Successful multidisciplinary treatment of the rare pathology of vulvar Crohn's disease.

A woman in her 50s with a medical history of rheumatoid arthritis (RA) and hypothyroidism presented with 3 months of vaginal pain and swelling. Physical examination showed vulvar oedema and erythema, ulcerations of the inferior vulva that extended to the buttocks and introitus with erythema and superficial erosions. MR pelvis showed a fibroid in the uterus but otherwise was unremarkable. Biopsies of the vagina showed extensive acute and chronic inflammation, necrosis and granulomas without concern for malignancy, which was consistent with a diagnosis of cutaneous Crohn's disease. MR enterography, upper endoscopy and colonoscopy revealed no gastrointestinal manifestations of Crohn's. She was treated initially with corticosteroids and metronidazole as well as vulvectomy and graft placement. Azathioprine was added to her regimen due to lack of response; however, despite 6 weeks of this therapy, the ulcerations did not heal thus infliximab was initiated. After 8 weeks the lesions had completely resolved.

Idiopathic Lichenoid and Granulomatous Vulvitis: A Distinct Clinicopathological Entity.

ABSTRACT: The combination of lichenoid and granulomatous inflammation is uncommon in vulval biopsies. We present a series of 5 patients with lichenoid and granulomatous vulvitis, presenting with clinical changes resembling lichen sclerosus. Despite detailed clinicopathological investigation and follow-up, there was no apparent association with an underlying recognized cause. All 5 cases occurred in postmenopausal women and displayed a distinctive histological pattern of superficial band-like inflammation with granulomas "anchored" to the dermoepidermal junction. There was no evidence of deeper granulomatous inflammation. Despite repeated biopsies over 2 years in 2 patients, neither developed typical histological features of lichen sclerosus. We postulate that idiopathic lichenoid and granulomatous vulvitis may represent a distinct clinicopathologically defined vulvar dermatosis.

Psoriasis vulvae.

OBJECTIVE: Case presentation, differential dia-gnostic, possible therapeutic procedure and a brief literature report. CASE REPORT: An 84-year-old woman was repeatedly examined for chronic extended dermatologic lesion on lower abdomen, external genitals, perianal region, groin and adjacent inner thighs. CONCLUSION: Neither colposcopy with dermatovenerologic consultation, nor histopathological examination led to a precise conclusion. Only a special dermatopathologic expertise showed a dia-gnosis of inverse psoriasis with suggested therapy.

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

OBJECTIVE: The aim of the study was to identify whether desquamative inflammatory vaginitis (DIV) and plasma cell vulvitis (PCV) are distinct clinicopathologic entities. MATERIALS AND METHODS: The 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. RESULTS: There 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. CONCLUSIONS: Desquamative 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.

Chikungunya associated vulvitis: case report and literature review / Vulvite associada a Chikungunya: relato de caso e revisão de literatura

Introduction: Chikungunya virus is spreading worldwide due to migration and globalization and could be presented with systemic and with unusual symptoms. Objective: To report a case of virus-transmitted infection detected in a woman during the gynecological examination at a vulvar clinic. Case report: A 73-year-old Caucasian woman attended a vulvar clinic because of dyspareunia and vulvar burning. Ulcers were observed on labia minora and perineum. A Chikungunya was diagnosed by seroconversion in paired specimens. She was prescribed prednisolone 40 mg once a day for 10 days. After oral steroid treatment, the woman had no body rashes or lesions on her genitals. Conclusion: This study emphasized that rare signs of unusual vulvitis with ulcers could be associated with Chikungunya infection.

Introdução: O vírus Chikungunya está se espalhando pelo mundo por conta da migração e da globalização, podendo apresentar sintomas sistêmicos e incomuns. Objetivo: Relatar um caso de infecção pelo vírus detectado em uma mulher por ocasião do exame ginecológico em clínica de patologia vulvar. Relato do caso: Uma mulher caucasiana de 73 anos foi a uma clínica vulvar por causa de dispareunia e queimação vulvar. Úlceras foram observadas nos pequenos lábios e no períneo. O diagnóstico de Chikungunya foi realizado por soroconversão em espécimes pareados. Foi prescrita prednisolona 40 mg uma vez ao dia por dez dias. Após o tratamento com esteróides orais, a mulher não apresentou erupções ou lesões nos órgãos genitais. Conclusão: Este estudo enfatizou que quadros raros de vulvite com úlcera podem estar associados à infecção por Chikungunya.

