Plasma Cell Vulvitis Research Articles

Plasma Cell Vulvitis: A Classification Into Two Clinical Phenotypes.

The etiology of plasma cell vulvitis (PCV) is debated. The authors aimed to test the hypothesis that PCV could be divided into 2 clinical phenotypes. Patients with a clinico-pathological diagnosis of PCV and with available vulvar photos seen in a vulvar clinic were retrospectively studied. The cases of PCV were divided into 2 groups: non-lichen-associated (primary PCV) and lichen-associated (secondary PCV). The 2 groups were compared in terms of age, menopausal status, location of the PCV, and 12 histologic parameters (Fisher exact test, p < .05). Thirty-five patients (20 primary and 15 secondary PCV) were included. The 2 groups did not differ in terms of age (mean, 65; range, 50-85) or menopausal status. Primary PCV was located exclusively on the vestibule for 19/20 patients, whereas secondary PCV was extravestibular for 14 of 15 patients, either exclusively (2) or both extravestibular and vestibular (12). One patient with secondary PCV had solely vestibular involvement. Five histological features were observed significantly more often in case of secondary PCV: epidermal atrophy, parakeratosis, dermal and epidermal neutrophils, and dermal eosinophils. Plasma cell vulvitis can be divided clinically into 2 phenotypes. Primary non-lichen-associated PCV is restricted to the vestibule and could be the vulvar counterpart of atrophic vaginitis. Secondary lichen-associated PCV is both extravestibular and vestibular, and its clinical and histological features should be looked for outside the PCV areas. This division of PCV into 2 clinical phenotypes could have therapeutic implications.

Plasma Cell Vulvitis: A Case Series and Follow-Up Study.

Plasma cell vulvitis (PCV) is a rare inflammatory vulvar condition. The aim of this study was to describe the natural history, treatment, impact on quality of life, and factors associated with poorer outcomes for PCV. A mixed-methods approach was used combining a retrospective case note review with a cross-sectional telephone questionnaire. All women diagnosed with PCV attending the vulvar disorders clinic at the Royal Women's Hospital between January 2011 and December 2020 were included. During the 10-year study period, 7,500 women were seen at the vulval disorders clinic, of whom 21 were diagnosed with PCV (0.28%). Of these women, 12 who were followed up for more than 12 months agreed to participate in the study. At a median of 5 years follow-up, there was variability in symptom severity, with more than half of the women still symptomatic with pain, precipitated by friction and dyspareunia, resulting in a moderate to large impact on quality of life. There were 5 women who were asymptomatic. Only 1 woman had a preexisting history of lichen planus and lichen sclerosus. Potent topical corticosteroids were identified as the preferred treatment. Women with PCV can remain symptomatic for many years with significant impacts on quality of life, which may consequently require long-term support and follow-up.

Plasma Cell Vulvitis Treated with Platelet-rich Plasma: A Case Report.

case report; immunosuppressant; platelet-rich plasma; vulvitis.

Is vulvar purpura a component of plasma cell vulvitis or mucosal porosis?

Here, we presented six women with isolated vulvar purpura. No associated lesions such as sclerosis, atrophy, or hypopigmentation were observed. All patients were in postmenopausal period. In this case series, we discussed if a non-blanching purpura could be a component of plasma cell vulvitis or “mucosal porosis”. The possibility of vulvar purpura was questioned as a mucosal equivalent of dermatoporosis which is an age-related degenerative process in the skin. While senile purpura is one of the morphological findings of dermatoporosis, vulvar purpura might be a part of “mucosal porosis”. Rapid response to topical estrogen was remarkable in all patients.

AB143. SOH22ABS060. Plasma cell vulvitis or Zoon’s vulvitis

Background: Plasma cell vulvitis, or Zoon’s vulvitis, is a rare, benign, idiopathic, chronic inflammatory condition that affects the vulva. In 1952, Zoon reported a histological plasmacytic infiltrate, called balanitis plasma cellularis, which affected the penis and prepuce. Similar lesions identified in the vulva were reported by Garnier in 1954 and described as Zoon’s vulvitis. Case reports of this condition are rarely described in literature.

