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Langerhans cell histiocytosis in a patient with perianal lesion. A case report
Nicolás A Breppe1, Paula Gaviot1, Darío O Rodríguez1
1Department of Pediatrics of Establecimiento Asistencial Dr. Lucio Molas, Santa Rosa, Argentina.
Insights
Langerhans cell histiocytosis, a myeloid dendritic cell disorder, can affect multiple organs. This case highlights a rare presentation in a 5-year-old boy with perianal, lung, and ear involvement, emphasizing an interdisciplinary diagnostic approach.
Area of Science:
- Pediatric Pathology
- Immunology
- Dermatology
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal myeloid neoplasm.
- It involves the abnormal proliferation of Langerhans cells, a type of dendritic cell.
- LCH typically presents in young children with diverse systemic manifestations.
Abstract:
Langerhans cell histiocytosis is an expression of myeloid dendritic cells, associated with a significant inflammatory component and varied systemic involvement. The most common age at presentation is between 1 and 4 years, and it prevails among male subjects. Here we describe the case of a 5-year-old boy who presented with a granulomatous lesion with perianal fistula and lung and external ear involvement. An interdisciplinary approach helped to make a diagnosis, provide the necessary interventions, and start an adequate treatment.