Vulvar diseases: Conditions in adults and children.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

Hypertrophic Herpes Simplex With Subsequent Development of Plasma Cell Vulvitis: A Potential Diagnostic Pitfall.

A 37-yr-old patient previously diagnosed with human immunodeficiency virus initially presented with a genital lesion which upon histologic assessment was diagnosed as a pseudotumor associated with herpes simplex virus infection. The pseudotumor responded to initial treatment with Acyclovir, however, the lesion recurred 2 yr later and was diagnosed as plasma cell vulvitis. We discuss the clinical presentation, diagnostic work up and treatment options of such a rare lesion.

Vulvar Silicone Granuloma.

We report a case of vulvar silicone granulomas following injection of liquid silicone into the labia. The patient is a 51-yr-old female who presented with vulvar pain and enlarged, indurated labia majora. In the past, she had undergone bilateral labial cosmetic augmentation with a silicone-based filler injected directly into the labia and into the gluteal regions. This had been performed in a nonmedical setting. At surgery, oblong firm soft tissue masses were removed from both labia. Microscopically, the lesions demonstrated replacement of the subcutaneous adipose tissue by fibrous tissue containing innumerable round empty spaces of different sizes, either within or surrounded by macrophages and occasional foreign-body giant histiocytes. The clear vacuoles corresponded to silicone fluid which had been dissolved during tissue processing. There are only rare case reports of vulvar silicone granuloma in the literature, and these were due to migration of silicone to the vulva from distant sites. Our report details a case of vulvar silicone granuloma secondary to direct injection of liquid silicone material into the labia.

Nivolumab in chemotherapy-resistant cervical cancer: report of a vulvitis as a novel immune-related adverse event and molecular analysis of a persistent complete response.

BACKGROUND: Treatment options for advanced cervical cancer are limited and patients experiencing recurrence after first-line cisplatin-based chemotherapy and bevacizumab have a poor prognosis. A recent phase II study in advanced cervical cancer has demonstrated a disease control rate of 68.4% with the immune checkpoint inhibitor nivolumab. By blocking immune checkpoints, immunotherapy puts the immune system into a state of hyper-activation that can cause immune-related adverse events. We present the clinical, pathological and molecular data of a patient with metastatic cervical cancer and progressive disease after second-line therapy. We report on the therapeutic response under third-line immunotherapy with nivolumab, the immune-related adverse events (IRAE), and their successful management. CASE PRESENTATION: We report the case of a 62-year-old woman who was diagnosed with advanced squamous cell carcinoma of the cervix with paraaortic lymph node metastases. After an initial combined radio-chemotherapy with cisplatin, she developed local and nodal (supraclavicular) recurrence. Second-line chemotherapy with 6 cycles of carboplatin, paclitaxel, and bevacizumab resulted in a partial response for 6 months. Checkpoint inhibition with nivolumab was started due to progression, leading to persistent complete remission. Immunotherapy was well tolerated for 8 months until the patient presented with an immune-related isolated vulvitis, which was successfully managed with topical corticosteroids. CONCLUSIONS: The persistent complete response after third-line treatment for relapsed chemotherapy-resistant cervical cancer presented in this case highlights the potential of immunotherapy for patients with advanced cervical cancer impressively. To our knowledge, this is the first report of an isolated immune-related vulvitis under nivolumab. This adverse event might be underdiagnosed and mistreated, however, it is of importance due to its impact on quality of life, sexual wellbeing and compliance of patients. Successful IRAE management may enable prolonged immune checkpoint inhibitor therapy. In the future, routine molecular tumour profiling is likely to aid in the stratification of cervical cancer patients for immunotherapy. Here, we provide the methylome data of a case with complete response.

