zinc levels as well as
serum alkaline
phosphatase activity (which
lowers later in the disease) may be helpful.
- Analysis of maternal breast milk
zinc concentrations may also help in differentiating AE from acquired
zinc deficiency.
- Differentiating AE from acquired zinc deficiencies can be difficult
because both conditions present in the same manner. Some studies have shown
that low zinc levels in the mother's
milk may produce an acquired zinc deficiency in full-term, breastfed infants.
Zimmerman et al has proposed that some
acquired zinc deficiencies may be due to a defect in mammary zinc secretion.
These studies tend to dispute the claim that human breast milk has a
protective effect against zinc deficiency.
Acquired zinc deficiency may also
occur in premature infants, whether or not maternal zinc levels are low or
normal, because of the infants' greater bodily demand or lower bodily stores
of zinc.*
- AE is lethal, usually within the first few years of life, if left
untreated. However, Graves et al reported an untreated adult survivor.*
- see
Vitamin & Mineral Deficiencies
- *E-medicine.com Acrodermatitis Enteropathica Last Updated: July 29, 2003.
http://www.emedicine.com/derm/topic5.htm