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1.
Acta Dermatovenerol Alp Pannonica Adriat ; 33(2): actaapa.2024.10, 2024 04 12.
Artigo em Inglês | MEDLINE | ID: mdl-38606648

RESUMO

Multiple autoimmune syndrome is a manifestation of polyautoimmunity with the co-occurrence of three or more autoimmune diseases in a single patient. We report a unique case of a 55-year-old female patient that presented with four autoimmune diseases: autoimmune thyroid disease, vitiligo, morphea, and lichen sclerosus. She was evaluated for progression of morphea and lichen sclerosus, and we confirmed histopathological overlapping of these two diseases in the same lesion. We discuss the increasing prevalence of autoimmune diseases and similar case reports on dermatological polyautoimmunity.


Assuntos
Doenças Autoimunes , Líquen Escleroso e Atrófico , Esclerodermia Localizada , Doenças da Glândula Tireoide , Vitiligo , Feminino , Humanos , Pessoa de Meia-Idade , Esclerodermia Localizada/complicações , Esclerodermia Localizada/patologia , Líquen Escleroso e Atrófico/patologia , Vitiligo/complicações , Doenças Autoimunes/complicações , Doenças da Glândula Tireoide/complicações
2.
J Low Genit Tract Dis ; 28(2): 189-197, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38518217

RESUMO

OBJECTIVES: Lichen planus (LP) and lichen sclerosus (LS) are the most common vulvar lichenoid dermatoses. The diagnostic challenges are due to site-specific variation in microscopic appearance and small-sized biopsies. Authentication of diagnostic criteria to distinguish LS and LP to uncover any resemblance or divergence in presentation of these conditions is attempted. METHODS: Cases of vulvar LP and LS diagnosed between January 2012 to December 2022 were included. The clinical details included age, presenting symptoms, examination findings, and other organ involvement. Histopathological analysis of epidermal, dermal, and adnexal findings was done. RESULTS: There were 28 cases of vulvar LP and 72 cases of LS, with a median age of 51 and 60 years, respectively. Depigmentation and atrophy were the major clinical features in LS, whereas ulcers/erosions and erythema were more prevalent in LP with a significantly higher incidence of oral involvement. The most diagnostic feature in LS was diffuse dermal sclerosis (76.8%) and interstitial pattern of inflammation (81.4%), whereas the characteristic features in LP cases was a lichenoid pattern of inflammation (85.7%), necrotic keratinocytes, and lymphocytic exocytosis. In 44.4% of LS, unconventional features like compact orthokeratosis, parakeratosis, thickened/wedge-shaped hypergranulosis, and sawtooth rete pegs were noted. Lichen sclerosus with lichenoid inflammation (21.4%) mimicked LP, from which it was distinguished by presence of thickened or diminished granular layer with basal melanin absence (60%) and dermal homogenization (80%). CONCLUSION: Although the classical, well-established variant of LS poses no diagnostic difficulty, the unconventional variant may mimic LP. Identification of the subtle histological clues demonstrated in this study can help to arrive at the correct diagnosis.


Assuntos
Líquen Plano , Líquen Escleroso e Atrófico , Líquen Escleroso Vulvar , Feminino , Humanos , Pessoa de Meia-Idade , Líquen Escleroso e Atrófico/patologia , Vulva/patologia , Líquen Plano/patologia , Inflamação/patologia , Biópsia , Líquen Escleroso Vulvar/diagnóstico , Líquen Escleroso Vulvar/patologia
3.
Am J Dermatopathol ; 46(5): 287-291, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38457688

RESUMO

ABSTRACT: Lichen sclerosus (LS) is a frequently encountered inflammatory skin disorder characterized by whitened, atrophic patches that can cause pain and pruritus. The underlying cause of this condition remains unknown. Primarily affecting the genital area, this condition carries an increased risk of developing cutaneous cancers and frequently co-occurs with autoimmune disorders. Our retrospective study aimed to explore histologic features of LS, with a particular focus on a newly established finding and its potential implications. We examined 53 histologic cases of LS collected over 2 years. Experienced pathologists evaluated and reached a consensus on the assignment of histologic features. Patient charts were manually reviewed to gather relevant demographic and clinical data. Statistical analysis was performed using IBM SPSS Statistics (2021). Of the 53 total patients identified as meeting criteria for inclusion in this study, only 8 (15%) were male. Eight cases (15%) demonstrated perineural inflammatory infiltrate. Notably, half of all samples from male patients exhibited perineural inflammatory infiltrate. A statistically significant increase ( P < 0.01) in the presence of dermal plasma cells was identified in cases with perineural inflammation versus cases without this feature. The findings of our study highlight the recurrent nature of perineural inflammation in LS, providing valuable insights into this condition. Furthermore, we observed a notable correlation between perineural inflammation, male patients, and the presence of dermal plasma cells. These discoveries contribute to a better understanding of the underlying mechanisms of LS and suggest avenues for future research into the condition.


