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A nested case-control study using the National Institutes of Health All of Us database was conducted, with patients ≥ 18 years with AA diagnosis matched with controls 1:4 by age, sex at birth, and race/ethnicity. Demographic characteristics of alopecia areata and control patients matched by age and self-reported race/ethnicity Controls (n = 3,828) Alopecia areata (n = 957) P value Age, mean (std) 55.8 (15.2) 55.8 (15.2) 1 Sex at birth  Male 892 (23.3) 223 (23.3) 1  Female 2,836 (74.1) 709 (74.1)  Other 100 (2.6) 25 (2.6) Self-reported race/ethnicity count (%)  White 1,560 (40.8) 390 (40.8) 1  Hispanic or Latino 956 (24.9) 239 (24.9)  Black or African American 816 (21.3) 204 (21.3)  Asian 168 (4.4) 42 (4.4)  Other 328 (8.6) 82 (8.6) Table 2. [5] Based on our findings, we recommend conducting a history and review of systems with special attention to thyroid (e.g., cold/heat sensitivity), gastrointestinal (e.g., diarrhea or weight loss suggestive of celiac disease), respiratory (e.g., wheezing suggestive of asthma), and dermatologic (e.g., pruritus) symptoms, and physical examination of wrists and oral cavity for LP, anogenital region for LSA, extensor regions, nails, and scalp for psoriasis, and face and hands for vitiligo. Funding The All of Us Research Program is supported by the National Institutes of Health, Office of the Director: Regional Medical Centers: 1 OT2 OD026549; 1 OT2 OD026554; 1 OT2 OD026557; 1 OT2 OD026556; 1 OT2 OD026550; 1 OT2 OD 026552; 1 OT2 OD026553; 1 OT2 OD026548; 1 OT2 OD026551; 1 OT2 OD026555; IAA #: AOD 16037; Federally Qualified Health Centers: HHSN 263201600085U; Data and Research Center: 5 U2C OD023196; Biobank: 1 U24 OD023121; The Participant Center: U24 OD023176; Participant Technology Systems Center: 1 U24 OD023163; Communications and Engagement: 3 OT2 OD023205; 3 OT2 OD023206; and Community Partners: 1 OT2 OD025277; 3 OT2 OD025315; 1 OT2 OD025337; 1 OT2 OD025276.

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.

Vulvar lichen sclerosus and lichen planus are T-cell–mediated chronic skin disorders. Although autoimmunity has been suggested, the exact pathogenesis of these disorders is still unknown. Therefore, the aim of the current study was to investigate the molecular and immunological mechanisms critical to the pathogenesis of vulvar lichen sclerosus and lichen planus. By using gene expression profiling and real-time RT-PCR experiments, we demonstrated a significantly increased expression of the pro-inflammatory cytokines (IFNγ, CXCR3, CXCL9, CXCL10, CXCL11, CCR5, CCL4, and CCL5) specific for a Th1 IFNγ-induced immune response. In addition, BIC/microRNA-155 (miR-155)—a microRNA involved in regulation of the immune response—was significantly upregulated in lichen sclerosus and lichen planus (9.5- and 17.7-fold change, respectively). Immunohistochemistry showed a significant T-cell response, with pronounced dermal infiltrates of CD4+, CD8+, and FOXP3+ cells. In conclusion, these data demonstrate an autoimmune phenotype in vulvar lichen sclerosus and lichen planus, characterized by increased levels of Th1-specific cytokines, a dense T-cell infiltrate, and enhanced BIC/miR-155 expression.

Lichen sclerosus is a chronic skin disease, mainly localised at the introitus and perineum. When the condition remains untreated, gradual atrophy of skin structures leads to permanent scarring, making early diagnosis and treatment crucial. We reviewed all patients diagnosed with lichen sclerosus presenting to a tertiary referral centre for paediatric and adolescent gynaecology between January 2011 and December 2015 to assess disease presentation and response to treatment. We identified 15 cases, with a mean age at diagnosis of 8.8 years. Their main presenting symptoms were vulvar pruritus and vulvar soreness. Seven girls had already atrophic changes, and in four girls, this amounted to clitoral phimosis, labial resorption or labial adhesion formation. The median delay in diagnosis was 7 months. Thirteen patients received local treatment with potent corticosteroids, responding well to treatment. However, 4 girls relapsed within 2 to 36 months. Two adolescents required surgical treatment, one because of urinary retention and the second because of dyspareunia caused by clitoral entrapment. Conclusions : There was a delay in diagnosis in most patients and this resulted in irreversible genital skin changes, which would have been preventable, had treatment been instituted promptly. The response to treatment with local corticosteroids was usually effective, leading to both symptom alleviation and prevention of disease progression. Atrophic changes and skin complications however were not reversed. What is Known: • Lichen sclerosus affects women of all ages, including girls, particularly prior to adolescence. • Lichen sclerosus responds well to local corticosteroid treatment . What is New: • In the majority of patients with lichen sclerosus there was a long delay between onset of symptoms and diagnosis . • Nearly half of the children diagnosed with lichen sclerosus had irreversible atrophic genital skin changes at the time of first presentation. These changes may have been prevented by a timely diagnosis and intervention .

Lichen planus (LP) is a common chronic inflammatory condition that can affect skin and mucous membranes, including the oral mucosa. Because of the anatomic, physiologic and functional peculiarities of the oral cavity, the oral variant of LP (OLP) requires specific evaluations in terms of diagnosis and management. In this comprehensive review, we discuss the current developments in the understanding of the etiopathogenesis, clinical-pathologic presentation, and treatment of OLP, and provide follow-up recommendations informed by recent data on the malignant potential of the disease as well as health economics evaluations.

Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly Bullous Pemphigoid (BP) or Lichen Planus (LP), a growing body of evidence suggests that it is a disease entity in its own right. In common with a range of autoimmune blistering diseases, including BP, pemphigoid gestationis (PG), mucous membrane pemphigoid (MMP) and linear IgA dermatosis (LAD), a key feature of the disease is the development of autoantibodies against type XVII collagen (COL17). However, accurately establishing the diagnosis is dependent on a careful correlation between the clinical, histological and immunological features of the disease. Therefore, we present an up to date summary of the epidemiology and etiopathogenesis of LPP, before illustrating the predisposing and precipitating factors implicated in the development of the disease. In addition to a selective literature search, we compare reports of potential drug-induced cases of LPP with pharmacovigilance data available via OpenVigil. We subsequently outline the cardinal clinical features, important differential diagnoses and current treatment options. We conclude by demonstrating that an improved understanding of LPP may not only lead to the development of novel treatment strategies for the disease itself, but may also shed new light on the pathophysiology of more common and treatment-refractory autoimmune blistering diseases.

To investigate the association between Epstein Barr virus (EBV) and Oral Lichen Planus (OLP) in both of its clinical phenotypes (erosive and non-erosive), 33 OLP cases divided into erosive and non-erosive phenotypes, and 26 non-OLP cases were evaluated for the presence of EBV using In Situ Hybridization. Immunohistochemistry was used to assess expressions of CD3, CD20, CD138 and p53 in both OLP clinical phenotypes. EBV was detected in 11 (33%) of the OLP cases and none of the non-OLP cases ( p  = 0.002). CD3 and CD20 were both over-expressed in all OLP cases, however CD138 was significantly over-expressed in the erosive OLP phenotype by comparison to the non-erosive one ( p  = 0.003), suggesting a possible role of plasma cell in erosive OLP. There was no association between EBV and CD-138, nor with erosive OLP. Interestingly however, EBV had an association with p53 expression among OLP cases ( p  = 0.038), inferring a role of EBV in possible neoplastic changes in OLP. There appears to be a potential role of EBV in causing at least some OLP cases, regardless of whether OLP is erosive or not. EBV might have an etiological role in causing some OLP cases. This could explain why some OLP cases are recalcitrant to corticosteroids treatment. Clinical trials are needed to establish whether EBV-infected OLP cases respond to antiviral therapy.

PurposeLichen sclerosus (LS) is a benign, cutaneous, chronic inflammatory (autoimmunological) disease. The differentiated vulvar intraepithelial neoplasia (dVIN) accounts for a precursor lesion of vulvar squamous cell carcinoma and is often associated with lichen sclerosus. Although the association between lichen sclerosus and vulvar carcinoma has long been recognized, there is a lack of evidence in literature.MethodsThis retrospective study examined pseudonymized data of 499 women diagnosed with vulvar pathology between 2008 and 2020 at the Department of Gynaecology and Obstetrics of Hannover Medical School (MHH). Data were further stratified for the time of onset, location of disease, accompanying disease, HPV status and progression of disease into vulvar squamous cell carcinoma (VSCC).ResultsIn total, 56 patients were diagnosed with vulvar lichen sclerosus. The mean onset of disease was at 60.3 years of age. After subdividing cases of diagnosed LS into those who did not develop vulvar carcinoma in their course and those who did, the ages at onset are 52.66 ± 17.35 and 68.41 ± 10.87, respectively. The incidence of vulvar cancer in women diagnosed with lichen sclerosus was 48.2%. Twenty-five patients reported a diagnosis of VIN in their self-reported history.ConclusionsIn our retrospective study, we showed a trend between vulvar lichen sclerosus and VSCC. The difference between the two age groups of patients diagnosed with lichen sclerosus who developed vulvar carcinoma and those who did not is statistically significant. Our results highlight the importance to diagnose lichen sclerosus early to ensure adequate follow-up and prevent progression to VSCC.

Introduction: Immune checkpoint inhibitors (ICIs) are recommended for various types of cancer. On the other hand, these ICIs may cause immune-related adverse events (irAEs). Lichen sclerosus (LS) and lichen planus (LP) are two distinct phenotypes of irAEs that occur in a subset of patients treated with ICIs. These adverse effects have a detrimental effect on the patient’s quality of life and treatment phases; however, the clinical evaluation and assessment of LS and LP remain uncertain. This study aims to assess and evaluate the risk of LS and LP associated with the use of ICIs via a systematic review of the literature and the USA FDA Adverse Events FAERS database. Method: The study searched electronic databases such as PubMed, Medline, Cochrane, and Google Scholar for case reports on immune-checkpoint-inhibitor-associated lichen sclerosus and lichen planus published in English between inception and 31 December 2021. The FDA’s adverse event reporting system (FAERS) database was also analyzed. Results: Thirty-eight case reports and two retrospective studies with a total of 101 patients, in addition to the FAERS data, were evaluated. More cases involved lichen planus (78.9%) than lichen sclerosis (21%). Nivolumab and pembrolizumab were most frequently reported with LS and LP, among other ICIs. Thirty-six out of thirty-eight patients with LS or LP experienced complete remission, while two patients experienced partial remission. Most of the cases had an excellent response to corticosteroids (92.1%), while the remainder had moderate (5.2%) and poor (2.6%) responses. Additionally, the reporting odds ratio (ROR) of the FAERS database indicated a favorable association for ICIs, the risk of LP, and LS. A stronger association was uniquely found between nivolumab and pembrolizumab. Conclusion: There have been published case reports for these adverse events. Healthcare providers should be aware of the possibility of lichen sclerosis and lichen planus developing in patients receiving ICIs which could necessitate hospitalization or discontinuation. Regulatory agencies are advised to monitor the risks as a potential safety signal.