OBJECTIVES: To investigate some epidemiological data of lichensclerosus patients; to verify its association with other conditionsand compare these data with the literature. METHODS:Retrospective analysis of medical records of patients submitted to12,724 vaginal/cervical examinations and vulvoscopies, at Hospitaldo Servidor Público Estadual “Francisco Morato de Oliveira” – SãoPaulo, from August 1998 to August 2001. RESULTS: The frequencyof lichen sclerosus confirmed by pathological examination was0.73% among these patients. Mean age was 63.6 years. Mostpatients presenting the condition were aged over 50 years (81/93), and the difference was statistically significant as comparedwith younger subjects (12/93). Caucasian women represented91.4% of the sample and black, 8.6%. Smokers accounted for 8.6%of the cases. Autoimmune diseases were detected in 31.2%,psychiatric disorders in 17.2%, and neoplasms in 11.8% of thosesuffering from lichen sclerosus. The colpocytology of one patientwas classified as Papanicolaou class V. CONCLUSION: These datashow the population studied presented findings similar to thosereported in the literature

Eduardo Sérgio Borges da Fonseca

José Juvenal Linhares

Julieta Massabni Zalc

Reginaldo Guedes Coelho Lopes

Renata de Loreto Ribeiro do Val

Umberto Gazi Lippi

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19einstein 2003; 1:19-21ABSTRACTOBJECTIVES: To investigate some epidemiological data of lichensclerosus patients; to verify its association with other conditionsand compare these data with the literature. METHODS:Retrospective analysis of medical records of patients submitted to12,724 vaginal/cervical examinations and vulvoscopies, at Hospitaldo Servidor Público Estadual “Francisco Morato de Oliveira” – SãoPaulo, from August 1998 to August 2001. RESULTS: The frequencyof lichen sclerosus confirmed by pathological examination was0.73% among these patients. Mean age was 63.6 years. Mostpatients presenting the condition were aged over 50 years (81/93), and the difference was statistically significant as comparedwith younger subjects (12/93). Caucasian women represented91.4% of the sample and black, 8.6%. Smokers accounted for 8.6%of the cases. Autoimmune diseases were detected in 31.2%,psychiatric disorders in 17.2%, and neoplasms in 11.8% of thosesuffering from lichen sclerosus. The colpocytology of one patientwas classified as Papanicolaou class V. CONCLUSION: These datashow the population studied presented findings similar to thosereported in the literature.Keywords: Lichen sclerosus et atrophicus/epidemiology; Vulvardiseases/pathology.RESUMOOBJETIVOS: Pesquisar alguns dados epidemiológicos referentes apacientes portadoras de líquen escleroso; verificar sua associaçãocom outras patologias e cotejar os dados com os da literatura.MÉTODOS: Análise retrospectiva dos prontuários de pacientes quese submeteram a 12.724 cervicovaginovulvoscopias, no Hospitaldo Servidor Público Estadual “Francisco Morato de Oliveira” – SãoPaulo, entre agosto de 1998 e agosto de 2001. RESULTADOS: Afreqüência do líquen escleroso, confirmado por exame anatomo-patológico, foi de 0,73% entre as pacientes estudadas. A idade média*Study carried out in the Division of Lower Genital Tract Diseases, Gynecology and Obstetrics Service, Hospital do Servidor Público Estadual Francisco Morato de Oliveira (HSPE - FMO) - São Paulo - SP.1 Resident Division of Lower Genital Tract Disease, Gynecology and Obstetrics Service, Hospital do Servidor Público Estadual “Francisco Morato de Oliveira” (HSPE - FMO) - São Paulo.2 Physician in Charge of the Division of Lower Genital Tract Disease (HSPE - FMO) - São Paulo.3 Resident Division of Lower Genital Tract Disease, Gynecology and Obstetrics Service, (HSPE - FMO) - São Paulo.4 Physician of the Gynecology and Obstetrics Service (HSPE - FMO) - São Paulo.5 Head of the