Congenital self-healing reticulohistiocytosis is the benign spectrum of Langerhans Cell Histiocytosis, characterized by cutaneous lesions at birth or in the neonatal period, absence of systemic manifestations and spontaneous resolution of clinical status. Despite the benign and often self-resolving course in most patients, studies show that in some cases there may be metastasis or recurrence of the disease, emphasizing that the clinical course is variable, requiring long-term follow-up. The monitoring of the patient for a long period is important to detect possible systemic involvement, as there is a report of recurrence involving the skin, mucosa, bone and pituitary gland. Keywords: Histiocytosis; Histiocytosis, Langerhans-Cell; Histiocytosis, Non-Langerhans-CellResumo: A retículo-histiocitose congênita autolimitada é o espectro benigno das histiocitoses de célu-las de Langerhans, caracterizada pela presença de lesões cutâneas ao nascimento ou no período neonatal, ausência de manifestações sistêmicas e resolução espontânea do quadro clínico. Apesar do curso benigno e frequente autorresolução na maior parte dos pacientes, estudos mostram que, em alguns casos, pode haver disseminação ou recaída da doença, enfatizando que o curso clínico é variável, havendo necessidade de seguimento em longo prazo. O acompanhamento do paciente por longo período é importante para detectar possível envolvimento sistêmico, pois existe relato de recorrência, envolvendo pele, mucosa, ossos e glândula pituitária. Palavras-chave: Histiocitose; Histiocitose de células de Langerhans; Histiocitose de células não Langerhans
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