-
Frontiers in Medicine 2023Lichen sclerosus (LS) is an underdiagnosed inflammatory mucocutaneous condition affecting the anogenital areas. Postmenopausal women are predominantly affected and, to a... (Review)
Review
Lichen sclerosus (LS) is an underdiagnosed inflammatory mucocutaneous condition affecting the anogenital areas. Postmenopausal women are predominantly affected and, to a lesser extent, men, prepubertal children, and adolescents. The etiology of LS is still unknown. Hormonal status, frequent trauma and autoimmune diseases are well-known associations for LS, yet infections do not seem to be clear risk factors. LS pathogenesis involves factors such as a genetic predisposition and an immune-mediated Th1-specific IFNγ-induced phenotype. Furthermore, there is a distinct expression of tissue remodeling associated genes as well as microRNAs. Oxidative stress with lipid and DNA peroxidation provides an enabling microenvironment to autoimmunity and carcinogenesis. Circulating IgG autoantibodies against the extracellular matrix protein 1 and hemidesmosome may contribute to the progression of LS or simply represent an epiphenomenon. The typical clinical picture includes chronic whitish atrophic patches along with itching and soreness in the vulvar, perianal and penile regions. In addition to genital scarring, and sexual and urinary dysfunction, LS may also lead to squamous cell carcinoma. Disseminated extragenital LS and oral LS are also reported. The diagnosis is usually clinical; however, a skin biopsy should be performed in case of an unclear clinical picture, treatment failure or suspicion of a neoplasm. The gold-standard therapy is the long-term application of ultrapotent or potent topical corticosteroids and, alternatively, topical calcineurin inhibitors such as pimecrolimus or tacrolimus. Collectively, LS is a common dermatological disease with a so far incompletely understood pathogenesis and only limited treatment options. To foster translational research in LS, we provide here an update on its clinical features, pathogenesis, diagnosis and (emerging) treatment options.
PubMed: 36873861
DOI: 10.3389/fmed.2023.1106318 -
Modern Pathology : An Official Journal... Oct 2022Vulvar squamous cell carcinomas and their precursors are currently classified by the World Health Organization based on their association with high-risk human... (Review)
Review
HPV-independent, p53-wild-type vulvar intraepithelial neoplasia: a review of nomenclature and the journey to characterize verruciform and acanthotic precursor lesions of the vulva.
Vulvar squamous cell carcinomas and their precursors are currently classified by the World Health Organization based on their association with high-risk human papillomavirus (HPV). HPV independent lesions often harbor driver alterations in TP53, usually seen in the setting of chronic vulvar inflammation. However, a group of pre-invasive vulvar squamous lesions is independent from both HPV and mutant TP53. The lesions described within this category feature marked acanthosis, verruciform growth and altered squamous maturation, and over the last two decades several studies have added to their characterization. They have a documented association with verrucous carcinoma and conventional squamous cell carcinoma of the vulva, suggesting a precursor role. They also harbor recurrent genomic alterations in several oncogenes, mainly PIK3CA and HRAS, indicating a neoplastic nature. In this review, we provide a historical perspective and a comprehensive description of these lesions. We also offer an appraisal of the terminology used over the years, going from Vulvar Acanthosis with Altered Differentiation and Verruciform Lichen Simplex Chronicus to Differentiated Exophytic Vulvar Intraepithelial Lesion and Vulvar Aberrant Maturation, the latter term having been recently proposed by the International Society for the Study of Vulvovaginal Diseases. In line with the recognition of these lesions by the 2020 World Health Organization Classification of Tumours as a neoplastic precursor, we herein propose the term HPV-independent, p53-wild-type verruciform acanthotic Vulvar Intraepithelial Neoplasia (HPVi(p53wt) vaVIN), which better conveys not only the pathology but also the neoplastic nature and the biologic risk inherent to these uncommon and challenging lesions. We outline strict morphologic and immunohistochemical criteria for its diagnosis and distinction from mimickers. Immunohistochemistry for p16 and p53 should be performed routinely in the diagnostic work-up of these lesions, and the morphologic alternative term vaVIN should be reserved for instances in which p16/HPV/p53 status is unknown. We also discuss management considerations and the need to further explore precursors within and beyond the spectrum of verruciform acanthotic vulvar intraepithelial neoplasia.
