Primary Langerhans Cell Histiocytosis of the Vulva: Case Report and Review of the Literature

Abstract

Langerhans cell histiocytosis (LCH) of the vulva is rare and even moreso in postmenopausal women. Twenty-six cases of primary vulvar LCH have been described in the current literature, and only 8 cases are in postmenopausal women. We report an additional case of primary vulvar LCH in a 59-yr-old woman with subsequent multiorgan involvement. In this article, we briefly describe the clinical presentation, histopathological findings, and immunohistochemistry results of vulvar LCH. We want to emphasize the importance of recognizing this entity in a woman with vulvar lesions both for the clinician and the pathologist.

Altered mental status and vulvar ulcer in a 10‐year‐old girl

“…Vulvar ulcers in patients who have never been sexually active are rare; they can be idiopathic or associated with systemic illness including Bechet's, Crohn's, EBV infection or neoplasm 1 . Vulvar lesions rarely present as the first manifestation of LCH, when they may manifest as ulcers, papules or nodules 4 . As with this case, biopsy of the lesion is crucial for diagnosis.…”

“…1 Vulvar lesions rarely present as the first manifestation of LCH, when they may manifest as ulcers, papules or nodules. 4 As with this case, biopsy of the lesion is crucial for diagnosis.…”

Vulvar Langerhans cell histiocytosis: Clinicopathologic characteristics, mutational profile, and treatment of 4 patients in a single-center cohort

“…Vulvar Langerhans cell histiocytosis (vLCH) is rare, occurs at any age, and often heralds MS-LCH. 3 , 4 Topical skin treatment encompasses medium-high potency corticosteroids, nitrogen mustard, or imiquimod. For more extensive and/or therapy-resistant lesions, phototherapy or systemic therapy can be considered (methotrexate 20 mg 4 times a week, thalidomide 100-200 mg/d, hydroxyurea 1000 mg/d, chemotherapy).…”

“…Vulvar LCH (vLCH) is rare, occurs at any age and often heralds MS-LCH [3,4]. Topical skin treatment encompasses medium-high potency corticosteroids, nitrogen mustard or imiquimod.…”