Zoon's Vulvitis Research Articles

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.

Vulvitis de Zoon en mujer en edad fértil. Reporte de un caso

La vulvitis de Zoon o vulvitis de células plasmáticas es una enfermedad inflamatoria crónica infrecuente, caracterizada por placas eritematosas bien delimitadas y brillantes que pueden afectar cualquier localización de la vulva. Suele presentarse en mujeres posmenopáusicas y plantea un difícil diagnóstico diferencial. Se insiste en la necesidad de realizar biopsia vulvar para obtener un diagnóstico histológico de certeza. Se presenta el caso de una paciente de 36 años y se comenta las opciones terapéuticas actuales descritas en la literatura.

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.

Mucinous Metaplasia of the Vulva in Zoon Vulvitis and Lichen Sclerosus et Atrophicus. Description of 3 Additional Cases of a Rarely Reported Histopathologic Finding

Mucinous metaplasia of the vulva (MMV) is a histopathologic finding that has been reported previously in only 3 patients and needs to be distinguished from vulvar extramammary Paget disease. We report 3 additional instances of MMV associated to Zoon vulvitis and vulvar lichen sclerosus et atrophicus. Histochemical and immunohistochemical studies were performed on biopsies from erythematous and erosive vulvar lesions of 3 women aged 64, 80 and 84 years, with features of Zoon vulvitis (2 cases) and lichen sclerosus et atrophicus (1 case). Mucin-containing epithelial cells were present on the uppermost layers of the squamous epithelium. On immunohistochemical study the metaplastic cells were positive for cytokeratin 7, epithelial membrane antigen and carcinoembryonic antigen, thus mimicking the phenotype of Paget disease. MMV is most likely related to chronic inflammation. Cytological and architectural features allow for distinction from Paget disease because the mucin-containing cells of mucinous metaplasia of the vulva lack atypia and are predominantly located on the most superficial layers of the surface epithelium.

Zoon's vulvitis and importance of histology in diagnosis of benign vulvar lesions in outpatient setting

Zoon's vulvitis and importance of histology in diagnosis of benign vulvar lesions in outpatient setting

Vulvar dermatoses: a histopathologic review and classification of 183 cases.

Vulvar dermatoses are often difficult to classify due to histopathologic overlap. We aimed to report our experience at a single institution. A total of 183 non-neoplastic, non-infectious vulvar biopsies were reviewed. Associations between histopathologic features and specific diagnoses were analyzed by Chi-squared tests. Twenty-two biopsies (12.0%) showed two concurrent processes. A limited differential rather than a definitive diagnosis was rendered in 15 cases (8.2%). The final diagnoses included lichen sclerosus (LS) (38.8%), lichen simplex chronicus (LSC) (29.0%), eczematous dermatitis (23.0%), Zoon vulvitis (8.2%), non-specific/resolved dermatitis (5.5%), hidradenitis suppurativa (2.7%), Behçet disease (2.2%), lichen planus (1.6%), ruptured cyst (1.6%), ulcer not-otherwise-specified (1.6%), psoriasis (1.1%), radiation dermatitis (1.1%), sebopsoriasis (1.1%), seborrheic dermatitis (1.1%), epidermolytic hyperkeratosis (0.5%) and granular parakeratosis (0.5%). Early LS and Zoon vulvitis were commonly included as part of a differential diagnosis. LS was associated with wiry collagen with lymphocyte entrapment (p = 0.0188). LSC was associated with zones of pale epithelium (p = 0.0084), and often displayed prominent fibroblasts (p = 0.0555). Zoon vulvitis was frequently misdiagnosed, and was associated with basal keratinocytic crowding (p < 0.0001). Our study has determined the relative frequencies of a wide variety of vulvar dermatoses, and identified new diagnostic clues for early LS, LSC and Zoon vulvitis.