Gynecology Division (HSPE - FMO) - São Paulo.6 Director of the Gynecology and Obstetrics Service (HSPE - FMO) - São Paulo.Correspodence to: Humberto Gazzi Lippi. Serviço de Ginecologia e Obstetrícia do HSPE. R. Pedro de Toledo, 1800 - 5º andar - CEP 04039-040 - São Paulo (SP).Recebido para publicação em 1/4/2003 – Aceito em 2/6/2003ORIGINAL ARTICLEEpidemiology of vulvar lichen sclerosus and its correlation withother conditionsEpidemiologia do líquen escleroso de vulva e correlação com outras afecções*José Juvenal Linhares1, Julieta Massabni Zalc2, Renata de Loreto Ribeiro do Val3, Eduardo Sérgio Borges da Fonseca4,Reginaldo Guedes Coelho Lopes5, Umberto Gazi Lippi6foi de 63,6 anos. Entre as portadoras, a maioria tinha 50 anos oumais (81/93), diferença estatisticamente significativa em relação àscom menos idade (12/93). Eram brancas 91,4% e negras, 8,6%. Astabagistas representaram 8,6% dos casos. Doenças auto-imunesforam detectadas em 31,2% das portadoras do líquen escleroso,doenças psiquiátricas em 17,2% e neoplasias em 11,8%. Umapaciente teve sua colpocitologia oncótica classificada como classeV de Papanicolaou. CONCLUSÃO: Os dados pesquisados mostraramque a população estudada tem um comportamento concordante comos dados da literatura.Descritores: Líquen escleroso e atrófico/epidemiologia; Doenças davulva/patologiaINTRODUCTIONLichen sclerosus (LE) corresponds to approximately 60% of thecases of non-neoplastic epithelial disorder of the vulva, ofunknown etiology, according to the classification proposed bythe International Society of Gynecological Pathologists, in 1987(1-2). It may be considered a skin disease, characterized by localizedareas of atrophy and discoloration of skin(1). LE is much morefrequent in women than in men (ratio of 10:1) and Caucasianpredominance is reported(3). Vulvar LE is a chronic disease andits onset is usually in postmenopause, but it may occur at anyage, even in childhood(4). A characteristic monoclonal gamma-T-cell receptor rearrangement was observed in this condition,suggesting presence of a lichen sclerosus-associated antigen(5).The process is asymptomatic in 15% of the cases(1); in theremaining, pruritus is the most frequent symptom, leading tovigorous itching with subsequent ulceration and ecchymosis,which may impair diagnosis(3). The etiology is unknown and thereare hypotheses of hormonal and genetic alterations, andautoimmune process(3,6). According to recent studies, the20 Linhares JJ, Zalc JM, Val RLR, Fonseca ESB, Lopes RGC, Lippi UGPSYCHIATRIC DISEASESPSIQUIÁTRICASNEOPLASMONCOLÓGICASAUTOIMMUNE DISEASESAUTO-IMUNESFigure 1. Distribution of associated diseases in lichen sclerosuspatientsl lllll l0 5 10 15 20 25 30161129suggested infectious etiology was practically excluded(7). Theclassic macroscopic image is a morphological alteration of thevulva, with dry skin, loss of hair and disappearing of subcutaneousfat(1). Fused labia minora over the urethra and importantinvolvement of the clitoris may occur(3). Deformity of vulvarstructures is characteristic of the most advanced phases of LE.In order of frequency, the most affected structures are clitoris,labia minora, vaginal orifice and labia majora, which presentdiverse grades of involution, from mild hypotrophy to totaleffacement. Vaginal orifice stenosis with dyspaurenia is the majorcomplication of the disease(8). The histopathological findings ofLE include a broad zone of hyalinization and papillar andreticular dermis edema. Epidermal ridges may be absent andepidermis becomes flat and hyperkeratotic. Atrophy may extendbeyond the dermis and affect subcutaneous tissue(9). In thehyperkeratotic area, collagen fibers are swollen and homoge-neous and contain only few nuclei. There is a significant changein distribution of collagen I and III, apart from reduction in elasticfibers(10). Blood and lymphatic vessels are