아토피피부염 환아에서 전신적인 피부 발진의 임상양상을 보인 혈구탐식증후군 1예

Abstract

We report a case of 15-year-old patient, previously diagnosed with atopic dermatitis, who presented with generalized erythematous purpuric patches and petechiae on the face, trunk and extremities. The skin lesions appeared 7 days prior to presentation, accompaied by a high grade fever (38.9oC). The lesions had rapidly become aggrevated after an upper respiratory infection, resulting in severe itching sensation. On physical examination, epatosplenomegly and cervical lymphadenopathy were noted. The laboratory test showed marked pancytopenia, elevated liver enzymes, hypertriglyceridemia, elevated lactic dehydrogenase level and increased ferritin level. Many hemophagocytic macrophages were detected in bone marrow biopsy. A skin biopsy revealed panniculitis composed of histiocytes and lymphocytes. Hemophagocytic syndrome was diagnosed, and treatment with combination chemotherapy resulted in complete remission.