Vulvitis circumscripta plasmacellularis in pre-existing lichen sclerosus

Abstract

a b s t r a c tVulvitis circumscripta plasmacellularis (VCP) is a rare, benign vulvar disorder that presents with erythematous patches and erosions. Patients with VCP usually experience vulvodynia, pruritus, or dyspareunia, which may cause severe discomfort. A 72-year-old woman presented with 10 years of vulvar pruritus and whitish patches on the labia minora and majora. A biopsy was performed on the labia minora and lichen sclerosus was diagnosed. Her condition improved after treatment with a topical steroid. After 5 months, however, her symptoms reappeared with aggravated skin lesions. A rebiopsy was performed and VCP was diagnosed. There have only been two reported cases of concurrence of VCP with lichen sclerosus. We herein report an unusual case of VCP in pre-existing lichen sclerosus. IntroductionVulvitis circumscripta plasmacellularis (VCP) was first described by Garnier 1 in 1954. It is a relatively rare chronic inflammatory disease. It is clinically and histopathologically similar to balanitis circumscripta plasmacellularis in males. Clinical symptoms of VCP are vulvodynia, pruritus, or dyspareunia, but VCP may be asymptomatic. 2 Only two cases have been reported on concurrent VCP and vulvar lichen sclerosus. 2,3 In this report, we describe an unusual case of VCP in pre-existing lichen sclerosus. Case reportA 72-year-old woman presented with 10 years of vulvar pruritus. She had localized whitish patches on the labia minora and majora ( Figure 1A). Her medical history included hypertension for 1 year. Laboratory test results were within normal limits. A biopsy was performed on the labia minora, including the majora, the results of which revealed pronounced edema and homogenization of collagen in the upper dermis. Hyperkeratosis and follicular plugging in the epidermis and inflammatory infiltrate in the middermis were observed (Figure 2). Lichen sclerosus was diagnosed, and the patient was treated with a topical potent corticosteroid (clobetasol-17-propionate) for 2 weeks. Her condition improved, but her symptoms were aggravated after 5 months. She presented with severe pruritus and pain in the vulvar area. A physical examination revealed localized whitish to erythematous eroded patches on the labia minora ( Figure 1B). A rebiopsy on the eroded patches of the labia minora was performed to rule out squamous cell carcinoma. The biopsy specimen showed epidermal thinning, absence of the stratum corneum, irregular acanthosis, and spongiosis in the epidermis. A dense dermal lichenoid infiltrate with many plasma cells and lymphocytes was observed (Figure 3). Results of an immunohistochemical analysis revealed that plasma cells were polyclonal for kappa and lambda light chains, and syphilis testing with venereal disease research laboratory was negative. Based on the results of the biopsy, VCP was diagnosed. After treatment with a systemic and topical corticosteroid, the patient's condition temporarily improved; however, her symptoms recurred, and she was advised to apply imiquimod 5% cream two to three times a wee...

Plasma Cell Vulvitis: A Systematic Review of Interventions

“…Topical corticosteroids (TCSs) are the most common first-line therapy, used in 32 studies. Of 60 patients, nearly 70% of the cases demonstrated therapeutic efficacy of TCS as monotherapy 7,10,12,16–18,21,22,24,25,32,37,40–44,47 . There are also reports of efficacy combining TCS with antiviral, 9 antibiotic, 7,43 saline cleanse, 20 nystatin vaginal suppository, 14 and estrogen 35 .…”

“…Another treatment option is imiquimod, a toll-like receptor 7 agonist, which may eliminate the plasma cell infiltrate via activation of nuclear factor κB and a T-helper 1–type cellular immune response with associated activation of CD8+ cytotoxic T cells 52 . All published cases of imiquimod report good efficacy 26,31,33,39,41 …”

“…Of 60 patients, nearly 70% of the cases demonstrated therapeutic efficacy of TCS as monotherapy. 7,10,12,[16][17][18]21,22,24,25,32,37,[40][41][42][43][44]47 There are also reports of efficacy combining TCS with antiviral, 9 antibiotic, 7,43 saline cleanse, 20 nystatin vaginal suppository, 14 and estrogen. 35 In addition, there is reported efficacy of intralesional corticosteroid in 2 patients 20 and systemic corticosteroids in 3 patients, 1 as monotherapy and 2 as combined therapy with TCS.…”

“…35 In addition, there is reported efficacy of intralesional corticosteroid in 2 patients 20 and systemic corticosteroids in 3 patients, 1 as monotherapy and 2 as combined therapy with TCS. 6,41 Topical Calcineurin Inhibitors Topical calcineurin inhibitors (TCIs), such as tacrolimus, have been shown to be effective for treatment of PCV. The 2 studies by Virgili et al 36,43 demonstrated significant improvement with tacrolimus in all 8 patients together with histopathological resolution in 2 of 4 patients on repeat biopsy.…”

“…Topical imiquimod achieved complete resolution in 7 recurrent and recalcitrant PCV cases. 26,31,33,39,41 Details regarding the strength and regime of imiquimod application were not specified.…”