Abstract:Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous disease of unknown aetiology. About 85% of total cases of LS are genital cases, while extragenital form is seen in only 15-20% of cases. Extragenital LS (EGLS) can occur simultaneously with genital form; however, in 6% of the cases, only extragenital form has been described. Genetic, autoimmune, infectious, environmental and hormonal factors are implicated in its aetiology. Extragenital LS presents as asymptomatic white opalescent papules, which clu… Show more
“…It usually occurs simultaneously with gLS, but in 6% of the cases, eLS is present as an isolated entity without any genital lesions. Most cases of eLS are diagnosed in middle-aged adults ( 26 , 27 ). Nevertheless, the prevalence of eLS may be underestimated,as it is also frequently asymptomatic ( 28 ).…”
Section: Epidemiologymentioning
confidence: 99%
“…( 3 ) LS can affect any part of skin or mucosa, however, in 85% of cases, the genital mucosa is involved, and extragenital lesions are seen only in around 15–20% of cases ( 23 ). Extragenital lesions often appear simultaneously with genital lesions, but in 6% of cases only extragenital forms have been reported ( 27 ).…”
Section: Clinicsmentioning
confidence: 99%
“…Extragenital manifestations are rare and found mostly in females, with female to male ratio ranging from 6:1 to 10:1 ( 27 ). Extent of the extragenital involvement vary from small well-defined area to widespread eruption, mostly localized to submammary area, neck, shoulders, inner thighs, wrists, upper back ( Figures 2E – H ) ( 98 ).…”
Section: Clinicsmentioning
confidence: 99%
“…In 5.7% of patients, morphea coexists with LS, especially the circumscribed and generalized types ( 117 ). While in men morphea associates typically with eLS, in women the frequent relation with VLS obliges the inspection of the anogenital area ( 27 ).…”
Section: Associationsmentioning
confidence: 99%
“…Beside vitiligo and morphea, generalized eLS may be confused for graft- vs -host disease. Bullous LS may pose a diagnostic challenge in bullous lichen planus, autoimmune blistering diseases, bullous scleroderma, bullous lupus erythematosus and bullous insect bite reactions ( 27 , 139 ).…”
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.
“…It usually occurs simultaneously with gLS, but in 6% of the cases, eLS is present as an isolated entity without any genital lesions. Most cases of eLS are diagnosed in middle-aged adults ( 26 , 27 ). Nevertheless, the prevalence of eLS may be underestimated,as it is also frequently asymptomatic ( 28 ).…”
Section: Epidemiologymentioning
confidence: 99%
“…( 3 ) LS can affect any part of skin or mucosa, however, in 85% of cases, the genital mucosa is involved, and extragenital lesions are seen only in around 15–20% of cases ( 23 ). Extragenital lesions often appear simultaneously with genital lesions, but in 6% of cases only extragenital forms have been reported ( 27 ).…”
Section: Clinicsmentioning
confidence: 99%
“…Extragenital manifestations are rare and found mostly in females, with female to male ratio ranging from 6:1 to 10:1 ( 27 ). Extent of the extragenital involvement vary from small well-defined area to widespread eruption, mostly localized to submammary area, neck, shoulders, inner thighs, wrists, upper back ( Figures 2E – H ) ( 98 ).…”
Section: Clinicsmentioning
confidence: 99%
“…In 5.7% of patients, morphea coexists with LS, especially the circumscribed and generalized types ( 117 ). While in men morphea associates typically with eLS, in women the frequent relation with VLS obliges the inspection of the anogenital area ( 27 ).…”
Section: Associationsmentioning
confidence: 99%
“…Beside vitiligo and morphea, generalized eLS may be confused for graft- vs -host disease. Bullous LS may pose a diagnostic challenge in bullous lichen planus, autoimmune blistering diseases, bullous scleroderma, bullous lupus erythematosus and bullous insect bite reactions ( 27 , 139 ).…”
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.
In this case report, we outline a case of a 71‐year‐old woman who presented to the dermatology clinic with a history of keratotic and atrophic warty‐like plaques on her lower limbs, which limited the movements in her right lower extremity. A skin radial biopsy of one of the plaques was performed and the diagnosis of extragenital lichen sclerosus (ELS) was established. She underwent an anogenital exam and fortunately, lesions were not found. The patient in this case showed an unusual variant of ELS.
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