dilated, and there mightbe hemorhagic areas(5,11). Several autoimmune diseases areassociated with LE(2,7,9,12,13).OBJECTIVESTo carry out a retrospective study of epidemiological data of LEpatients and verify frequency and types of associated conditions.METHODSThe medical records of patients submitted to 12,724 vaginal/cervical examinations and vulvoscopies, in the Division of LowerGenital Tract Disease, Colposcopy and Prevention of Gynecologi-cal Cancer of Hospital do Servidor Público Estadual “FranciscoMorato de Oliveira”, from August 1998 to August 2001, wereanalyzed. Among the cases submitted to vulvoscopy followed bybiopsy, the prevalence of LE was confirmed by histopathologicalexamination. Its epidemiology and association with autoimmuneand psychiatric diseases and neoplams was evaluated. Thestatistical analysis was performed by chi-square test, using Epi-Info 5.01 (CDC).RESULTSIn 12,724 vulvoscopies performed, 93 patients (0.73%) sufferedfrom LE. The mean age was 63.6 (+ 11.7) years, ranging from 32to 93 years. There was a significant difference between the numberof patients with the condition aged under (12/93) and over (81/93) 50 years (p < 0.00001).Caucasian patients represented 91.4% (85/93), and blackpatients, 8.6% (8/93). Smokers accounted for 8.6% (8/93).Forty-nine (52.7%) out of 93 patients had one or moreassociated diseases (fig 1). Autoimmune diseases (diabetesmellitus type I, rheumatoid arthritis and systemic lupuserythematous were the conditions most often observed) weredetected in 29 patients (31.2%); 16 patients (17.2%) sufferedfrom psychiatric disorders (mainly anxiety and depression); and11 individuals (11.8%) had neoplastic diseases (ovary, uterinecervix and gastrointestinal tract were the most common). Theassociations observed among these patients were as follows: threewomen presented psychiatric disorders and neoplams, three hadneoplasms and autoimmune diseases, and two suffered fromconditions related to the three groups. There was statisticallysignificant difference between the group of LE patients and thegroup not presenting this condition for frequency of thesecomplications (chi-square = 11.5; p = 0.03). Pap smears showed12.9% (12/93) of the patients in class I, 86.0% (80/93) in class IIand 1.1% (1/93) in class V.DISCUSSIONAccording to the literature, the incidence of LE in the generalpopulation is very low, as observed in this study. Regarding agegroups, the reports show higher incidence in subjects aged over 60years and in postmenopausal period, although it may occur at anyage, even in prepubertal girls and in postpubertal period(4). Thesample assessed in this study was older.As to race, the population evaluated presented findingssimilar to those in the literature. There was a predominance ofCaucasian patients. The other factor assessed was smoking habit,and most subjects were non-smokers. Although this aspect isrelevant, there are not enough data to justify it; on the otherhand, smoking is not a factor valued in the literature as associatedwith this disease.The etiology of LE is unknown, and there are hypothesesof hormonal and genetic alterations, autoimmune processes andexcessive activation of elastase, which would digest the normalelastic fibers(3). Results of a study of 30 vulvar LE casesdemonstrated significantly reduced serum dihydrotestosterone,free testosterone and androstenedione levels, and it wasconcluded there would be a decrease in 5a reductase levels(5).The possible influence of genetic factors is demonstrated inoccasional reports of LE patient relatives, although familialoccurrence of the disease is rare(2). There are clinical andhistological descriptions of association with lichen planus andlocalized scleroderma(7). Autoimmune phenomena are observedin LE patients and their relatives: antithyroid antibodies andeinstein 2003; 1:19-2121antigastric parietal