Zoon Vulvitis as a Differential Diagnosis in an HIV-Infected Patient

Vulvitis circumscripta plasmacellularis or Zoon vulvitis is a rare benign condition that affects the vulva chronically. We herein report a case of Zoon vulvitis in a 52-year-old HIV-infected patient with an uncommon ulcerovegetating lesion diagnosed by histopathologic findings. The patient was treated with clobetasol propionate ointment and oral corticosteroid. Zoon vulvitisis is a rare vulvar disorder that offers a challenging diagnose and therapy. The patient was appropriately treated and remains disease free.

Zoon Vulvitis as a Differential Diagnosis in an HIV-Infected Patient: A Short Report

Zoon Vulvitis as a Differential Diagnosis in an HIV-Infected Patient: A Short Report

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.

Extensive mucinous metaplasia of the vulva arising within Zoon's vulvitis

Extensive mucinous metaplasia of the vulva arising within Zoon's vulvitis

Successful treatment of Zoon’s vulvitis with high potency topical steroid

Zoon's vulvitis is a rare, chronic condition of the vulva that presents with burning, pruritus, and dysuria with characteristic lesions and histopathology. Several treatment options have been reported with limited success. A 63-year-old woman with Zoon's vulvitis diagnosed on histopathology was treated with clobetasol propionate 0.05%. Complete resolution of her symptoms and lesions occurred in less than 1 week. No recurrence of her symptoms has occurred after 9 months. Zoon's vulvitis may be successfully and expeditiously treated with high potency topical steroids.

Chronic vulvar purpura: persistent pigmented purpuric dermatitis (lichen aureus) of the vulva or plasma cell (Zoon's) vulvitis?

Lichen aureus is localized variant of persistent pigmented purpuric dermatitis that typically affects the legs and can be associated with delayed hypersensitivity reactions or vascular abnormalities. Plasma cell vulvitis (Zoon's vulvitis) is a rare condition that frequently contains hemosiderin deposits and is suspected to be a mucosal reaction pattern due to variety of insults, most often local irritation or trauma. A 50-year-old female with longstanding complaints of spotting, vulvar dryness, irritation, and dyspareunia presented with circumscribed, purpuric, erythematous vulvar patches. Past estrogen cream treatment evoked symptoms of discomfort. On biopsy, siderophages and extravasated red blood cells were found in conjunction with a lichenoid, lymphocyte and plasma cell infiltrate, and dilated dermal and intraepithelial vessels. Reported herein is an unusual vulvar dermatosis that is best classified as a localized variant of persistent pigmented dermatosis (lichen aureus) but overlaps clinically and histologically with Zoon's vulvitis. This constellation of findings may represent a site-specific mucosal reaction to an erosive process that could either be inflammatory (hypersensitivity reaction) and/or traumatic in nature.

VULVITIS CIRCUMSCRIPTA PLASMACELLULARIS

A 56‐year‐old woman reported that an erythematous lesion, accompanied by severe burning, had appeared on the left labium minus about 2 years earlier. She had treated herself with topical deoxymethasone and estrogen topical medications that temporarily relieved the subjective synnptoms, but did little to help the clinical manifestations. The patient's history revealed an adnexectomy and ovariectomy at age 40 after an ectopic pregnancy and a left saphenectomy for ectatic varicose veins at age 54.We noted a bright red, shiny, almost lacquerlike lesion with clearly defined edges on the left labium minus that started from the external vaginal orifice and extended over half of the labium (Fig. 1). The lesion was not infiltrated and there was no inguinal lymphoadenopathy.Routine blood chemistry was normal and culture tests for bacteria and fungi were negative. The histologic examination showed a basically healthy epidermis with the exception of a focal reduction of the granular layer, a flattening of the dermoepidermal junction, capillary vasodilatation in the superficial dermis, and an infiltrate consisting of granulocytes, lymphocytes, and numerous plasma cells (Fig. 2).Direct immunofluorescence (DIF) performed with antigammaglobulin antiserum revealed numerous cell elements that tested positive with anti‐lgA antiserum with cytoplasmatic fluorescence at the dermal and subjunctional level (Fig. 3).On the basis of the clinical and histopathologic data we diagnosed a chronic circumscribed plasmacellular vulvitis, or Zoon's vulvitis. Excellent results were obtained after 3 months of topical treatment with cyclosporine.