Vulvar inflammatory disorders: A review

Vulvar inflammatory disorders (VID) are a miscellaneous group of non-infectious conditions of the vulvar area, which can be broadly classified based on etiology and pathology. Here, we discuss a few pertinent VID including vulvar dermatitis, psoriasis, lichen planus, and lichen sclerosus. This review will focus only on common VID, and a few rare conditions with characteristic features such as Crohn’s disease, plasma cell vulvitis, acantholytic disorder of genitocrural area, desquamative vulvitis, and atrophic vulvovaginitis. A thorough history taking and relevant investigations including dermoscopy and biopsy are relevant to proper diagnosis and management of VID. Early management of VID is essential since these conditions are prone to interfere with psychosexual functioning of the patient. Potent topical steroids are the first line management in most cases, followed by topical calcineurin inhibitors, emollients, and antihistamines. Vulvar area is resistant to steroid induced atrophy; hence, use of potent topical steroids does not pose a problem. Oral steroids or steroid sparing agents are used in resistant cases.

Desquamative Inflammatory Vaginitis and Plasma Cell Vulvitis Represent a Spectrum of Hemorrhagic Vestibulovaginitis.

ObjectiveThe aim of the study was to identify whether desquamative inflammatory vaginitis (DIV) and plasma cell vulvitis (PCV) are distinct clinicopathologic entities.Materials and MethodsThe pathology database identified biopsies described as “vaginitis” or “vulvitis” occurring in nonkeratinized epithelium or mucocutaneous junction. Exclusions were age less than 18 years, unavailable slides or records, concurrent neoplasia, or histopathology consistent with other entities. Clinical data included demographics, symptoms, examination, microbiology, treatment, and response. Histopathologic review documented site, epithelial thickness and characteristics, infiltrate, and vascular abnormalities. Cases were analyzed according to histopathologic impression of DIV or PCV based on previous pathologic descriptions.ResultsThere were 36 specimens classified as DIV and 18 as PCV from 51 women with mean age of 51 years; 3 (6%) had concurrent biopsies with both. Pain was more common in PCV, but rates of discharge, itch, and bleeding were comparable. Rates of petechiae or erythema were similar and vaginal examination was abnormal in 72% of PCV cases. All DIV and 33% of PCV occurred in squamous mucosa; the remaining PCV cases were from mucocutaneous junction. Mean epithelial thickness, rete ridge appearance, exocytosis, and spongiosis were similar in DIV and PCV. Epithelial erosion, wide-diameter lesions, plasma cells, and stromal hemosiderin occurred in both but were more common in PCV. Lymphocyte-obscured basal layer, narrow-diameter lesions, hemorrhage, and vascular congestion were seen in both, but more common and marked in DIV.ConclusionsDesquamative inflammatory vaginitis and PCV have overlapping symptoms, signs, and histopathologic features. They may represent a single condition of hemorrhagic vestibulovaginitis with varying manifestations according to location and severity.

Les cinq lésions inflammatoires les plus fréquentes en anatomie pathologique vulvaire

Lichen sclerosus, lichen planus, psoriasis and plasma cell vulvitis are the most common diagnosis amongst inflammatory vulval diseases, the most frequently suspected by the clinicians and the most frequently diagnosed by the pathologist. We expose their clinical and pathological aspects and detail the most recurrent diagnosis difficulties.

Plasma Cell Vulvitis: A Systematic Review.

