LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS MOUTH OPENING LIMITATION IN A PEDIATRIC PATIENT

Abstract

Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm, with mutations in the BRAF and MAP2K1 genes. LCH often affects pediatric patients, not uncommonly in the head and neck region. Currently, this disease is classified into 4 categories. Pediatric patients with involvement of the liver, spleen, lung, or bone marrow belong to the high-risk group, with high mortality rates. A 13-year-old male patient was referred presenting facial asymmetry and mouth opening limitation. Imaginologic analysis showed an osteolytic lesion affecting the right side of the mandibular angle. After incisional biopsy, microscopy revealed LCH, confirmed by immunopositivity for S-100, CD1a, and CD207. The patient gradually recovered mouth opening. Uncommonly, LCH can present initially as mouth opening limitation, which should be recognized to establish the appropriate treatment for good prognosis in pediatric patients. Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm, with mutations in the BRAF and MAP2K1 genes. LCH often affects pediatric patients, not uncommonly in the head and neck region. Currently, this disease is classified into 4 categories. Pediatric patients with involvement of the liver, spleen, lung, or bone marrow belong to the high-risk group, with high mortality rates. A 13-year-old male patient was referred presenting facial asymmetry and mouth opening limitation. Imaginologic analysis showed an osteolytic lesion affecting the right side of the mandibular angle. After incisional biopsy, microscopy revealed LCH, confirmed by immunopositivity for S-100, CD1a, and CD207. The patient gradually recovered mouth opening. Uncommonly, LCH can present initially as mouth opening limitation, which should be recognized to establish the appropriate treatment for good prognosis in pediatric patients.