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Acrodermatitis enteropathico. Abnormalities of fat metabolism and integumental ultrastructures in infants
Insights
Acrodermatitis enteropathica, a childhood disease, involves skin issues and diarrhea. Treatment with diiodohydroxyquin improved patients, suggesting linoleic acid
Area of Science:
- Pediatric Dermatology
- Nutritional Science
- Biochemistry
Background:
- Acrodermatitis enteropathica is a rare genetic disorder affecting children.
- It presents with characteristic skin lesions, diarrhea, and hair loss.
Observation:
- Two patients with acrodermatitis enteropathica showed abnormal fat distribution despite adequate linoleic acid intake.
- Skin biopsies revealed increased lipid droplets in epidermal cells.
Findings:
- Treatment with diiodohydroxyquin (Diodoquin) led to clinical improvement in both patients.
- Serum linoleic acid levels increased and correlated with improved health in the more severely affected child.
- Skin linoleic acid levels were found to be decreased.
Implications:
- Linoleic acid and zinc deficiency may play a role in the pathogenesis of acrodermatitis enteropathica.
- Monitoring both serum and skin linoleic acid levels could aid in evaluating disease progression.
- Further research into the metabolic pathways of essential fatty acids in this condition is warranted.
Abstract:
Acrodermatitis enteropathica is a rare disease of childhood, characterized by periorificial and perioral dermatitis, acrodermatitis, diarrhea, and alopecia. Two patients were successfully treated with diiodohydroxyquin (Diodoquin). Lipid concentrations disclosed abnormal distribution of fats that resembled those of less mature infants fed diets low in linoleic acid even though the patients were actually on diets with high linoleic acid levels. Fatty acid distribution showed increased levels of linoleic acid that correlated with clinical improvement in the patient who was more ill. Skin fatty acids showed decreased levels of linoleic acid. Electron micrographs and histochemical staining disclosed increased lipids droplets in epidermal cells. Linoleic acid and zinc may have a role in the origin of the disease, and measurement of both skin and serum linoleic acid is suggested in the evaluation of its progress.