This systematic review aimed to present the available literature on plasma cell vulvitis (PCV), a relatively uncommon form of inflammatory vulvovaginal dermatitis. A literature search was performed in PubMed, Science Direct, and Google Scholar using the following key words: "plasma cell vulvitis," "Zoon vulvitis," and "vulvitis circumscripta plasmacellularis." Specific variables were assessed in each article, including patient age, menopausal status, comorbidities, presenting symptoms, symptom duration, histological description, treatment, and treatment response. Thirty-nine articles met inclusion criteria, including 38 case reports and 1 observational study, with a total of 96 cases of PCV reported. The mean age of diagnosis was 52.9 years, with an age range of 8-76 years. Most common presenting symptoms included pruritis and vaginal discomfort, with average duration of symptoms 28.2 months (range = 2 months to 10 years). All reports demonstrated subepithelial plasma cell infiltrate on histology. Five percent of PCV cases reported concomitant autoimmune conditions and 6% sexually transmitted infections. Most common treatment modalities included topical corticosteroids (n = 41), tacrolimus (n = 6), and imiquimod (n = 6). In 53 reported outcomes, 88.7% of patients had resolution of symptoms with treatment. Clinical research is needed to better determine the diagnostic criteria and to assess the efficacy of treatment options for PCV.

Plasma Cell Vulvitis: A Systematic Review of Interventions.

Plasma cell vulvitis (PCV) is a rare chronic inflammatory disorder, where the symptoms can be severe and may affect patient's quality of life. However, there are currently no evidence-based treatment guidelines. The aims of this systematic review were to evaluate efficacy of individual treatments and to inform future research. A systematic search was conducted of publication between 1952 and August 2020 via MEDLINE, Embase, and Emcare. All publications that evaluated the efficacy of treatments for patients with PCV were included. Forty-seven publications comprising 45 case reports and case series and 2 cohort studies were included. To date, there are no randomized controlled trials evaluating the efficacy of different treatment options. There are also no studies that assess the impact of treatment on quality of life. Topical corticosteroids are the most frequently used first-line therapy, with limited evidence to support their efficacy, followed by topical imiquimod and surgical excision. The least supported intervention is cryotherapy. Although descriptive studies support the use of topical corticosteroids and, to a lesser degree, topical calcineurin inhibitors and imiquimod, this review highlights the need for standardized outcome measures and randomized clinical trials for more definitive therapeutic recommendations for women with PCV.

Plasma cell vulvitis: A systematic review.

BackgroundPlasma cell vulvitis (PCV) is an inflammatory vulvar dermatosis that is not well characterized. Diagnosis is often delayed, and the condition can be refractory to treatment. To date, there are no systematic reviews on this topic.ObjectiveThis study aimed to provide a systematic review of PCV, including epidemiologic, clinical, and histopathologic findings, as well as associated comorbidities and treatment options.MethodsA primary literature search was conducted using the PubMed, Ovid Medline, Cochrane, and CINAHL databases.ResultsFifty-three publications with 196 patients (mean age: 55.3 ± 14.5 years) were included. The majority of studies were case reports and case series. Common symptoms included burning/stinging (52%), dyspareunia (44%), and pruritus (41%). Common findings included erythema (84%), glistening/shiny appearance (29%), well-demarcated lesions (25%), and erosions (22%). Common anatomic sites were the labia minora (45%), introitus (31%), and periurethral (19%). Fifty-three percent of patients had a solitary lesion. Common histologic findings were a predominant plasma cell infiltrate (88%), presence of other inflammatory cells (55%), hemosiderin/siderophages (46%), and epidermal atrophy (43%). Topical corticosteroids (64%) and tacrolimus ointment (13%) were the most frequent treatment modalities. In most reports, previous treatments were tried, and there was a diagnostic delay.ConclusionPCV is likely underrecognized and should be considered in patients with erythema of the mucous and modified mucous membranes, symptoms of burning or stinging, and a predominant plasma cell infiltrate on histopathology. First-line therapy should begin with high-potency topical corticosteroids, with the most evidence for clobetasol 0.05% or tacrolimus 0.1% ointment. Prospective studies are needed to further characterize this condition and to develop treatment guidelines.