Assuntos
Doenças Autoimunes , Líquen Escleroso e Atrófico , Humanos , Masculino , Feminino , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/patologia , Estudos Retrospectivos , Inflamação , Prurido
4.
Am J Dermatopathol ; 45(12): 820-821, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-37883982

RESUMO

ABSTRACT: The presence or absence of tissue eosinophilia has previously aided in the diagnosis of inflammatory skin conditions. However, recent studies have elucidated the presence of eosinophils in traditionally eosinophil-poor inflammatory skin diseases, such as dermatomyositis (DM), psoriasis, and lichen sclerosus (LS). This systematic review of the literature explores previous studies of tissue eosinophilia in skin biopsies of dermatoses that are believed to be classically poor in eosinophil. We identified 26 studies, the majority of which were retrospective reviews. The percent of specimens with increased eosinophils in psoriasis ranged from 18%-73%, pityriasis rubra pilaris (PRP) 22%-63%, LS 29%-53%, DM 15%-44%, morphea 8%-45%, hypertrophic lichen planus (LP) 0%-21%, and oral LP 0%-4%. These reports of tissue eosinophilia in reputed eosinophil-poor dermatologic conditions present a diagnostic pitfall and suggest that tissue eosinophilia itself should not be used to rule out a diagnosis of one of these conditions.


Assuntos
Eosinofilia , Líquen Escleroso e Atrófico , Pitiríase Rubra Pilar , Psoríase , Humanos , Eosinofilia/patologia , Eosinófilos/patologia , Líquen Escleroso e Atrófico/patologia , Pitiríase Rubra Pilar/patologia , Psoríase/patologia , Estudos Retrospectivos , Pele/patologia
5.
J Dermatol ; 50(11): 1497-1500, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37533335

RESUMO

Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis of unknown pathogenesis, characterized by porcelain-white atrophic plaques around the vulvar and anal areas in girls. With this communication, we performed the study on 16 female girls with clinically and histologically confirmed VLS, described the main identifying characteristics of the lesions in reflectance confocal microscopy (RCM) and elucidated the corresponding relationship between RCM findings and histology. We recommend RCM, a noninvasive technique, as a complementary diagnostic tool for VLS.


Assuntos
Líquen Escleroso e Atrófico , Líquen Escleroso Vulvar , Neoplasias Vulvares , Feminino , Humanos , Líquen Escleroso Vulvar/diagnóstico por imagem , Líquen Escleroso Vulvar/patologia , População do Leste Asiático , Líquen Escleroso e Atrófico/diagnóstico por imagem , Líquen Escleroso e Atrófico/patologia , Vulva/diagnóstico por imagem , Vulva/patologia , Microscopia Confocal
6.
Exp Dermatol ; 32(10): 1734-1743, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37486173

RESUMO

Incorrect and delayed diagnosis of vulvar high-grade squamous intraepithelial neoplasia (vHSIL) and lichen sclerosus (LS) increases malignant progression risks and negatively impacts prognosis and quality of life. There is a need to improve diagnosis and monitoring. Reflectance confocal microscopy is a non-invasive imaging tool that visualizes skin structures at cellular resolution. The objectives were to explore feasibility and patient acceptability of vulvar RCM imaging and to identify RCM characteristics that are discriminative for vulvar HSIL and LS. This was a prospective, cross-sectional, observational clinical trial in patients with vHSIL and LS compared to healthy volunteers. RCM images and vulvar tissue samples were obtained. Five (5) patients with vHSIL, 10 patients with LS and 10 healthy volunteers were enrolled. In total, 100 image series of vulvar skin were obtained, including lesional and nonlesional sites. The RCM technique was considered acceptable for application by patients and healthy controls. Healthy vulvar skin was characterized by a homogenous, normal honeycomb patterned epidermis and a clear epidermal-dermal junctions. Vulvar HSIL and LS displayed an atypical honeycomb pattern of the epidermis and lymphocytic influx with presence of melanophages. Distinct features specifically observed in LS included the presence of hyalinised vessels and sclerotic areas in the dermis. RCM is a non-invasive imaging technique that is feasible and clinically acceptable to apply on vulvar skin, both in patients with premalignant lesions and healthy controls. Recognition and validation of disease-specific characteristics could make reflectance confocal microscopy a clinical tool to non-invasively aid identification of vulvar premalignancies.


