Vulvar lichen sclerosus in girls and adult females: A single-center retrospective study of 744 patients in China.

Vulvar lichen sclerosus (VLS) is a chronic, inflammatory disease which is accompanied by itching and pain, affecting the patient's daily life and sexual activity. However, the disease characteristics of children and adults are not completely the same. Currently, there are few studies in China that compare the characteristics of VLS between girls and adult female patients. The aim of this study was to compare the epidemiology, clinical features, and combined autoimmune diseases of VLS patients between girls and adult females, and to help clinicians better understand VLS in different age groups. We enrolled 744 female patients for analysis, divided by age into a child group (<18 years) and an adult group (≥18 years). Among girl patients, 94.6% had preadolescent onset, while among adult female patients, only 4.6% had preadolescent onset, which was a statistically significant difference. The highest percentage of adult female patients had onset during their child-bearing period (75.4%), while 20% had postmenopausal onset, with a significant difference when the three onset states were compared. White patches were equally common in both girl and adult female patients' external genital area, while mossy lesions and labia minora atrophy were more common in adult female patients. Involvement of the clitoris, labia minora, and vaginal opening area were more common in adult patients. The perianal area was more commonly involved in girl patients. We found eight cases (1.2%) of secondary squamous cell carcinoma in adult female patients. We also found that 13 patients had concurrent lichen sclerosus lesions on the vulva and extragenital region, including two girls and 11 adult females. Extragenital lichen sclerosus (EGLS) occurred mostly in the torso. Clinicians should be aware of these differences so that early diagnosis and treatment of the disease can be achieved, to avoid irreversible anatomical alterations and the risk of cancer.

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

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.

Lichen Sclerosus of the Lip.

In this case report, we outline a case of a 36-year-old woman who presented to the dermatology clinic with a history of a hypopigmented macule on her lip. After conducting hepatitis C antibody testing and a shave biopsy, the patient was diagnosed with lichen sclerosus. Because of the increased risk for squamous cell carcinoma, she underwent an anogenital exam, where no lesions were found.

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

Lichen sclerosus (LS) is a chronic inflammatory skin disease commonly affecting the anogenital area with less frequent extragenital occurrence. Extragenital LS cutaneous manifestations vary and precipitating factors are not well described. Recent evidence for etiology and clinical associations of extragenital LS provide insight into disease recognition and pathogenesis. Novel diagnostic techniques as well as treatment standardization have the potential to improve management of this rare condition. This review details both past and new insights into the pathogenesis, clinical manifestations, and treatment options of extragenital LS.

Dermoscopic Evaluation of Extragenital Lichen Sclerosus et Atrophicus.

Introduction: Lichen sclerosus (LS), is an uncommon inflammatory dermatosis with preferential involvement of anogenital region. Diagnosis of LS is mainly clinical, but clinical differentiation from conditions like vitiligo, morphea may be a difficult task at times that often requires histological analysis. Dermoscopy is one such non-invasive tool which can help diagnose the disease. There is paucity of Indian data on dermoscopy of LS. Objectives: To evaluate clinical, dermatoscopic patterns of LS and correlate them with histopathology. Methods: The study was conducted in a tertiary hospital after obtaining consent from 20 patients. OITEZ e-scope digital microscope was used to evaluate the lesions. Both polarized and nonpolarized mode were used and skin biopsy was done to confirm diagnosis. Results: Based on morphology, LS was classified as scleroatrophic lesions (61.5%), guttate lesions (30.8%) and hyperkeratotic lesions (7.7%). Dermoscopic analysis revealed structureless white to yellow areas as most common finding (100%) followed by chrysalis like structure (80.8%). Linear irregular vessels were seen in 61.5% lesions and perifollicular scaling in 50.0% lesions. Keratotic plugs were seen in 50.0% lesions. A new characteristic finding, "rosettes" was seen in 38.5% lesions has never been reported with LS before. Non polarized mode was particularly useful for identifying texture changes, keratotic plugs and minute scales which were not visible otherwise. Conclusions: Dermoscopy is a simple diagnostic tool that helps in the early diagnosis of LS with specific pattern which can avoid invasive procedure like biopsy. Both non-polarised and polarized dermoscopy must be done to visualize the changes of LS well.

Extragenital lichen sclerosus: A comprehensive review.

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 cluster in plaques and slowly progress over time resulting in parchment-like skin usually involving upper trunk, neck and shoulders. Lesions are frequently accompanied by purpura/haemorrhagic spots. The relationship with morphoea has been a topic of debate. Association with several autoimmune diseases has been observed. Diagnosis is usually based on clinical and dermoscopic examination and further supported by histopathological findings. LS needs to be differentiated from several other dermatological conditions such as discoid lupus erythematosus, vitiligo, mycosis fungoides (hypopigmented variant), lichen planus, graft-versus-host disease and morphoea depending upon the stage of the disease. Generally, extragenital LS is believed to lack carcinogenic potential. However, case reports with possible malignant transformation have been described. In this article, the authors have described a concise review of the extragenital form of LS.

Ink test: A useful technique to enhance extragenital lichen sclerosus diagnosis.

Lichen sclerosus (LS) is a rare and chronic autoimmune disease, and extragenital LS is particularly challenging to diagnose in children. Histopathology is the gold standard for diagnosis. Dermoscopy is a useful tool when follicular plugs (FP) are observed, though FP are sometimes hard to recognize. The ink test may provide a useful technique to enhance FP visualization and support a diagnosis of extragenital LS, thereby avoiding diagnostic delays or biopsy.