cells, vitiligo, pernicious anemia, atrophicgastritis, Sjögren’s syndrome, rheumatoid arthritis, systemiclupus erythematous and scleroderma (the disease most oftenassociated with LE); however, more accurate studies arenecessary to explain these associations(8,14). A possible enzymaticmechanism, related to increased activity of enzymes, such aselastase, collagenase or amidase, in the fibroblasts of the skininvolved, could explain destruction of elastic dermal fibers(9).In this casuistry, apart from association with autoimmunediseases, a significant correlation between LE and psychiatricdiseases, or even with neoplasms, was found. Nevertheless,despite the statistically significant difference when comparingwith the non-LE population, it is very difficult to establish ifthere is a causal relation between the conditions or if theassociation is related to older age groups. The literatureprovides data on lichen sclerosus associated with HPV-negativevulvar squamous cell carcinoma ranging from 7 to 96%(15).These findings may trigger some prospective studies, evaluatingpsychiatric diseases and their subtypes, as well as neoplasms, inorder to select the conditions that are more often associated withLE and to determine screening procedures in this specificpopulation.CONCLUSIONSThe results of the present study confirm the literature dataand raise questions about association of LE with autoimmuneand psychiatric diseases and neoplasms. This should beprospectively evaluated in order to confirm the associationwith LE; hence, a specific screening in this specific populationmay be addressed.REFERENCES1. De Palo G, Chanen W, Dexeus S. Doenças da vulva: alterações epiteliais nãoneoplásicas. In: De Palo G, Chanen W, Dexeus S. Patologia e tratamento dotrato genital inferior. Rio de Janeiro: MEDSI; 2002. p. 222-34.2. Araujo MMV, Azevedo LMS, Souza MCB, Simões PM. Líquen escleroso eatrófico. Femina 1996;24:135-42.3. Sampaio SAP, Rivitti EA. Afecções atrófico-escleróticas. In: Sampaio SAP, RivittiEA. Dermatologia 2ª. ed rev. Rio de Janeiro: Artes Médicas; 2001. p. 259.4. Azevedo LMS, Santos NAL, Pereira AC Jr. Líquen escleroso e atrófico. An BrasDermatol 1989;64:178-80.5. Friedrich EG Jr, Kalpa PS. Serum levels of sex hormones in vulvar lichen sclerousand the effect of topical testosterone. N Engl J Med 1984;310:488-91.6. Ridley CM. Lichen sclerosus. Dermatol Clin 1992;10:309-23.7. Azevedo LMS, Azulay RD. Líquen escleroso y atrófico. Monograf Dermatol1993;6:60-171.8. Bovo AC. Propionato de clobetasol no tratamento do líquen escleroso vulvar. Efeitosclínicos e histopatológicos [tese]. São Paulo: Universidade Federal de São Paulo; 1996.9. Singer A, Monaghan JM. Neoplasia intra-epitelial vulvar. In: Singer A, MonaghanJM. Colposcopia, patologia e tratamento do trato genital inferior. Rio de Janeiro:Artes Médicas; 1995. p. 199-202.10.Farrel AM, Dean D, Millard PR, Charnock FM, Wojnarowska F. Alterations infibrillin as well as collagens I and III and elastin occur in vulval lichen sclerosus.J Eur Acad Dermatol Venerol 2001;15:212-7.11.Elder D, Ioffreda M, Miller J, Elenitsas R, Johnson Jr B, Miller III OF. Doenças daumidade reativa cutânea. In: Lever WF, Schaumburg-Lever G. Histopatologiada pele. São Paulo: Manole; 2001. p. 90-3.12. Friedrich EG Jr, Kalpa PS. Serum levels of sex hormones in vulvar lichen sclerousand the effect of topical testosterona. N Engl J Med 1984;310:488-91.13.Ridley CM. Lichen sclerous. Dermatol Clin 1992;10:309-11.14. Tasker GL, Wojnarowska F. Lichen sclerosus. Clin Exp Dermatol 2003;28:128-33.15. Scurry JP, Vanin K. Vulvar squamous cell carcinoma and lichen sclerosus. AustralJ Dermatol 1997;38(Suppl 1):S20-5.einstein 2003; 1:19-21Epidemiology of vulvar lichen sclerosus

Epidemiology of vulvar lichen sclerosus and its correlation with other conditions

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