Extragenital lichen sclerosus: a comprehensive review of clinical features and treatment

Burshtein, Aaron; Burshtein, Joshua; Rekhtman, Sergey

doi:10.1007/s00403-022-02397-1

Cited by 6 publications

(8 citation statements)

References 105 publications

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“… 8 Moreover, evidence showed an increased rate of squamous cell carcinoma in genital LS with 0.4–6% prevalence. 9 In contrast, extragenital LS is usually asymptomatic and exhibits less association with malignant transformation, 10 with only two reported cases of squamous cell carcinoma arising from extragenital LS lesions on the leg and buttock. 11 , 12 To our knowledge, malignant change has never been documented among previously reported oral LS cases.…”

Section: Discussionmentioning

confidence: 99%

Lichen Sclerosus of the Labial Mucosa: A Case Report and Literature Review

Phuwaraks,

Rutnin,

Suchonwanit

2024

CCID

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Lichen sclerosus (LS) is an uncommon, chronic, inflammatory mucocutaneous disorder found predominantly in females with unknown etiology. It presents as a white sclerotic plaque commonly located on the anogenital area. Extragenital LS is less prevalent, and LS affecting the oral mucosa is extremely rare, with only 39 biopsy-confirmed cases reported in the literature. Due to its several mimicking conditions, histological examination is usually required for a definitive diagnosis, particularly in patients with oral LS. Current evidence-based treatment recommendations for oral LS are unavailable; however, most cases tend to improve after treatment with topical or intralesional corticosteroids. We report a case of a 58-year-old female referred from the otolaryngology department for evaluating an asymptomatic whitish sclerotic plaque on the lower lip mucosa that had existed for 1 year. Following a punch biopsy, the patient was diagnosed with LS of labial mucosa. The condition improved after 2 months of treatment with topical and intralesional corticosteroids. The present case report raises awareness in recognizing oral LS and contributes to knowledge of this rare disorder.

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Section: Discussionmentioning

confidence: 99%

Lichen Sclerosus of the Labial Mucosa: A Case Report and Literature Review

Phuwaraks,

Rutnin,

Suchonwanit

2024

CCID

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“…There was a statistically significant decrease in the median DLQI score after treatment (median 2.0 [IQR, 1-5]) compared to before (median 6.0 [IQR, [1][2][3][4][5][6][7][8][9][10][11]), presented in Table 3. There were significant differences in DLQI scores between men and women before treatment (median 3.0 in men and 8.0 in women) and after treatment (median 1.0 in men and 4.0 in women).…”

Section: Total Dlqi Scorementioning

confidence: 99%

“…1 It most commonly occurs in the anogenital region in both sexes, but some patients may have an extragenital presentation. 2,3 The prevalence of LS is approximately 1.7% in females 4 and 0.07% in males. 5 The symptoms a Department of Dermatology and Venereology, Ryhov County Hospital, Sjukhusgatan, Jönköping, Sweden b Division of Cell Biology, Department of Biomedical and Clinical Sciences, The Faculty of Medicine and Health Sciences Linkoping University, Linköping, Sweden What is known about this subject in regard to women and their families?…”

Section: Introductionmentioning

confidence: 99%

The impact of genital lichen sclerosus in men and women on quality of life: a prospective cohort study

Jerkovic Gulin,

Liljeberg,

Seifert

2024

International Journal of Women’s Dermatology

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Background: Genital lichen sclerosus (LS) is a chronic inflammatory skin disorder that affects both sexes of all ages. The clinical characteristics include erosions, redness, and white plaques with atrophic skin, with symptoms such as pruritus, pain, dysuria, and dyspareunia. Objective: This prospective cohort study aimed to assess quality of life (QoL) in men and women with genital LS, both before and after treatment, using the Dermatology Quality of Life Index (DLQI) questionnaire. Methods: Patients diagnosed with genital LS were enrolled continuously in the study and were asked to complete the DLQI questionnaire before treatment and again after individualized treatment 12 weeks apart. Results: This study included 136 patients (48 females and 88 males) diagnosed with genital LS, with a median age of 62 years (range 18–86). The results showed a statistically significant decrease (P < .001) in DLQI score before treatment (median 6.0 [interquartile range (IQR), 1.0–11.0]) compared to after treatment (median 2.0 [IQR, 0.0–4.0)]. In males and females, the median DLQI scores before treatment were 3.0 (IQR, 0.0–10.0) and 8.0 (IQR, 4.5–11.5), respectively, and after treatment were 1.0 (IQR, 0.0–3.0) and 4.0 (IQR, 0.0–9.0), respectively. Females scored significantly higher (P < .001) than males. Limitations: The study’s limited generalizability stems from a small sample size of 136 patients, potentially restricting the application of findings to a broader population with genital lichen sclerosus. Additionally, the 12-week follow-up period may not adequately capture the long-term effects of interventions on quality of life. Reliance on self-reported data through the DLQI questionnaire introduces the possibility of bias, as participants may not accurately represent their symptoms and quality of life. The absence of a control group hinders the ability to attribute observed changes solely to the treatment, and the lack of detail on specific interventions makes it challenging to assess the effectiveness of individualized treatment approaches. The wide age range among participants (18–86 years) introduces potential confounding variables, as different age groups may respond differently to treatment. Conclusion: The study findings confirmed that individuals with genital LS experience a small decline in QoL, as observed in both males and females. This study also highlights that effective management of genital LS can significantly improve QoL in both sexes.

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“…In some cases, the typical LS lesions are accompanied by telangiectasias, and hemorrhagic- or non-hemorrhagic bullae. Bullous lesions may lead to ulcerations or erosions that turn into white sclerotic plaques ( 100 – 102 ). The Koebner phenomenon is often observed, with extragenital manifestations of LS arising at pressure points, sites of surgical scars or traumas ( 19 ).…”

Section: Clinicsmentioning

confidence: 99%

Lichen sclerosus: The 2023 update

et al. 2023

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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.

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Extragenital lichen sclerosus: a comprehensive review of clinical features and treatment

Cited by 6 publications

References 105 publications

Lichen Sclerosus of the Labial Mucosa: A Case Report and Literature Review

Lichen Sclerosus of the Labial Mucosa: A Case Report and Literature Review

The impact of genital lichen sclerosus in men and women on quality of life: a prospective cohort study

Lichen sclerosus: The 2023 update

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