027 Plasma Cell Mucositis of the Vagina and Cervix

In 1952, Professor Johannes Jacobus Zoon described Zoon’s balanitis, an inflammatory condition which causes increased plasmocytic infiltrate of the penis and prepuce (1,2). Zoon’s vulvitis, commonly referred to as plasma cell vulvitis (PCV) or vulvitis circumscripta plasmacellularis, was first documented by Garnier in 1954 (1,2,3). PCV is a rare, chronic inflammatory disease characterized by erythematous plaques with a red, orange hue and increased plasma cells affecting the vulva. PCV predominantly affects the vulvar vestibule, but may extend to the labia minora and majora.

Pictorial Representation of Illness and Self-Measure to assess the perceived burden in patients with chronic inflammatory vulvar diseases: an observational study.

Chronic inflammatory vulvar diseases can have a huge detrimental impact on patient welfare. A few studies have addressed this issue so far, mainly measuring patients' quality of life or sexual dysfunction. To assess the burden of suffering in patients with chronic inflammatory vulvar diseases using the Pictorial Representation of Illness and Self-Measure (PRISM), a visual, non-verbal instrument. We also assessed (i) the concordance between PRISM and the Dermatology Life Quality Index (DLQI), (ii) whether some variables, both patient-related and disease-related, affect the patient's suffering and quality-of-life impairment. We evaluated for inclusion in this cross-sectional study all patients who attended our Vulva Unit over a 9-month period with histologically proven lichen sclerosus (LS), lichen planus (LP), lichen simplex chronicus (LSC), eczema, plasma cell vulvitis and psoriasis (at least five for each disease). Demographics and disease-related subjective and objective scores were recorded. The PRISM and DLQI were administered. We included 87 patients affected with LS, 13 with LSC and seven with LP. Median PRISM values (0-273mm) ranged from 95 to 120mm, depending on the disease, and median DLQI scores (0-30) were five for all three groups. Neither PRISM nor DLQI scores differed significantly among the three groups. Moderate coherence was found between PRISM and DLQI (ρ=0.5455, P<0.001). Global subjective score was the only variable significantly associated with the degree of suffering and quality-of-life impairment. Pictorial Representation of Illness and Self-Measure proved to be a valuable, highly reliable tool for measuring the perceived burden in these patients. In spite of a moderate correlation, PRISM resulted more sensitive in capturing patient distress than DLQI.

Zoon’s Vulvitis: A case report

Zoon’s vulvitis or plasma cell vulvitis (PCV) is a rare, benign inflammatory condition of the vulvar mucosa [1]. PCV can present with an asymptomatic lesion, or can cause discomfort, dyspareunia and pruritus [2]. In this way, PCV can mimic other lesions of the vulval mucosa, such as lichen planus.

Лихеноидные дерматозы вульвы — клиника, морфология и дерматоскопическая картина

Lichenoid vulvar dermatoses are a heterogeneous group of diseases characterized by a number of overlapping signs and/or morphological features. Clinical similarity is accounted for by the appearance of small discrete papules with closely adjacent superficial squamae. The prototype of lichenoid dermatoses is lichen ruber planus. Histologically, lichenoid pattern is characterized by basal epithelial cell damage and a band-like infiltrate in the upper dermis. The International Society on Vulvovaginal Diseases categorizes lichen ruber planus and early lichen sclerosus as dermatoses with this histological pattern. Some authors also classify plasma cell (Zoon) vulvitis as lichenoid vulvar dermatoses. The cause of diagnostic errors lies in the similarity of the clinical and histological presentations of lichenoid vulvar dermatoses. Dermatoscopy should be considered as an additional diagnostic test. Only few studies address the key dermatoscopic signs of vulvar dermatoses. Histological study is important for the differential diagnosis. However, at some stages of disease evolution, vulvar dermatoses can have an overlapping histological pattern. This paper summarizes the data on the clinical, histological, and dermatoscopic presentation of the most common lichenoid vulvar dermatoses, i.e., lichen planus, lichen sclerosus, lichen simplex chronicus, and plasma cell vulvitis. KEYWORDS: lichenoid dermatoses, vulvar dermatoses, lichen sclerosus, lichen planus, lichen simplex chronicus, plasma cell vulvitis, Zoon vulvitis, dermoscopy. FOR CITATION: Parygina O.V., Smirnova I.O., Oganesyan M.V. et al. Lichenoid vulvar dermatoses: clinical presentation, morphology, and dermatoscopic signs. Russian Medical Inquiry. 2020;4(10):617–624. DOI: 10.32364/2587-6821-2020-4-10-617-624.