Assuntos
Carcinoma in Situ , Líquen Escleroso e Atrófico , Neoplasias Cutâneas , Neoplasias Vulvares , Feminino , Humanos , Líquen Escleroso e Atrófico/diagnóstico por imagem , Líquen Escleroso e Atrófico/patologia , Estudos Transversais , Voluntários Saudáveis , Estudos Prospectivos , Qualidade de Vida , Neoplasias Vulvares/diagnóstico por imagem , Neoplasias Vulvares/patologia , Neoplasias Cutâneas/patologia , Carcinoma in Situ/química , Carcinoma in Situ/patologia , Microscopia Confocal
7.
Am J Dermatopathol ; 45(8): 588-592, 2023 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-37462209

RESUMO

ABSTRACT: Several vulvar lichen sclerosus (VLS) clinical severity scales have recently been proposed. In this prospective case series, we characterized histopathology in the context of clinical severity in 6 treatment-naïve postmenopausal patients with VLS. The Vulvar Quality of Life Index (VQLI) and an adaptation of the 2018 International Society for the Study of Vulvovaginal Disease Delphi consensus VLS severity score were administered. Vulvar skin punch biopsies were obtained to measure inflammatory density, constituent inflammatory cells, thickness of the stratum corneum and other epidermal layers, dermal edema, and dermal sclerosis. Clinicopathologic correlations were assessed. Two cases demonstrated sparse inflammatory densities, 1 case demonstrated patchy and nodular inflammatory density, 1 case demonstrated dense lichenoid inflammatory density, and 2 cases demonstrated dense lichenoid and epitheliotropic inflammatory densities. Those patients who reported severe pruritus demonstrated the greatest lymphocytic inflammatory densities on histopathological examination. Both cases of ulceration or erosion were associated with severe VQLI scores. Severe VQLI scores were also associated with trends for higher average thickness of the epidermal layers and of dermal sclerosis. Altogether, histopathologic grading of biopsy sites may reflect clinical severity in patients with VLS.


Assuntos
Líquen Escleroso e Atrófico , Líquen Escleroso Vulvar , Feminino , Humanos , Líquen Escleroso Vulvar/patologia , Qualidade de Vida , Esclerose/patologia , Vulva/patologia , Epiderme/patologia , Líquen Escleroso e Atrófico/patologia
8.
World J Urol ; 41(9): 2495-2501, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37421420

RESUMO

OBJECTIVE: To describe differences in the urinary microbiome of patients with pathologically confirmed lichen sclerosus (LS) urethral stricture disease (USD) vs non-lichen sclerosus (non-LS) USD pre- and post-operatively. METHODS: Patients were pre-operatively identified and prospectively followed, all underwent surgical repair and had tissue samples obtained to make a pathological diagnosis of LS. Pre- and post-operative urine samples were collected. Bacterial genomic DNA was extracted. Alpha and beta diversity measurements were calculated and compared. A zero-inflated negative binomial model was utilized to compare taxa abundances between disease status and surgery status. RESULTS: Urine samples were obtained from both cohorts, 69 samples in total: 36 samples were obtained pre-operatively and 33 samples were obtained post-operatively. Ten patients provided both a pre-operative and post-operative urine sample. Twenty-six patients had pathological evidence of LS and 33 patients did not. There was a statistically significant difference in alpha diversity between the pre-operative urine samples of patients with non-LS USD and LS USD, (p = 0.01). There was no significant difference in alpha diversity within post-operative urine samples between patients with non-LS USD and LS USD, (p = 0.1). A significant difference was observed in Weighed UniFrac distances with respect to disease and operative status, (p = 0.001 and 0.002). CONCLUSIONS: LS USD have significant alterations in diversity and differential abundance of urine microbiota compared to non-LS USD controls. These findings could be used to guide further investigations into the role of the urinary microbiome in LS USD pathogenesis, severity of presentation, and stricture recurrence.


