Langerhans ’ cell histiocytosis diagnosed due to dermatological perianal lesion

Publication date: Available online 10 May 2017 Source:Journal of Coloproctology Author(s): Bruno Lorenzo Scolaro, Gustavo Becker Pereira, Daniel Cury Ogata, Fernanda Souto Padrón Figueiredo Vieira da Cunha, Ana Cristina Martins Effting, Rafael Oselame Guanabara Langerhans’ cell histiocytosis is a rare disease characterized by proliferation of Langerhans cells in the body. It affects mainly males, predominantly in childhood. Ulcerated plaques are one of the cutaneous forms of presentation. Diagnostic confirmation is done through immunohistochemistry. As therapeutic options, topical corticosteroids and chemotherapy are good choices. The case is reported of a male patient, aged 14, with perianal ulceration. He consulted a coloproctologist, who performed a biopsy of the region and started local triamcinolone applications. Immunohistochemistry diagnosed Langerhans’ cells histiocytosis. Further investigation revealed diabetes insipidus, osteolytic lesions in the skull and lower limbs, enlarged liver, and encephalic alterations. Chemotherapy was started with Vinblastine, with significant improvement of the lesions.
Source: Journal of Coloproctology - Category: Gastroenterology Source Type: research