Gene/Protein Disease Symptom Drug Enzyme Compound
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A wide range of genital infections and skin conditions may present with vulval pruritus. Lichen sclerosus is one cause. This is often associated with visible skin changes which include atrophy, resorption or fusion of the labia, sclerosis and excoriation. Ulceration may indicate malignant transformation and requires urgent biopsy. Many experienced clinicians diagnose lichen sclerosus on clinical appearance, however we recommend a low threshold for biopsy, especially for non-responding or odd looking lesions. We report a patient whose vulval skin biopsy for clinically suspected lichen sclerosus revealed extramammary Paget's disease of the vulva. We review the pathology, prognosis and treatment of this condition.
Int J STD AIDS 2004 Feb
PMID:Extramammary Paget's disease masquerading as lichen sclerosus. 1500 79

Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin and mucosa, presenting to genitourinary physicians and dermatologists. It affects both sexes and all age groups. Although the exact aetiology is uncertain, genetic predisposition, infections and autoimmune factors have been implicated in its pathogenesis. Symptoms include pruritus and soreness, but asymptomatic presentations are not uncommon. The classical clinical picture is of atrophic white plaques in the anogenital region. Histopathology is specific with basal cell degeneration, upper dermal oedema, homogenization of collagen and a chronic inflammatory infiltrate. Short courses of potent topical corticosteroids form the mainstay of treatment. The condition tends to be remitting and relapsing, with spontaneous regressions reported in a few. In men, the term balanitis xerotica obliterans is sometimes used to describe late and severe LS of the penis. Scarring and progression to squamous cell carcinomas can occur in chronic LS, resulting in significant morbidity. A multidisciplinary approach to care and the need for long-term monitoring cannot be overemphasized.
Int J STD AIDS 2005 Jul
PMID:Lichen sclerosus. 1630 84

The genital area in women is covered by a keratinized squamous stratified epithelium outside the body (vulva), and a non keratinized epithelium inside the body (vagina). These characteristics can have an effect on the clinical aspects of the diseases and/or on the choice of the treatment. Symptoms (itching, pain, vaginal discharge), preferential localisation of skin diseases (psoriasis, lichen planus, lichen sclerosus, atopic dermatitis and allergic contact dermatitis, irritative dermatitis) and the aspect of primary lesions are to be investigated. The implication of this region in sexual activity places it at risk of sexually transmitted diseases (STD's) and dyspareunia. These have numerous causes that have to be sought and taken care of, often by multidisciplinary teams. After a careful history and clinical examination, additional tests allow to exclude infections or confirm a skin condition or neoplasia by a skin biopsy. If contact dermatitis is suspected, specific allergy testing is done. Treatment starts with correction of harmful habits (excessive use of soaps, inappropriate cosmetic products,...) that add to the local irritation. Patients are then reassured of common misconception regarding cancer, STD's and fertility. In the vast majority of cases, the treatment will target an infection (fungal, bacterial, STD's), will relieve irritation by the use of local immunosuppressant drugs (local corticosteroids) and/or relief itching symptoms with anti-histamine drugs.
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PMID:[How to diagnose and how to treat diseases of the genital mucosa?]. 1989 85

Forty-five cases of lichen sclerosus (LS) were retrospectively found between 2000 and 2008 among those attending an associate university teaching hospital sexually transmitted infection (STI) clinic (genitourinary [GU] medicine clinic) and 26 responders of the 45, to a questionnaire about psychological morbidity and psychiatric morbidity, were evaluated. Sixteen percent of the patients were worried about the possibility of infecting their partners with the condition, despite counselling to the contrary. Twenty-seven percent felt that the condition's cosmetic appearance adversely affected libido. There was moderate to severe anxiety at one time or another in 58% while 27% experienced depression at one time or another; 19% admitted to insomnia as a result of the condition; 23% were stressed while 11.5% were worried about starting a new relationship. LS has a profound effect on mental health. Selected patients with LS may benefit from routine referral to a clinical psychologist, within the sexually transmitted disease setting to elaborate and institute coping strategies.
Int J STD AIDS 2010 Jan
PMID:Psychological and psychiatric morbidity in lichen sclerosus in a cohort recruited from a genitourinary medicine clinic. 1991 38

Imiquimod has immune-stimulant properties that can precipitate autoimmune conditions like eczema, psoriasis and lichenoid conditions. We report two cases here where imiquimod induced florid lichen sclerosus in one patient and lichen planus in another. In both patients the condition was so aggressive and unresponsive to steroid treatment that circumcision was necessary.
Int J STD AIDS 2010 Mar
PMID:Imiquimod use in the genital area and development of lichen sclerosus and lichen planus. 2021 34