Assuntos
Líquen Escleroso e Atrófico , Estreitamento Uretral , Humanos , Estreitamento Uretral/etiologia , Constrição Patológica , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/patologia
9.
J Low Genit Tract Dis ; 27(4): 358-364, 2023 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-37467474

RESUMO

OBJECTIVE: Nonsclerotic lichen sclerosus (NSLS) refers to the clinicopathologic situation of examination findings consistent with lichen sclerosus (LS) but without dermal sclerosis on microscopy. This review aims to describe the features of NSLS and provide a classification framework. METHODS: The International Society of the Study of Vulvovaginal Diseases tasked the Difficult Pathologic Diagnoses Committee with development of consensus documents for conditions with problematic histopathology. The Difficult Pathologic Diagnoses Committee reviewed the literature on NSLS and formulated descriptions and diagnostic criteria, then approved by the International Society of the Study of Vulvovaginal Diseases membership. RESULTS: Nonsclerotic LS may be categorized into 4 histopathologic subtypes: lichenoid dermatitis, hypertrophic lichenoid dermatitis, dermal fibrosis without acanthosis, and dermal fibrosis with acanthosis. Each has a pathologic differential diagnosis of 1 or more entities, so clinical correlation is required for final diagnosis of LS. There is no evidence to support a reliable association between absent sclerosis and clinical appearance, duration, or oncogenic potential of LS. CONCLUSIONS: Pathologists and clinicians should be familiar with the concept of NSLS and its implications for patient management. Use of the term "early LS" to indicate a lack of sclerosis in presumed LS should be abandoned. Clinical correlation is required to confirm LS from among the differential diagnoses.


Assuntos
Dermatite , Líquen Escleroso e Atrófico , Doenças Vaginais , Feminino , Humanos , Líquen Escleroso e Atrófico/diagnóstico , Líquen Escleroso e Atrófico/patologia , Esclerose , Fibrose
10.
Arkh Patol ; 85(3): 29-39, 2023.
Artigo em Russo | MEDLINE | ID: mdl-37272438

RESUMO

BACKGROUND: Vulvar lichen sclerosus (VLS) is a chronic and recurrent dermatosis of an inflammatory nature with severe focal atrophy of the skin. Connective tissue changes are polymorphic and are still not taken into account in histological diagnostics due to the difficulty of interpreting routine histological methods. In this work, we use multiphoton microscopy (MPM) as a new imaging technique that provides detailed information about the organization of collagen fibers in the dermis based on a non-linear second harmonic generation (SHG) process. OBJECTIVE: To determine the degree of connective tissue damage in lichen sclerosus using standard histological techniques and to reveal the diagnostic capabilities of multiphoton microscopy. MATERIAL AND METHODS: We studied 42 biopsies with a histopathological diagnosis of VLS and 10 biopsies of normal vulvar skin. Histological, histochemical and immunohistochemical evaluation was used in comparison with MPM data. Quantitative analysis included the determination of the thickness, length of collagen fibers and the average intensity of the SHG signal. RESULTS: A comprehensive study of the skin showed 4 groups of changes that can be regarded as the degree of the dermis damage: initial, mild, moderate, severe. The affected area at the initial and mild degree has subtle changes, however, it is reliably identified by quantitative analysis of the SHG signal. So, the initial degree is characterized by thin (1.3-1.8 µm) long (56-69 µm) collagen fibers, with a moderate degree, the fibers are thickened (3.4-4.3 µm) and fragmented (22-37 µm). The affected area in moderate and severe cases undergoes homogenization, which is associated with the deposition of extremely thin (0.6-0.9 µm) short (16-28 µm) collagen fibers and the expression of type V collagen. CONCLUSION: Multiphoton microscopy in the second harmonic generation mode is a reliable method for identifying collagen fibers in tissues. The study made it possible to identify 4 degrees of the dermis damage in vulvar lichen sclerosus.


