Histiocitosis de células de Langerhans con compromiso vertebral

Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare disease, more common in the first three years of life. It is characterized by single or multiple osteolytic lesions due to clonal proliferation of cells histologically similar to Langerhans cells; its clinical presentation is heterogeneous. Case report: 7-year-old female patient with 5 days of progressive lower extremity weakness and difficulty to walk. Physical exam findings were consistent with pyramidal syndrome and lower extremities hypoesthesia. Magnetic resonance imaging (MRI) of spine and cranial computed tomography (CT) were performed. Intracranial pathology was ruled out. The MRI findings showed vertebra plana with epidural and paravertebral involvement, so treatment with steroids and surgical decompression initiated. Partial resection and biopsy of the lesion was performed. Due to histological findings and positive CD1a and CD207 markers, diagnosis of LCH was confirmed. Conclusions: LCH is an uncommon disease with a challenging diagnosis due to its heterogeneous clinical presentation. Eosinophilic granuloma and vertebra plana as imaging findings may guide the diagnosis. However, it should always be confirmed with histological evidence.