Objective The aim of the German-language S3 guideline on lichen sclerosus (LS) is to provide an evidence- and consensus-based foundation for the diagnosis and management of this disease. The present publication offers a concise summary of the relevant content, focusing on genital LS in girls and women. This first part addresses, among other aspects, the definition, symptomatology, and diagnosis of LS, as well as its management during pregnancy. The separately published second part focuses on the treatment of LS. Methods The German-language, evidence- and consensus-based S3 guideline on LS was developed under the leadership of the German Society of Gynecology and Obstetrics (DGGG) and the German Dermatological Society (DDG), based on the European "EuroGuiDerm Guideline on Lichen Sclerosus." The interdisciplinary guideline panel included 24 experts from 16 professional societies and actively involved patient representatives in the development process. Recommendations LS is a chronic inflammatory skin disease with predominant manifestation in the anogenital region. It may be associated with pruritus, pain, sexual dysfunction, urinary symptoms, and constipation, although it can occasionally remain asymptomatic. Awareness of the broad spectrum of symptoms, as well as reversible and irreversible clinical signs, is essential to enable early diagnosis and appropriate treatment. Early and adequate therapy can not only alleviate symptoms but also reduce the risk of permanent damage and the development of associated carcinomas. During pregnancy, treatment with topical glucocorticoids should be continued as needed to control disease activity and prevent complications.

Lichen sclerosus (LS) is a chronic inflammatory dermatosis of the anogenital region with limited therapeutic options. Emerging evidence implicates dysregulation of the Janus kinase (JAK)-signal transducer and activator of transcription (STAT) pathway in LS pathogenesis, supporting investigation of targeted therapies. We report a series of 6 patients treated with topical ruxolitinib for vulvar LS, applied twice daily. Clinical outcomes were assessed using the Clinical Scoring System (CSS) and Patient Global Assessment (PtGA) over a mean follow-up of 7.5 months. All patients reported early symptomatic improvement in pruritus and burning. In parallel, PtGA scores showed significant improvement (p < 0.05). No local or systemic adverse events were observed. These findings suggest that topical JAK-STAT inhibition may represent a steroid-sparing option for achieving symptomatic improvement in LS, particularly in patients with concomitant autoimmune conditions. Larger controlled studies are warranted to confirm these preliminary observations.

Lichen sclerosus (LS) is a chronic, lymphocyte-mediated inflammatory skin disease characterized by porcelain-white atrophic skin lesions. Localized LS is relatively common and predominantly occurs in the anogenital region. Generalized LS involving both genital and extragenital sites is clinically rare and frequently refractory to conventional therapies like topical corticosteroids. As an emerging targeted therapy, Janus kinase (JAK) inhibitors demonstrate broad therapeutic potential. The highly selective JAK1 inhibitor upadacitinib demonstrates robust efficacy across various inflammatory dermatoses; however, reports of its application in LS remain scarce. Herein, we report the case of a 62-year-old female patient with refractory generalized LS who presented with a 2-year course. The skin lesions involved the trunk, extremities and vulvar region, and failed to respond to multiple conventional treatments. Upadacitinib at a dose of 15 mg daily was administered to the patient. During the 10-month follow-up period, pruritus was significantly alleviated, and skin sclerosis as well as atrophic patches showed marked improvement. No new blisters or skin lesions emerged, and no severe adverse reactions were observed. This case supports the effectiveness of upadacitinib in refractory generalized LS and offers a brief review of relevant literature.

Vulvar pruritus refers to itching affecting the skin and mucosal surfaces of the external genital and perineal regions. It is most frequently associated with infections, inflammatory skin disorders, or neoplastic conditions. Due to the distinctive anatomical and physiological features of the anogenital area, clinical manifestations in this region are often subtle or atypical, which can complicate both diagnosis and management. Because vulvar itch can be highly distressing, timely identification and appropriate intervention are crucial for improving patient quality of life. A comprehensive clinical approach is essential when evaluating patients with vulvar pruritus. This includes a detailed medical history, focused physical examination, and relevant diagnostic testing. Management should involve elimination of contributing or exacerbating factors and treatment directed at the underlying cause. This review article discusses the common causes of vulvar pruritus, emphasizing the diagnostic approach and outlining current treatment strategies. The importance of an individualized patient-centered management plan is emphasized.