Assuntos
Líquen Escleroso e Atrófico , Líquen Escleroso Vulvar , Feminino , Humanos , Líquen Escleroso Vulvar/diagnóstico , Líquen Escleroso Vulvar/patologia , Microscopia , Líquen Escleroso e Atrófico/diagnóstico , Líquen Escleroso e Atrófico/patologia , Pele/patologia , Colágeno
11.
Urologie ; 62(7): 735-747, 2023 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-37314487

RESUMO

The urological examination includes the inspection of the external male genitals. Harmless normal variants, such as heterotopic sebaceous glands and pearly penile papules must be differentiated from malignant and infectious manifestations. Lichen sclerosus et atrophicus is a frequent connective tissue disease that can lead to functional impairments and an associated high level of suffering for those affected. Both conservative and invasive treatment options are available. Sexually transmitted diseases, such as syphilis, are gaining increasing importance in routine clinical and daily practice due to the increasing incidence in recent years. An early diagnosis and treatment of malignant neoplasms, such as Queyrat's erythroplasia can be carried out by routine inspection of the genital skin.


Assuntos
Líquen Escleroso e Atrófico , Neoplasias Penianas , Infecções Sexualmente Transmissíveis , Neoplasias Cutâneas , Masculino , Humanos , Neoplasias Penianas/patologia , Líquen Escleroso e Atrófico/patologia , Infecções Sexualmente Transmissíveis/complicações , Genitália Masculina/patologia
12.
Maturitas ; 175: 107767, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37302181

RESUMO

Vulvar lichen sclerosus is a chronic inflammatory disease involving vulvar skin. The risk of developing invasive vulvar cancer for women with LS is reported in the literature, but the risk of extra-vulvar tumors has been under-investigated. This multicentric study aims to estimate the risk of developing cancers in a cohort of women with a diagnosis of vulvar lichen sclerosus. METHODS: A cohort of women diagnosed with and treated for vulvar lichen sclerosus in three Italian gynecological and dermatological clinics (Turin, Florence, and Ferrara) was retrospectively reviewed. Patient data were linked to cancer registries of the respective regions. The risk of subsequent cancer was estimated by dividing the number of observed and expected cases by the standardized incidence ratio. RESULTS: Among 3414 women with a diagnosis of vulvar lichen sclerosus corresponding to 38,210 person-years of follow-up (mean 11.2 years) we identified 229 cancers (excluding skin cancers and tumors present at the time of diagnosis). We found an increased risk of vulvar cancer (standardized incidence ratio = 17.4; 95 % CL 13.4-22.7), vaginal cancer (standardized incidence ratio = 2.7; 95 % CL 0.32-9.771), and oropharyngeal cancer (standardized incidence ratio = 2.5; 95 % CL 1.1-5.0), and a reduced risk of other gynecological tumors (cervical, endometrial, ovarian) and breast cancer. CONCLUSIONS: Patients with vulvar lichen sclerosus should undergo annual gynecological check-up with careful evaluation of the vulva and vagina. The increased risk of oropharyngeal cancer also suggests the need to investigate oropharyngeal cavity symptoms and lesions in patients with vulvar lichen sclerosus.


Assuntos
Carcinoma de Células Escamosas , Líquen Escleroso e Atrófico , Neoplasias Orofaríngeas , Líquen Escleroso Vulvar , Neoplasias Vulvares , Humanos , Feminino , Líquen Escleroso Vulvar/complicações , Líquen Escleroso Vulvar/epidemiologia , Líquen Escleroso Vulvar/patologia , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/epidemiologia , Líquen Escleroso e Atrófico/patologia , Neoplasias Vulvares/complicações , Neoplasias Vulvares/epidemiologia , Estudos Retrospectivos , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/epidemiologia , Carcinoma de Células Escamosas/patologia , Vulva/patologia , Neoplasias Orofaríngeas/complicações , Neoplasias Orofaríngeas/patologia
13.
J Biophotonics ; 16(8): e202300078, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37184513

RESUMO

This paper describes a methodology to differentiate morphea from lichen sclerosus based on examination with multiphoton microscopy (MPM) composed of two-photon excited fluorescence (TPEF) and second harmonic generation (SHG). Subcellular-resolution images were acquired by MPM from unstained lesion tissues then process spectral analysis to quantify the TPEF and SHG signals. Moreover, U-Net was employed to segment elastic fiber in TPEF images to combine with collagen fiber in SHG images for precise fiber quantification. Predictions of segmentation showed excellent performance on several evaluation indicators. The mIoU, mPA, and F1 score reach 0.8516, 0.9281, and 0.941. The quantitative analysis demonstrated the increase of collagen fibers in morphea compared to that in lichen sclerosus cases. Meanwhile, the great diminution of elastic fiber in the dermis of lichen sclerosus was depicted based on MPM imaging. Thus, MPM was comparable to the histopathological examination and our experimental results accurately distinguish between morphea and lichen sclerosus.


