Abstract

Langerhans cell histiocytosis (LCH) is a rare clonal proliferation disorder that has an estimated incidence of 3 - 5 per million in children and 1 - 2 per million in adults. It is documented more commonly in children (1-3 years). Primary LCH of the female genital tract with isolated cutaneous vulvar involvement is reported in less than 20 cases. The prognosis is dependent on the disease extent. While unifocal LCH follows a good prognosis, a disseminated and multifocal disease follows a worse clinical course. We report two cases of isolated vulvar LCH in 70- and 71-year-old females. Vulva shaved biopsy revealed dermal histiocyte-rich infiltrates admixed with numerous eosinophils. Histiocytes cells demonstrated eosinophilic cytoplasm and a coffee bean-shaped nucleus. Immunohistochemical staining showed diffuse positive staining for CD1a and S-100, supporting the diagnosis of LCH. BRAF exon 15, and V600E mutations were detected in both cases. The distinction// of Isolated vulvar LCH is essential due to its resemblance to other vulvar lesions and its tendency to follow an aggressive clinical behavior, local recurrence, and disseminated disease. A thorough systemic, radiologic, hematologic, and lymph node workup was highly recommended.

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