RESUMO
Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a rare systemic disorder arising from clonal proliferation of immature CD207-positive (langerin) myeloid dendritic cells (histiocytes) in the skin and visceral organs with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. The incidence of LCH is very less in adult and occurs almost exclusively in children. Genital, perianal, and lung lesions are considered to be rare manifestations of adult LCH. We describe a case of 31-year-old, nonsmoker female who presented in February 2020 with itching and burning sensation in perianal and vulvar regions accompanied with multiple nonhealing ulcers and papillomatous lesions. These lesions gradually increased in size with no response to antibiotics and topical steroids. She was advised positron-emission tomography- computed tomography (PET-CT) scan for further evaluation. After PET-CT scan, her provisional diagnosis of multisystem, multifocal Langerhans cell histiocytosis with high-risk organ involvement was made. Both vulvar and perianal lesions were biopsied which was suggestive of Letterer-Siwe variant of LCH. The prognosis of this variant is very poor even with aggressive chemotherapy and 5-year survival rate of only 50%. Hence, it requires careful consideration during diagnosis and management.