Assuntos
Líquen Escleroso e Atrófico , Esclerodermia Localizada , Humanos , Líquen Escleroso e Atrófico/diagnóstico por imagem , Líquen Escleroso e Atrófico/patologia , Esclerodermia Localizada/diagnóstico por imagem , Microscopia , Tecido Elástico/patologia , Colágeno , Microscopia de Fluorescência por Excitação Multifotônica/métodos
15.
Lasers Surg Med ; 55(6): 521-527, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37061890

RESUMO

OBJECTIVES: To investigate the histologic characteristics of vulvar tissues before and after completion of fractionated carbon dioxide (CO2 ) laser therapy (FxCO2) for vulvar lichen sclerosus (LS). The secondary objective was to assess subjective improvement in symptoms via the Skindex-16 questionnaire. METHODS: This prospective single-arm study was conducted from April 2021 to August 2022 at one academic medical center. Ten postmenopausal women with biopsy-proven LS planning FxCO2 laser treatment were enrolled. Exclusion criteria included prior transvaginal mesh for prolapse, topical corticosteroid use within 8 weeks, prior pelvic radiation, malignancy, active genital infection, or pregnancy. The vulvovaginal SmartXide2-V2-LR laser system fractionated CO2 laser (DEKA) was utilized to treat visually affected areas of vulvar and perianal LS with a single pass. Subjects underwent three treatments 4-6 weeks apart. Subjects completed the Skindex-16 questionnaire and had vulvar biopsy at baseline and at 4 weeks after completion of fractionated CO2 laser therapy. Blinded histologic slides were scored by one dermatopathologist (Michael A. Cardis) rating from 1 to 5 the degree of dermal sclerosis, inflammation, and epidermal atrophy. Change scores were calculated as the difference between pre- and post-treatment scores for each subject. RESULTS: The 10 subjects enrolled had a mean age of 61 and most were white, privately insured, and had a college/graduate-level education. Post-fractionated CO2 laser treatment vulvar biopsies showed significant improvement in sclerosis and epidermal atrophy compared with pretreatment baseline biopsy specimens (p < 0.05) with no statistically significant change found in inflammation score. Skindex-16 and FSFI scores showed a trend towards improvement (p > 0.05 for both). A statistically significant correlation was found between change in sclerosis and Skindex-16 symptoms scores with an average change of 21.4 units in Skindex-16 symptoms score for every one-point change in histologic sclerosis score (p = 0.03). CONCLUSIONS: In postmenopausal women with vulvar LS undergoing fractionated CO2 laser, symptomatic improvements correlated with histologic change in degree of sclerosis on vulvar biopsy. These results demonstrate FxCO2 laser therapy as a promising option for the treatment of LS and suggest that further studies should assess degree of sclerosis on histopathology.


Assuntos
Líquen Escleroso e Atrófico , Líquen Escleroso Vulvar , Humanos , Feminino , Pessoa de Meia-Idade , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/patologia , Dióxido de Carbono , Projetos Piloto , Pós-Menopausa , Esclerose/complicações , Estudos Prospectivos , Líquen Escleroso Vulvar/complicações , Líquen Escleroso Vulvar/patologia , Líquen Escleroso Vulvar/terapia , Inflamação , Biópsia , Atrofia/complicações
16.
Exp Dermatol ; 32(6): 859-868, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36922453

