ANAIS DO XXV WORLD CONGRESS OF NEUROLOGY
EP1221 / #3660 LANGERHANS CELL HISTIOCYTOSIS: CASE REPORT Mariane Ferreira, Letícia Rigoti, Carlos Rodrigues, Eduardo Pessoa Júnior, Caroline Rincolato, Gustavo Bauer, Eloisa Neres, Leomara De Oliveira, Kevin Zilnyk, Marina Coronetti, Jaisla Colombi, Caio Goulart, Eugênio Da Silva, Fernanda Nacke, Luana Massarotto Universidade Estadual do Oeste do Paraná, Ccs Centro De Ciências Da Saúde, Francisco Beltrao, Brazil Background and Aims:: Langerhans Cell Histiocytosis (LCH), is characterized by expansive and erosive accumulations of Langerhans cells, which can occur in single or multiple tissues. The disease is rare, identified in approximately 0.5 per 100,000 children up to 2 years of age, shows a diverse clinical presentation, pathological commitment, and outcomes. Those factors make diagnosis and treatment difficult in LCH. Methods:: We describe a case of Histiocytosis X in a male patient, aged 1 year and 6 months, with normal weight, height, and neuropsychomotor development for his age, who presented a tumor in the right temporal region and erythematous-squamous skin lesions with yellowish color on the scalp and trunk since his four months of age. Results:: First, cranial Computed Tomography (CT) and temporal lesion biopsy were requested. The cranial CT revealed intense osteoclastic activity. Histopathological examination suggested LCH, confirmed by an Immunohistochemistry test that demonstrated CD1a-positive and S100-positive. To investigate systemic LCH, chest, abdomen, pelvis, and myelogram radiographs were requested, all without visible alterations. In addition, the patient's whole-body bone scintigraphy was requested, which identified multiple osteogenic reactions. The research proceeded with bone marrow biopsy, which showed infiltration by LCH, concluding the diagnosis of multisystem LCH. The patient had a favorable evolution to corticosteroid therapy associated with chemotherapy and has been monitored annually for recurrence research, the segment is maintained indefinitely. Conclusions:: The case reported is notorious since its initial symptoms were discrete, with a single temporal tumor and skin lesions. This patients’ LCH diagnosis occurred early, however, late diagnosis is common.
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