Plasma cell vaginitis and cervicitis

Introduction: Plasma cell vulvitis (PCV), also known as Zoon’s vulvitis, is a rare, chronic inflammatory disease characterized by erythematous plaques with a red, orange hue and increased plasma cells typically affecting the vulva.

Symptoms in Plasma Cell Vulvitis: First Observational Cohort Study on Type, Frequency and Severity

Background: Studies assessing symptoms of plasma cell vulvitis (PCV) are lacking. Objectives: To assess the prevalence and severity of PCV-related symptoms and identify possible associations between patient characteristics, clinical features of PCV and treatments administered before a definitive diagnosis. Methods: Thirty-six patients affected with PCV were included. Data were collected by direct interview and clinical examination. Results: Thirty patients (83.3%) complained of symptoms: burning was the most frequent (80.6%) while dyspareunia was the severest. Of the symptomatic patients, 73.3% experienced severe symptoms. Severity of symptoms was not associated with age at onset and duration of PCV. Almost 70% of the patients had previously undergone treatments. Conclusions: Symptoms in PCV are frequent and more than often severe. Neither age at onset nor duration of the disease nor the extent of vulvar involvement were associated with symptom severity. Both the delay in diagnosis and the inappropriate previous treatments seem to indicate frequent misdiagnosis.

Plasma Cell Vulvitis: A Case Report and Review of Literature

Plasma cell vulvitis (PCV) is a rare disease affecting anogenital skin. It is also known by multiple names including Zoon’s vulvitis and vulvitis circumscripta plasmacellularis. It is an inflammatory condition that can cause severe local discomfort and psychosocial distress. The diagnosis is challenging and often remains unrecognized. It usually presents as a glistening, red, well-demarcated plaque or patch. The pathophysiology is unknown. Treatment recommendation is mostly based on case reports and case series and this condition is often recalcitrant to topical and systemic treatment.

Case Series of Zoon Vulvitis

We report 3 cases of plasma cell vulvitis (Zoon vulvitis) over a 10-year period and their management. This series highlights 1 case that was refractory to all modalities of treatment and hence had to be managed surgically, which has only been reported once before.

Tacrolimus 0.1% Ointment: Is It Really Effective in Plasma Cell Vulvitis?

Background: Plasma cell vulvitis is a clinically and histologically well-characterized chronic disease that usually relapses after various topical therapies. Considering the inflammatory nature of the disease, the new topical calcineurin inhibitors have been also employed successfully in few cases of Zoon’s balanitis, the corresponding male condition. Objective: The aim of our study is to evaluate the effectiveness of tacrolimus ointment in a small group of plasma cell vulvitis sufferers. Methods: 4 women affected by biopsy-proved plasma cell vulvitis were enrolled, after informed consent. The topical drug was applied twice daily for 6 weeks, then tapered on the basis of the clinical results. Symptoms and objective parameters were obtained periodically at the beginning, after 6 weeks and up to the end of the topical treatment. A final biopsy was performed in 3 out of our 4 patients. The follow-up is still ongoing. Results: The comparative analysis of subjective, objective and histopathological data has shown discordant and less encouraging results than those reported for the corresponding male condition. Conclusion: At the moment, topical tacrolimus could be considered an alternative treatment for plasma cell vulvitis only in cases resistant to conventional therapies.