Topics: Biological Products; Carcinoma in Situ; Carcinoma, Squamous Cell; Class I Phosphatidylinositol 3-Kinases; Female; Humans; Papillomaviridae; Papillomavirus Infections; Precancerous Conditions; Squamous Intraepithelial Lesions; Tumor Suppressor Protein p53; Vulva; Vulvar Neoplasms
PubMed: 35437330
DOI: 10.1038/s41379-022-01079-7 -
International Journal of Gynaecology... Oct 2021Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. There is no specific screening and the most effective strategy to reduce...
Vulvar cancer is an uncommon gynecological malignancy primarily affecting postmenopausal women. There is no specific screening and the most effective strategy to reduce vulvar cancer incidence is the opportune treatment of predisposing and preneoplastic lesions associated with its development. While vulvar cancer may be asymptomatic, most women present with vulvar pruritus or pain, or have noticed a lump or ulcer. Therefore, any suspicious vulvar lesion should be biopsied to exclude invasion. Once established, the most common subtype is squamous cell carcinoma. Treatment of vulvar cancer depends primarily on histology and surgical staging. Treatment is predominantly surgical, particularly for squamous cell carcinoma, although concurrent chemoradiation is an effective alternative, particularly for advanced tumors. Management should be individualized and carried out by a multidisciplinary team in a cancer center experienced in the treatment of these tumors.
Topics: Carcinoma, Squamous Cell; Chemoradiotherapy; Female; Humans; Neoplasm Staging; Vulva; Vulvar Neoplasms
PubMed: 34669204
DOI: 10.1002/ijgo.13881 -
Deutsches Arzteblatt International Feb 2020In Germany, 17-23% of the population suffers from chronic itching of the skin; in 5-10% of cases, the female genitalia are affected, specifically, the vulva. Vulvar... (Review)
Review
BACKGROUND
In Germany, 17-23% of the population suffers from chronic itching of the skin; in 5-10% of cases, the female genitalia are affected, specifically, the vulva. Vulvar pruritus is thus a common symptom that often markedly impairs the affected women's quality of life.
METHODS
This review is based on pertinent publications that were retrieved by a selective search in MEDLINE/PubMed for articles on the pathogenesis, diagnosis, and treatment of vul- var pruritus. The search terms were (in German and English) "vulvärer Juckreiz," "pruritus vulvae," and "genital itch," alone and in combination with "Behandlung," "Therapie," or "treat- ment."
RESULTS
The most common cause of vulvar pruritus is vulvo- vaginal candidiasis followed by chronic dermatoses, such as lichen sclerosus and vulvar eczema. Especially in refractory cases, an invasive or preinvasive lesion such as squamous epithelial dysplasia (VIN, vulvar intraepithelial neoplasia) should be borne in mind in the differential diagnosis. Rarer causes include infection, atrophy, and vulvodynia. The essen- tial elements of treatment are topical/oral antimycotic drugs and high-potency glucocorticoids, along with consistently ap- plied, basic moisturizing care and the avoidance of potential triggering factors.
CONCLUSION
As vulvar pruritus has multiple causes, standard- ization of its diagnostic evaluation and treatment would be l efficacy and to meet the diverse needs of women who suffer from this condition.