Lichen sclerosus (LS) is a chronic inflammatory condition predominantly affecting the anogenital region of postmenopausal women. It is associated with considerable aesthetic and functional impairments and an increased risk of squamous cell carcinoma. While high-potency topical corticosteroids remain the cornerstone of treatment, therapeutic options for patients with refractory LS are scarce. Fractional CO2 laser therapy has emerged as a potential second-line intervention aiming to mitigate symptoms and improve quality of life. This prospective observational study investigated the short-term efficacy and safety of fractional CO2 laser therapy in 75 women with refractory LS who underwent four treatment sessions between January 2022 and February 2024. Sixty-nine patients completed the protocol, demonstrating significant reductions in key symptoms, including pruritus (VAS score from 7.53 ± 3.02 to 4.08 ± 3.07), pain (5.83 ± 3.84 to 2.42 ± 2.85), and dyspareunia (8.26 ± 2.82 to 6.34 ± 3.30). Quality of life, sexual function, and psychological well-being also improved, as evidenced by reductions in Dermatology Life Quality Index (DLQI) scores (10.72 ± 7.25 to 5.94 ± 5.16), enhancements in sexual function (FSFI scores from 10.48 ± 8.46 to 15.52 ± 9.59), and decreased depression severity (BDI scores from 16.66 ± 12.64 to 5.94 ± 5.16). Importantly, no adverse effects were reported during the study period. Although these findings highlight the potential of fractional CO2 laser therapy as a safe and effective adjunct for refractory LS, it is essential to acknowledge the study's limitations, particularly the relatively short follow-up period. Longer-term studies are warranted to confirm sustained benefits and to evaluate the broader applicability of this approach.

Papular acantholytic dyskeratosis (PAD), often found to occur in the vulvar or anogenital area, is an exceedingly rare skin condition that usually presents in adulthood and features multiple smooth skin-colored or grayish-white papules with or without pruritus. Although the pathogenesis of PAD is unknown, PAD may be associated with mutations in ATP2C1 and ATP2A2 genes. Here, we report on an 18-year-old female patient with multiple gray-white flat papules in the anogenital area. Skin biopsy revealed hyperkeratosis of the epidermis, acantholysis, formation of fissures or lacunae, and disappearance of desmosomes. Whole exon sequencing (WES) of the patient indicated mutations in the ATP2C1 gene.

Neuropathic itch is a type of chronic pruritus resulting from neural dysfunction along the afferent pathway. It is often accompanied by abnormal sensations such as paresthesia, hyperesthesia, or hypoesthesia. This condition, which may involve motor or autonomic neural damage, significantly impacts patients' quality of life, causing severe itch and associated comorbidities such as depression, disrupted sleep, and social strain. Neuropathic itch accounts for 8% of chronic pruritus cases, though this may be underestimated. This comprehensive review focuses on nerve impingement as the primary pathophysiological mechanism for various forms of neuropathic itch including brachioradial pruritus (BRP), notalgia paresthetica (NP), and anogenital itch. BRP, often seen in middle-aged white women, manifests as pruritus in the dorsolateral forearms typically exacerbated by ultraviolet (UV) exposure and related to cervical spine pathology. NP, prevalent in middle-aged women, presents as pruritus in the upper back due to thoracic spine nerve compression. Anogenital pruritus, affecting 1-5% of adults, is often linked to lumbosacral spine issues after ruling out dermatologic conditions such as lichen sclerosus or lichen simplex chronicus. The pathophysiology of neuropathic itch involves both peripheral and central mechanisms, with nerve damage being a key factor. Diagnosis requires a thorough history, physical examination, and potentially imaging studies. Topical agents such as menthol, capsaicin, and lidocaine are used for mild cases, while systemic medications such as gabapentin, pregabalin, and antidepressants are prescribed for moderate to severe cases; however, no US Food and Drug Administration (FDA)-approved therapies currently exist specifically for neuropathic itch. Understanding the underlying neural dysfunction and appropriate therapeutic strategies is crucial for managing neuropathic itch effectively.