Isolated Vulvar Langerhans Cell Histiocytosis: Report of Two Cases

Abstract

Two cases of Langerhans cell histiocytosis involving the vulva are reported. The clinical features of Case 1 have been previously reported. The patients, aged 31 (Case 1) and 52 (Case 2) years, had disease limited to the vulva at the time of diagnosis. In both cases, the vulvar lesions were composed of Langerhans cells with twisted nuclei and nuclear grooves, associated with eosinophils and other inflammatory cells. Immunohistochemical studies using fixed, paraffin-embedded tissue sections showed that the Langerhans cells were positive for CD1A and S-100 protein in both cases, supporting the morphologic diagnosis. The first patient had multiple local recurrences during 3 years despite radiation therapy and vulvectomy, but is currently in remission and being treated with thalidomide. The second patient experienced disseminated disease involving multiple bony sites and the mouth despite radiation therapy. Including the present two cases, 6 of 18 (33%) cases of isolated vulvar LCH subsequently disseminated, most commonly to bone; other patients had local recurrences. Thus isolated vulvar LCH has the potential for aggressive clinical behavior, either as local recurrence or disseminated disease. New treatment modalities are needed for this disease.

Diagnosis of Primary Langerhans Cell Histiocytosis of the Vulva in a Postmenopausal Woman

“…In some cases, topical steroids may treat LCH, but, in some other cases, LCH may stay in remission during the treatment period, and after the cessation of therapy life-threatening distant metastases may occur if not checked [7]. …”

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

“…Complete regression, partial improvement, persistent lesions and recurrences were found in all the cases described so far. Genital LCH can be the first presentation of systemic disease as described by Padula et al [8]. According to the literature, 33% of isolated vulvar LCH subsequently disseminated [7].…”

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.…”

“…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.…”