Topics: Female; Germany; Humans; Pruritus Vulvae
PubMed: 32181734
DOI: 10.3238/arztebl.2020.0126 -
American Journal of Clinical Dermatology Sep 2021Ten percent of all women have pigmented vulvar lesions. Fortunately, most of these are benign but 1% of all melanomas in women affect the vulva. While the mortality rate... (Review)
Review
Ten percent of all women have pigmented vulvar lesions. Fortunately, most of these are benign but 1% of all melanomas in women affect the vulva. While the mortality rate of cutaneous melanoma has dropped by 7% annually during the last 5 years, the prognosis of vulvar melanoma remains dismal: the 5-year overall survival rate is 47% compared with 92% for cutaneous melanoma. The current evidence suggests that this likely results from a combination of delayed diagnosis and different tumor biology, treatment strategies, and treatment response. Although many landmark trials on checkpoint inhibitors included mucosal and vulvar melanomas, the results were often not reported separately. Post-hoc analyses indicate overall response rates between 19 and 37% for checkpoint inhibitors. A recently published retrospective study on vulvar melanomas suggests an objective response in 33.3% with a similar safety profile to cutaneous melanoma. Tyrosine kinase inhibitors may be considered in recurrent disease if a c-KIT mutation is present.
Topics: Diagnosis, Differential; Female; Humans; Melanoma; Skin Neoplasms; Vulva; Vulvar Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 34125416
DOI: 10.1007/s40257-021-00614-7 -
BMJ Case Reports Feb 2020Genital tuberculosis (TB) in women is a chronic disease with low-grade symptoms. Genital tract tuberculosis is usually secondary to extragenital TB. The fallopian tubes...
Genital tuberculosis (TB) in women is a chronic disease with low-grade symptoms. Genital tract tuberculosis is usually secondary to extragenital TB. The fallopian tubes are most commonly affected, and along with endometrial involvement, it causes infertility in such patients. Involvement of the cervix and the vulva is very rare. We present one such rare case of vulvar tuberculosis presented with a large ulcer diagnosed on histopathology and treated with antitubercular chemotherapy.
Topics: Aged; Antitubercular Agents; Female; Humans; Tuberculosis, Female Genital; Vulva
PubMed: 32051158
DOI: 10.1136/bcr-2019-232880 -
Proceedings (Baylor University. Medical... 2022Syringomas on the vulvar skin are relatively rare and generally bilateral. They are usually asymptomatic but can be symptomatic, with vulvar discomfort, burning, and/or...
Syringomas on the vulvar skin are relatively rare and generally bilateral. They are usually asymptomatic but can be symptomatic, with vulvar discomfort, burning, and/or pruritus. Management options include topical steroids, topical retinoids, and oral antihistamines. Cases refractory to conservative treatment may require procedural intervention, such as cryotherapy, excision, or electrosurgery. Here we describe a case of symptomatic vulvar syringomas refractory to medical management that were successfully treated with electrodessication and curettage.
PubMed: 34970057
DOI: 10.1080/08998280.2021.1977076 -
Indian Journal of Sexually Transmitted... 2020Lichen sclerosus (LS) was first described by Hallopeau in 1887. It is a chronic inflammatory condition most commonly involving the anogenital region with a relapsing... (Review)
Review
Lichen sclerosus (LS) was first described by Hallopeau in 1887. It is a chronic inflammatory condition most commonly involving the anogenital region with a relapsing course and a potential for destruction, functional impairment, atrophy, and malignant changes. LS affects both sexes with a female preponderance of 5:1. The exact prevalence of the disease is difficult to predict as the lesions are asymptomatic in the initial phase and later when the complications arise patients might visit the surgeon, pediatrician, gynecologist, or urologist. The etiology of LS has a complex interplay of genetic factors, autoimmunity, infections, and trauma. Physical examination to assess the extent of the disease and decide the line of management is the most crucial step in the management. Corticosteroids, calcineurin inhibitor, retinoids, phototherapy, and surgery can be helpful. Self-examination and long-term follow-up are necessary.
PubMed: 33062974
DOI: 10.4103/ijstd.IJSTD_49_17 -
Urology Jul 2022Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the... (Review)
Review
Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (Part I) and benign and malignant vulvar neoplasms (Part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.
Topics: Female; Humans; Skin; Skin Neoplasms; Vulva; Vulvar Diseases; Vulvar Neoplasms
PubMed: 35218865
DOI: 10.1016/j.urology.2022.02.007