RESUMO

Lichen planus (LP) is a cutaneomucosal chronic inflammatory disease characterized by a CD8+ cytotoxic T-lymphocytes (CTL) infiltrate. In erosive oral LP, we found HPV16-specific activated CTL in lesions, supporting a pathogenic contribution of HPV16. Here, we investigated whether a similar scenario occurs in other clinical forms of LP and in lichen sclerosus et atrophicus (LSA), another chronic disease also affecting the mucosa and/or the skin. Blood CTL from LP and LSA patients expressed significant higher levels of granzyme B, perforin and CD107a proteins than healthy donors. Expansions of TCRVß3+ CTL, with presence of TCR clonotypes identical to those previously detected in erosive oral LP, were found both in blood and mucosal/skin lesions of LP, and not of LSA patients. These expansions were enriched with HPV16-specific CD8+ T-cells as shown by their recognition of the E711-20 immunodominant epitope. In LSA patients, the peripheral repertoire of CTL was oligoclonal for TCRVß6+ CTL. Finally, although patients with LP and LSA have developed antibodies against HPV16 capsid L1, antibodies against HPV16 E6 were only observed in patients with LP. Overall, our data collectively suggest an involvement of HPV16-specific CTL in different clinical forms of LP, not only in erosive oral LP, while a different scenario operates in LSA.


Assuntos
Líquen Plano Bucal , Líquen Plano , Líquen Escleroso e Atrófico , Humanos , Papillomavirus Humano , Linfócitos T CD8-Positivos/metabolismo , Papillomavirus Humano 16 , Líquen Escleroso e Atrófico/metabolismo , Líquen Escleroso e Atrófico/patologia , Líquen Plano/patologia
18.
J Low Genit Tract Dis ; 27(2): 156-160, 2023 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-36821789

RESUMO

OBJECTIVE: Lichen sclerosus (LS) is a chronic inflammatory disease with a significant impact on quality of life. The aim of this cross-sectional case-control study was to characterize concomitant urogynecological and gastrointestinal disorders in female patients with LS. METHODS: A medical records search between 2004 and 2012 yielded 455 women and girls (mean age 64 years) with LS. The study cohort was compared with a 10-fold age- and sex-matched control cohort. Gynecological cancers and their precursors; gynecological, urinary, and gastrointestinal disorders; and pain syndromes were evaluated. RESULTS: The well-known association between LS and increased risk of vulvar cancer and its precursors was also found in our study (relative risk [RR] = 100.0; p < .001 and high-grade squamous intraepithelial lesions RR = 110.0; p < .001, respectively), but we also found an increased risk for cervical cancer (RR = 6.0; p = .005) and endometrial cancer (RR = 2.9; p < .001). Gynecological pain syndromes such as dyspareunia (RR = 20.0; p < .001) and interstitial cystitis (RR = 5.0; p < .001) and urinary incontinence (RR = 4.8; p < .001) were also increased. Among gastrointestinal disorders, we found increased risk for celiac disease (RR = 6.8; p < .001), diverticular intestine diseases (RR = 1.9; p < .001), functional intestinal disorders (RR = 2.3; p = .003), and anal and rectal fissures (RR = 2.4; p = .046). CONCLUSIONS: We found that female patients with LS have an increased risk for gynecological cancers as well as for several urogynecological and gastrointestinal disorders. Increased awareness is required to identify and treat these concomitant disorders.


Assuntos
Gastroenteropatias , Líquen Escleroso e Atrófico , Líquen Escleroso Vulvar , Humanos , Feminino , Pessoa de Meia-Idade , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/epidemiologia , Líquen Escleroso e Atrófico/patologia , Líquen Escleroso Vulvar/patologia , Estudos de Casos e Controles , Qualidade de Vida , Estudos Transversais , Síndrome , Comorbidade , Gastroenteropatias/epidemiologia , Gastroenteropatias/complicações , Dor
19.
Ann Diagn Pathol ; 63: 152098, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-36610314

