Short answer · Medically reviewed summary · Last updated: 2026-05-08
Acrodermatitis enteropathica is classified under the ICD-10-CM code E83.2 (Disorders of zinc metabolism), while its corresponding ICD-9-CM code is 276.9 (Other disorders of fluid, electrolyte and acid-base balance). These codes are used globally for clinical billing and medical record tracking for patients diagnosed with this rare genetic disorder. What is the clinical nature of Acrodermatitis enteropathica? Acrodermatitis enteropathica is a rare, autosomal recessive metabolic disorder characterized by the body's inability to properly absorb zinc from the diet.
ICD10 code of Acrodermatitis Enteropathica and ICD9 code
ICD-10 and ICD-9 codes for Acrodermatitis Enteropathica, with classification details for clinicians, coders and patients.
Acrodermatitis enteropathica is classified under the ICD-10-CM code E83.2 (Disorders of zinc metabolism), while its corresponding ICD-9-CM code is 276.9 (Other disorders of fluid, electrolyte and acid-base balance). These codes are used globally for clinical billing and medical record tracking for patients diagnosed with this rare genetic disorder.
What is the clinical nature of Acrodermatitis enteropathica?
Acrodermatitis enteropathica is a rare, autosomal recessive metabolic disorder characterized by the body's inability to properly absorb zinc from the diet. This impairment is typically caused by mutations in the SLC39A4 gene, which encodes a zinc transporter protein. Without adequate zinc levels, patients with Acrodermatitis enteropathica frequently experience a triad of classic symptoms: acral and periorificial dermatitis, alopecia (hair loss), and diarrhea.
How is a diagnosis of Acrodermatitis enteropathica confirmed?
Diagnosis is usually suspected based on physical examination and is confirmed through biochemical testing. Medical professionals focus on the following diagnostic criteria for Acrodermatitis enteropathica:
- Measurement of serum or plasma zinc levels, which are typically significantly low.
- Evaluation of alkaline phosphatase activity, a zinc-dependent enzyme that is often depressed in Acrodermatitis enteropathica.
- Genetic testing to identify pathogenic variants in the SLC39A4 gene.
- Clinical observation of skin lesions, which often present as erythematous, vesiculobullous, or psoriasiform rashes around the mouth, eyes, and anus.
Is Acrodermatitis enteropathica a lifelong condition?
Yes, Acrodermatitis enteropathica is a chronic genetic condition that requires lifelong management. The primary treatment involves high-dose oral zinc supplementation, which typically leads to rapid resolution of symptoms within days or weeks. Because Acrodermatitis enteropathica is a metabolic defect, patients must remain under the care of a specialist to monitor zinc levels and adjust dosages throughout their life, particularly during periods of increased metabolic demand like puberty or pregnancy.
Next steps
- Consult with a metabolic specialist or a pediatric dermatologist to manage Acrodermatitis enteropathica.
- Join the DiseaseMaps.org community to connect with others who understand the day-to-day management of rare metabolic disorders.
- Ensure all primary care providers are aware of your diagnosis, as consistent zinc supplementation is vital to preventing recurrent symptoms.
Medical disclaimer: This information is for educational purposes only and does not constitute medical advice; always consult with a qualified healthcare professional regarding your specific diagnosis and treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Acrodermatitis enteropathica.
- Orphanet: ORPHA27 - Acrodermatitis enteropathica.
- OMIM (Online Mendelian Inheritance in Man): Entry #201100 - Acrodermatitis enteropathica.
