A case of Langerhans cell histiocytosis with a soft tissue lesion and an atypical clinical course

Abstract

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of abnormal Langerhans-like cells. Bone is the most commonly involved site, while isolated soft tissue lesion involvement is rare. We report a case of a 5-year-old girl diagnosed as having multisystem LCH with thoracic spine lesions, a pleural lesion, and multiple soft tissue lesions at the posterior neck, which lacked continuity to any bone lesion. After administration of antibiotics because of a suspicion of subcutaneous abscess and purulent spondylitis, fever, and posterior neck swelling were ameliorated. Because her symptoms were ameliorated only with antibiotics, we were unable to obtain her parents’ consent to administer anticancer chemotherapy. LCH recurred twice in the following 9 months, and each time, her symptoms were ameliorated by the administration of antibiotics alone. Finally, she received chemotherapy and attained complete remission that lasted for four years. This case may be important for understanding the complex nature of LCH, considering the rareness of a soft tissue lesion and the temporary efficacy of antibiotics.