A Case of Acrodermatitis Enteropathica with Chronic Diarrhea and Disseminated Intravascular Coagulation

만성 설사와 범발성 혈관내 응고 증후군을 초래한 장병성 선단 피부염 1례

Acrodermatitis enteropathica, an autosomal recessive disease, usually presents with severe acral and circumorificial dermatitis, diarrhea, alopecia, intercurrent bacterial infection during early infancy, and is eventually fatal if left untreated. We report a case of acrodermatitis enteropathica in a 2-month-old male infant who presented with chronic diarrhea not responsive to conventional therapy and developed disseminated intravascular coagulation (DIC). He showed the characteristic eczematoid skin lesions, chronic diarrhea, failure to thrive, and low serum zinc concenturation. $Zn^{2+}$ was administered with dramatic improvement of skin lesions, DIC and diarrhea. He rapidly catched up normal growth and development on continuing zinc supplementation.

저자들은 만성설사와 범발성 혈관내 응고 증후군을 동반한 2개월된 남아에서 장병성 선단 피부염을 의심하여 아연을 경구 투여한 후, 증상호전을 보인 장병성 선단 피부염 환아 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.