Vulvitis migratoria causada por gnatostomiasis importada / Migratory vulvitis caused by imported gnathostomiasis

Resumen La gnatostomiasis es una parasitosis emergente en países no endémicos. Este nematodo zoonótico requiere de agua dulce para su ciclo de vida, donde sus larvas se enquistan principalmente en peces. La migración subcutánea de las larvas produce habitualmente una paniculitis eosinofílica de rápido avance. Se describe un caso clínico de un paciente con una lesión migratoria, sin mejoría clínica con terapia antibacteriana. La búsqueda de factores de riesgo, sumado a la evolución y a los hallazgos de laboratorio hizo sospechar el diagnóstico. La gnatostomiasis debe ser sospechado en pacientes con lesiones de piel migratorias, que han consumido pescado crudo durante viajes a países endémicos en Sudamérica o Asia.

Gnathostomiasis is an emerging disease in non-endemic countries. This zoonotic nematode requires aquatic freshwater environments to complete its life cycle where larvae get encrusted in fishes. Typically, the infection manifests as migratory subcutaneous lesion caused by the larvae trak, which produces an eosinophilic panniculitis. Here we describe a patient who presented a migratory lesion with no response to antimicrobial therapy, a careful travel and food history together with specific laboratory tests led to the correct diagnosis. Gnathostomiasis should be suspected in patients with migratory skin lesions who have consumed raw freshwater fish during travel to endemic countries in South America or Asia.

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 vulvitis - a rare form of chronic inflammation of the vulva.

OBJECTIVE: Description of finding out rare disease Zoon vullvitis. Desing: Case report and differential diagnosis. SETTING: Department of Obstetric and Gynecology, University Hospital Ostrava. CASE REPORT: Zoon vulvitis or plasma cell vulvitis (PCV) belongs to extremely rare and often misdiagnosed inflammatory disease of the vulva. Lesions may look like shiny, sharply bordered , erythematosus patches or macular lesions. Less than 50 cases have been reported. This article is devoted to clinical findings, differential diagnosis and treatment of PCV. We present one clinical case with typical clinical and histopathological manifestations. Risk of dysplasia exists and long-term follow-up is desirable.

Female Genital Itch.

Vulvar pruritus is a common complaint among young girls and women presenting to primary care physicians, gynecologists, and dermatologists. Female genital itch is especially disruptive because of its interference with sexual function and intimacy. Causes of vulvar itch are vast and may be inflammatory, environmental, neoplastic, or infectious, often with several causes coexisting simultaneously. Diagnosis may be difficult because of the unique anatomy and inherent properties of genital and perianal skin. Treatment is aimed at eliminating outside irritants, restoring epidermal barrier function, and suppressing inflammation.

[Miescher's granulomatous vulvitis]. / Vulvite granulomateuse de type Miescher.

BACKGROUND: Miescher's cheilitis, whether occurring alone or as part of Melkersson-Rosenthal syndrome, is a rare type of granulomatous inflammation of unknown cause with an even rarer genital equivalent. Herein, we describe a case of the latter condition developing over a 12-year period. PATIENTS AND METHODS: A 27-year-old woman presented episodes of unilateral vulvar oedema, which initially regressed but resulted over time in permanent vulvar and perineal tumefaction. Histology revealed epithelioid histocytic granulomas so mild that they were not noticed at the start of the disease. The oedema remained the sole sign for 12years and two gastrointestinal screening tests for Crohn's disease proved negative, diagnosis of the latter condition was ruled out. DISCUSSION: Miescher's granulomatous vulvitis requires differential diagnosis, essentially with regard to Crohn's disease. Our case illustrates the difficulty in diagnosing this rare disease as well as the uncertainties surrounding its physiopathology. Diagnosis rests upon repeated sampling and biopsies with repeated levels of sections in order to identify the characteristic perivascular granulomas, which may be very mild.

Zoon vulvitis.

Zoon vulvitis or vulvitis chronica plasmacellularis is a rare, chronic benign inflammation of the vulvar mucosa, diagnosed histologically, with variable therapeutic responses. It is important to be diagnosed because it mimics the presentation of other genital conditions, such as lichen planus and squamous cell carcinoma, which require specific treatment. We report a case of a female patient with three asymptomatic shallow ulcers on the labia minora. Laboratory tests ruled out infectious diseases and the biopsy was consistent with Zoon Vulvitis.