Association of autoimmune diseases with lichen sclerosus in 532 male and female patients.

Lichen sclerosus is a relatively common chronic inflammatory skin disease that predominantly affects the anogenital area. Accumulating evidence indicates that lichen sclerosus in women may be associated with other autoimmune disease, whereas this association seems to lack in male patients. We retrospectively evaluated the prevalence of autoimmune diseases and serological parameters indicative for autoimmunity in male and female patients with lichen sclerosus. Of the 532 patients (396 women, 136 men; 500 adults, 32 children; mean age: 49 years; range 1-89 years; female:male ratio 3:1), 452 (85%) had genital and 80 (15%) had extragenital disease. In women, lichen sclerosus was significantly more often associated with at least one autoimmune disease as compared to men (odds ratio [OR] 4.3, 95% confidence interval [CI] 1.9-9.6; p<0.0001). Moreover, female patients with lichen sclerosus had sinificantly more often associated autoimmune thyroid diseases (OR 4.7, 95% CI 1.8-11.9; p<0.0002), antithyroid-antibodies (OR 2.7, 95% CI 1.1-6.5; p=0.023), and elevated autoantibodies (OR 4.1, 95% CI 1.9-9.3; p<0.0001) as compared to male patients. This observation is suggestive for a different pathogenetic background in male and female patients.

HLA markers in familial Lichen sclerosus.

BACKGROUND: Lichen sclerosus (LS) has been identified with increased frequency in families,often associated with HLA markers, mainly DQ7. A genetic co-etiology seems likely in this setting. Moreover, there is an association of LS with autoimmune disorders, such as the presence of anti-thyroid peroxidase autoantibodies (anti-TPO), a hallmark of autoimmune thyroid diseases. PATIENTS AND METHODS: In 3 families affected by LS, we verified their HLA markers, and identified previously undiagnosed cases of LS and autoimmune disorders. 30 individuals were examined with history, skin biopsy, HLA class I and II typing by PCR-SSP, and measurement of anti-TPO, free thyroxine and thyroidstimulating hormones (TSH) levels. RESULTS: There were 8 cases of LS, 50 % of them anti-TPO+. Autoimmune disorders were found in 40 % (total) and in 87.5 % of those affected. Most common HLA markers were B*15, B*57, CW*03, CW*07, CW*18, DRB1*04, DRB1*07, DRB4*. The three latter have been previously associated with LS. CONCLUSION: New cases of LS and autoimmune disorders can be detected in first degree relatives of patients with LS. The presence of anti-TPO antibodies strongly suggests autoimmune thyroiditis. There is intra-familial association between the haplotype HLA-B*15 -DRB1*04 -DRB4* and anti-TPO,emphasizing their link with thyroiditis. New familial approaches might help to make clear the pathogenesis of LS and its association with autoimmune diseases.

Vulvar lichen sclerosus: comparative study of sexual plasmatic steroids

O líquen escleroso (LS) é uma doença de etiologia desconhecida, com marcada predileçäo pela vulva, em torno da menopausa. Há relatos de involuçäo espontânea de casos infantis na época da puberdade. O tratamento com testoterona tópica controla os sintomas e melhora a lesäo cutânea. Sugeriu-se que uma deficiência da enzima 5-alfa-redutase pudesse ser responsável pela doença. O objetivo deste estudo foi investigar alterçöes nos esteróides sexuais plasmáticos em mulheres co LS vulvar. Realizamos um estudo transversal controlado. Andrógenos e estrógenos foram dosados no plasma de 67 mulheres, entre 21 e 81 anos de idade, com LS vulvar antes de iniciarem o tratamento com testoterona tópica, em 23 destas pacientes durante o tratamento e num grupo controle de 30 mulheres com LS apresentaram níveis plasmáticos de estradiol e de testoterona livre superiores aos dos controles e niveis de DHEA-SO e de estrona inferiores. Durante o tratamento com testoterona tópica houve elevaçäo significativa da testoterona total. A involuçäo de estruturas anatômicas andrógeno-dependentes, assim como a alteraçäo de componentes da pele, também influenciados pelos andrógenos, característicos do LS vulvar, e as alteraçöes encontradas nos níveis plasmáticos dos esteróides sexuais destas pacientes fortemente sugerem que um distúrbio do metabolismo androgênico participe da patogenia do LS