The aim of this study was to review the utility of penile biopsy in genitourinary (GU) medicine clinics, its acceptability and the range of penile dermatoses diagnosed histologically. A retrospective case-notes review of 401 cases attending a dedicated penile dermatoses clinic from 1 June 2001 to 30 November 2007 was carried out. In 115/401 (28.7%) of cases a biopsy was invoked to resolve ambiguities of diagnosis. In 60/401 (15%) of those cases differential diagnosis was resolved, in 26/401 (6.5%) clinically suspected diagnoses were confirmed and in 22/401 (5.5%) clinically unsuspected diagnoses were identified. In 7/401 (1.7%) of cases the biopsy was also the treatment. Targeted penile biopsy has an important role in the diagnosis of penile dermatoses, particularly where there is clinical uncertainty. As a diagnostic tool in GU medicine departments it should be readily available even if not for routine use. There were indications that circumcision may reduce the incidence of penile dermatoses. There was also some indication of a possible ethnic factor in the relative incidences of penile lichen sclerosus and lichen planus in biopsied patients.
Int J STD AIDS 2010 May
PMID:Role of penile biopsy in the diagnosis of penile dermatoses. 2172 70

A retrospective case-notes review was undertaken of all women with lichen sclerosus seen during a 12-month period to review their characteristics and care. A total of 273 case-notes were reviewed. The mean age was 61 years (range, 14-94), and the mean duration of symptoms was 6.4 years (range, 1-50). The mean age at diagnosis was 55 years (range, 7-92). Sixty-two (23%) had at least one other autoimmune condition. Autoimmune conditions were seven times more frequent overall compared with United Kingdom prevalences. On-going symptoms were reported as none/minimal in 196 (72%), moderate in 65 (24%) and severe in 12 (4%). A total of 233 women (85%) had on-going treatment with clobetasol propionate (Dermovate) ointment with a mode of eight applications per month (range, 0-30). Forty-three women (16%) reported sexual dysfunction and 13 (5%) had needed at least one surgical procedure to restore sexual function. Eighty-six (32%) had undergone at least one biopsy. Nine squamous cell cancers (3%) had been diagnosed in six women (2%). These patients were managed in line with all current guidance. It is surprising that there is still no evidence to direct long-term management.
Int J STD AIDS 2015 Apr
PMID:The management of lichen sclerosus in a genitourinary medicine setting: a 12-month retrospective case-notes review. 2490 46

Several factors such as genetic susceptibility, autoimmunity, hormones, infections, local trauma, urine, and occlusion have been speculated to play a role in the pathogenesis of lichen sclerosus. We report two male patients with lichen sclerosus around 'ectopic' urethral openings and the opposing surfaces of the penile shaft and scrotum, providing further evidence in support of urine and occlusion as contributing factors in the development of lichen sclerosus.
Int J STD AIDS 2017 08
PMID:Genital lichen sclerosus developing around 'ectopic' urethral orifices supports the role of occlusion and urine in its pathogenesis. 2808 82

Vulval lichen planus-lichen sclerosus overlap is an emerging observation. Few clinical reports exist with no reviews of literature. We present a focused update of this phenomenon and discuss a clinical case. We report a 63-year-old woman with a 20-year history of ulcerative vulvo-vaginitis, initially diagnosed as benign mucous membrane (cicatricial) pemphigoid. This led to prolonged treatment with oral corticosteroids with minimal improvement in symptoms. Subsequent complications of long-term use of systemic corticosteroid ensued. A clinico-pathological diagnosis of severe erosive lichen planus was made on clinical findings and on non-specific biopsy changes of ulceration and inflammation. Treatment with topical clobetasol propionate 0.05% ointment twice daily led to dramatic improvement of ulceration, easing of discomfort and marked improvement in quality of life. Clinical examination revealed Wickham's striae on the labia majora supporting the diagnosis. Six years after commencement of topical clobetasol, white plaques were noticed on the labia majora, perineum and peri-anal region consistent with lichen sclerosus, confirmed by repeat vulval skin biopsy and on vulvectomy. This case highlights the challenge of diagnosis of extensive vulvo-vaginal ulceration and the necessity to re-examine a previous diagnosis if there is poor response to treatment.
Int J STD AIDS 2018 09
PMID:Vulval lichen planus-lichen sclerosus overlap. 2949 May 71

BackgroundThe pathogenesis of male genital lichen sclerosus (MGLSc) is controversial. Incriminated factors include infection with human papillomavirus (HPV) and autoimmunity (e.g. Human Leukocyte Antigen [HLA]). To address the roles of HLA and HPV in MGLSc we studied adult Caucasian males with a clinical and histological diagnosis of MGLSc. The men in the study attended two specialised Male Genital Dermatoses Clinics between July 2011 and September 2012 and were selected and phenotyped from the clinical records. DNA was extracted from blood and paraffin-embedded biopsy sections, for HLA and HPV typing, respectively. HLA allele frequencies were compared with those derived from the UK-based Caucasian population. Eighty-eight cases of MGLSc were identified. HPV DNA was detected in 33/88 (37.5%) cases of MGLSc. HPV16 was the most prevalent type found: 11/88 (12.5%) MGLSc. No statistically significant HLA associations were established but HLA-B*35, -B*51, -C*15, -DRB1*04, -DRB1*10 (predisposition) and -DQA1*01 (protection) were revealed as alleles of interest. HPV16-associated MGLSc cases showed no statistically significant association with HLA genotype. The relationship between HPV and MGLSc suggests a passenger effect rather than a pathogenic role. HLA is not associated with MGLSc nor co-existent HPV16.
Int J STD AIDS 2020 Dec
PMID:Immunogenetics and human papillomavirus (HPV) in male genital lichen sclerosus (MGLSc). 3308 49


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