RESUMO

Lichen sclerosus (LS) is a cutaneous disease of unknown etiology that often involves the vulva or foreskin but also can affect extragenital sites. Regardless of the anatomic site, the histomorphology and presumably pathogenesis are similar. Perhaps a clue to the pathophysiology of LS lies in its frequent association with morphea, specifically, when occurring in an extragenital context. In our experience a striking feature evident in established lichen sclerosis (LS) is one of superficial vascular drop out whereby residual vessels exhibited endothelial cell necrosis and microvascular basement membrane zone thickening, the latter reflective of antecedent episodes of microvascular injury. We sought to understand the pathophysiology that underlies the distinct vascular changes and in doing so, shed light on the pathogenesis of LS. We examined 44 cases of LS over a period of 2019 to 2021. We were able to obtain past medical histories in 34 of the 44 cases. Regarding pathological assessment, the predominant focus was on microvascular changes. We assessed the role of C5b-9 mediated vascular injury in the pathogenesis of the vasculopathy and enhanced type I interferon signaling in vessels given the morphologic semblance to the select interferonopathy syndromes, namely fibrosing dermatomyositis and Kohlmeier Degos disease. We examined the expression of CMV DNA and protein based on prior observations in an earlier study that isolated early protein expression in the microvasculature in the setting of LS and scleroderma. From a clinical perspective, the most striking association was an older age at the time of diagnosis (mean age of 62 years and median age of 61.5 years) and the presence of vascular comorbidities of diabetes, hypertension, and hyperlipidemia in almost 80% of cases. All cases showed significant microvascular changes in the superficial corium with the most frequent findings being those of significant basement membrane zone reduplication and vascular drop out. A number of cases showed prominent microvascular deposits of C5b-9 in the zone of hyalinizing fibrosis or subjacent to the discernible table of fibroplasia in the absence of enhanced type I interferon signaling. In no case were there viral cytopathic changes associated with CMV affecting the endothelium. The studies that encode CMV DNA or protein did not show a significant role for CMV reactivation in endothelium in the majority of the studied cases. It is concluded that the pathophysiology of LS includes a microvascular injury syndrome within the papillary dermis. The mechanism of endothelial cell injury is complement mediated at least in part and could reflect an adaptive immune response targeting endothelium indicative of classic complement pathway activation when coexisting with morphea or occurring in younger individuals. A non-immune based endothelial dysfunction and complement mediated injury unrelated to antibody driven classic complement pathway activation are more likely pathogenetically in the setting of certain diseases like diabetes mellitus and hypertension. Vascular drop out can be explained by the diminished endothelial progenitor pool needed to repopulate the damaged microvessels in certain settings like hypertension and diabetes.


Assuntos
Infecções por Citomegalovirus , Hipertensão , Interferon Tipo I , Líquen Escleroso e Atrófico , Esclerodermia Localizada , Adulto , Humanos , Pessoa de Meia-Idade , Complexo de Ataque à Membrana do Sistema Complemento , Infecções por Citomegalovirus/complicações , Hipertensão/complicações , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/patologia , Esclerodermia Localizada/complicações , Esclerodermia Localizada/patologia
20.
Int Urogynecol J ; 34(3): 779-781, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36102942

RESUMO

INTRODUCTION AND HYPOTHESIS: Lichen sclerosus is a chronic inflammatory dermatitis, with a predilection for the anogenital area. In later stages, lichen sclerosus may develop into widespread scarring, and occasionally leading to severe introital stenosis and urinary retention. Our video is aimed at presenting a case of surgical management of lichen sclerosus-related introital stenosis determining urinary retention. METHODS: An 82-year-old woman was evaluated for almost complete urinary retention, with concomitant continuous enuretic urinary leakage all day and night and recurrent urinary tract infection symptoms. The gynecological evaluation demonstrated a complete introital obliteration, without obvious communications for urine passing. After proper informed consent, the patient was admitted for vulvo-perineoplasty. RESULTS: The featured procedure was completed in 25 min and blood loss was negligible. No surgical complications were observed. On postoperative day 1, the patient was successfully discharged home with topical steroid treatment. Histological examination confirmed typical features of lichen sclerosus pathology. At follow-up visits the patient was asymptomatic and examination confirmed persistence of introital patency. CONCLUSIONS: The featured video shows a vulvo-perineoplasty performed in a patient with lichen sclerosus-related complete introital obliteration and urinary retention. The procedure was successful in obtaining anatomical repair and relieving urinary symptoms.


Assuntos
Líquen Escleroso e Atrófico , Retenção Urinária , Feminino , Humanos , Idoso de 80 Anos ou mais , Líquen Escleroso e Atrófico/complicações , Líquen Escleroso e Atrófico/diagnóstico , Líquen Escleroso e Atrófico/patologia , Constrição Patológica , Retenção Urinária/complicações , Vulva/patologia , Vagina/patologia , Inflamação
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