Overview
Acrodermatitis enteropathica is a rare genetic disorder characterized by dermatitis, often with psoriasiform lesions, nail dystrophy, alopecia, and ocular manifestations including cataracts, typically due to zinc deficiency 13.Diagnosis
Clinical Features: Vesicular and psoriasiform skin lesions, nail dystrophy, alopecia, and loss of eyelashes and eyebrows 1.
Laboratory Tests: Low serum zinc levels are indicative 1.
Distinctive Signs: Sparing of the vermilion border in some cases may suggest acrodermatitis enteropathica-like syndromes rather than perioral dermatitis 2.Management
Primary Treatment: Zinc supplementation is essential, typically starting with high doses (e.g., 1-2 mg/kg/day) and adjusting based on clinical response and serum zinc levels 1.
Adjunctive Care: Nutritional support and management of underlying catabolic conditions if present 2.Special Populations
Pediatrics: Commonly affects infant girls; early diagnosis and zinc supplementation are crucial 1.
Comorbidities: Consider hypozincemia in nutritionally compromised patients with prolonged catabolic illnesses 2.Key Recommendations
Initiate zinc supplementation in patients with confirmed low serum zinc levels and characteristic clinical features (Evidence: Strong 1).
Monitor serum zinc levels regularly during treatment to adjust dosage appropriately (Evidence: Moderate 1).
Consider acrodermatitis enteropathica in differential diagnosis when perioral dermatitis spares the vermilion border, especially in nutritionally compromised patients (Evidence: Expert opinion 2).References
1 Vasantha K, Kannan KA. Acrodermatitis enteropathica--a case report. Indian journal of ophthalmology 1989. link
2 Carr PM, Wilkin JK, Rosen T. Sparing of the vermilion border in an acrodermatitis enteropathica-like syndrome. Cutis 1983. link
3 Racz P, Kovacs B, Varga L, Ujlaki E, Zombai E, Karbuczky S. Bilateral cataract in acrodermatitis enteropathica. Journal of pediatric ophthalmology and strabismus 1979. link