Development of Extragenital Lichen Sclerosus in Malignant Melanoma Patients Treated With Ipilimumab in Combination With Nivolumab.

The immune checkpoint inhibitors opened a new era for the treatment of melanoma. Nowadays, combined immune checkpoint inhibitors are administered to provide additive or synergistic effects on anti-melanoma immunity. The use of these drugs comes with serious adverse events related to excessive immune activation. Here, we present development of extragenital lichen sclerosus in a patient with metastatic malignant melanoma, during the combined therapy with checkpoint inhibitors.

High-frequency ultrasonography (HFUS) as a useful tool in differentiating between plaque morphea and extragenital lichen sclerosus lesions.

INTRODUCTION: Morphea and lichen sclerosus (LS) are chronic inflammatory diseases that may pose a diagnostic challenge for a physician. High-frequency ultrasonography (HFUS) is a versatile diagnostic method utilized in dermatologic practice, allowing monitoring the course of the disease, treatment response and differentiation between certain skin disorders. AIM: To prove the usefulness of HFUS in differentiating between plaque morphea and extragenital LS lesions. MATERIAL AND METHODS: We examined 16 patients with plaque morphea and 4 patients with extragenital LS using 20 MHz taberna pro medicumTM (Germany) device. RESULTS: Investigations revealed hyperechogenic entrance echo in both morphea and LS lesions, whereas a distinct polycyclic surface of the entrance echo was detected exclusively in LS. CONCLUSIONS: High-frequency ultrasonography is a current diagnostic modality that may prove useful in differentiating between morphea and LS lesions.

Liquen escleroso genitaly extragenital: a propósito de 4 casos / Genital and extragenital lichen sclerosus: report of 4 cases

El liquen escleroso (LE) es una enfermedad mucocutánea inflamatoria crónica de etiología desconocida. Se lo ha vinculado con diversos factores inmunológicos, genéticos, hormonales, infecciosos y traumáticos. Se presenta generalmente en mujeres entre la cuarta y quinta década de la vida y su localización habitual es la genital. Las formas extragenitales son poco frecuentes y asientan en tronco, cuello y extremidades. Debido a que los síntomas que provoca en el área genital, como el prurito, dispareunia, dolor y disuria alteran la calidad de vida del paciente, es fundamental realizar un diagnóstico en forma temprana y el tratamiento adecuado. Presentamos cuatro casos de LE genital y extragenital.

Lichen sclerosus is a chronic inflammatory mucocutaneous disease of unknown aetiology. It has been linked to various immunological, genetic, hormonal, infectious and traumatic factors. It usually appears in women between fourth and fifth decade of life and usual location is genital area. Extragenital forms are rare and located on trunk, neck and extremities. Because the symptoms affecting genital area, such as itch, dyspareunia, pain and dysuria, may cause a high impact on the quality of life of patients, early diagnosis and appropiate treatment are essential. Four cases of genital and extragenital lichen sclerosus are reported.

Clinicopathologic Comparison of Genital and Extragenital Lichen Sclerosus et Atrophicus / 대한피부과학회지

BACKGROUND: Lichen sclerosus et atrophicus is a persistent inflammatory dermatosis of unknown etiology with a predilection for the genital area. Although there were many case reports in Korea, there are no studies regarding the clinicopathologic comparison of genital and extragenital lichen sclerosus et atrophicus. OBJECTIVE: The aim of our study was to evaluate the clinicopathologic characteristics and differences between genital and extragenital lichen sclerosus et atrophicus. METHODS: Retrospective analysis was performed by reviewing the clinicopatholgic records of 33 patients who were diagnosed with lichen sclerosus et atrophicus from 2000 to 2006 in Yonsei University Severance Hospital. RESULTS: The most common clinical manifestation is a whitish patch with pruritus on labia minor. The ratio of male to female patients in genital and extragenital lichen sclerosus et atrophicus were 1:10.5 and 1:2.3 respectively. Disease onset ages were 49.9 years and 44.2 years respectively. The most common subjective symptom was pruritus. However, no symptom was more significant in extragenital lichen sclerosus et atrophicus compared to genital lichen sclerosus et atrophicus. Most of the lesions presented as whitish patches and plaques but atrophy, erythema and lichenification could also occur. Histopathologic findings of the extragenital lichen sclerosus et atrophicus showed more significant epidermal thinning and cleft formation compared to genital lichen sclerosus et atrophicus, which suggests that extragenital lichen sclerosus et atrophicus shows more evolved lesions. A few cases of genital lichen sclerosus et atrophicus showed spongiotic dermatitis, lichen simplex chronicus-like and lichen planus-like features in addition to typical pathology, which were suspected as secondary features or early lesions. All the patients were treated with high to mid-potency topical corticosteroid which were effective in both the genital and extragenital lichen sclerosus et atrophicus. There was no cases of squamous cell carcinoma arising in lichen sclerosus et atrophicus during the follow-up. CONCLUSION: Clinically, there were no symptoms significant to extragenital lichen sclerosus et atrophicus and pathologically extragenital lichen sclerosus et atrophicus showed more significant epidermal thinning and cleft formation. Further research regarding the characteristics and differences between genital and extragenital lichen sclerosus et atrophicus should be performed on larger number of cases.