Acrodermatite enteropática: relato de caso em criança com doença de Crohn

Abstract

OBJECTIVES: Acrodermatitis Enteropathica (AE) is a rare disease that can be transmitted by autosomal recessive inheritance, it can be related to parenteral nutrition without zinc, or it can be secondary to malabsorptive syndromes. Clinically, it is characterized by the triad of acral and periorificial dermatitis, alopecia and diarrhea, accompanied by growth restriction. The condition can be fatal if left untreated. METHODS: We describe the case of a child with AE and Crohns Disease (CD), with AE being a known complication, secondary to malabsorption caused by CD. RESULTS: Treatment for CD was started with systemic steroids, Azathioprine and Mesalazine, and local care with the diaper area. Oral zinc replacement was also started. The child showed significant clinical and dermatological improvement within a few weeks. CONCLUSIONS: The diagnosis of AE is made with serum zinc levels lower than 70 ug/dL. Treatment involves zinc supplementation, at a dose ranging from 1 to 3 mg/kg/day. In the case described, the zinc value was 65 ug/dL. In this case, we started replacement with 2mg/kg/day, with rapid clinical improvement. Zinc deficiency AE is a well-known complication of CD, secondary to malabsorption. In the case described, the cutaneous condition came after the intestinal condition, with the deficiency in the absorption of zinc being secondary to Crohns Disease, leading to a condition with extensive lesions, but with a quick response to the treatment and with an important improvement of the condition.

Keywords

• acrodermatitis
• crohn disease
• zinc deficiency