Langerhans cell histiocytosis of vulva in adolescent

Abstract

Langerhans cell histiocytosis (LCH) affecting child vulva alone is a very rare disease. Only 13 cases of primary vulvar LCH have been previously reported in the medical literature. We describe an additional case in which the LCH was confined to the vulva, with review of the literature. A 16.5-year-old girl presented with papulous and ulcerative lesions on her labia majora and minora. The biopsy revealed a typical histopathologic finding consistent with LCH. A metastatic work-up did not reveal any evidence of the disease except on the vulva. Treatment was carried out according to LCH II protocol. The patient was diagnosed with a recurrent disorder in the vulva 8 months after the completion of primary chemotherapy. For this reason, she underwent second line treatment with 2-chlorodeoxyadenosine. Eighteen months after the second line chemotherapy, the patient has no signs of a local or systemic recurrence. Primary LCH of vulva is very unusual, but we have to keep in mind this possibility when an adolescent girl presents with an atypical chronic lesion on the vulva. This patient appears to be the first case of adolescent 16.5 year old having a solely cutaneous lesion of the vulva.

Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids

“…9,10,15 Primary vulval LCH should be considered in any female patient who presents with chronic pruritus, pain, ulceration, nodules or intermittent rashes unresponsive to treatment. [1][2][3]6,14 This case highlights the successful treatment of a rare condition that is difficult to treat. Treatment of LCH is poorly defined as there is no epidemiologic evidence and limited follow-up.…”

“…4 Treatment modalities with variable efficacy include surgery, immunosuppression, isotretinoin, thalidomide, trimethoprimsulfamethoxozole, radiotherapy, topical and systemic chemotherapy and ultraviolet light therapy. 1,3,9,15,16 Application of topical corticosteroids does not usually lead to resolution of the symptoms or signs of LCH. 1 Langerhans cell histiocytosis has a variable clinical course.…”

“…1,3,9,15,16 Application of topical corticosteroids does not usually lead to resolution of the symptoms or signs of LCH. 1 Langerhans cell histiocytosis has a variable clinical course. 4 Unifocal LCH generally has an excellent prognosis.…”

“…8 There have been only 19 case reports of primary isolated vulval adult LCH with the youngest affected patient being 16.5 years old as summarized in Table 3. [1][2][3][6][7][8][9]11,15,16,[19][20][21][22][23] Vulval ulcers are the most common presentation of genital LCH. 10 Genital LCH can also present as erythematous plaques, papules or nodules.…”

“…4 Primary unifocal involvement of the vulva is even less frequent. 1,2,[5][6][7][8][9][10][11][12] In 1987, the Writing Group of the Histiocyte Society endorsed LCH to cover the broad spectrum of clinical presentations ranging from a diffuse fulminant form (LittererSiwe disease) to a chronic progressive form (HandSchüller-Christian syndrome) to an isolated benign lesion (eosinophilic granuloma). 13 Langerhans cell histiocytosis is the most common of the dendritic group of Histiocytic disorders, the other two groups being the erythrophagocytic macrophage and malignant histiocytosis types.…”