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.

Single lesion leprosy pacients multicentric cohort treated with single dose drug therapy: findings on three year follow up and public health perspective in Brazil

Single skin lesion, paucibacillary (SSL PB) leprosy is considered an early disease manifestation. This study evaluated the clinical outcome of a cohort of 259 newly diagnosed SSL PB treated with one dose of rifampicin, ofloxacin, minocycline (ROM) and followed up three years. Patients were recruited from the North, Central West and Southeast regions in Brazil (1997-2001). The result expected with ROM therapy was disappearance or the reduction of lesion size. Manifestations that required additional intervention were considered as poor clinical outcome: type 1 reaction (T1R) with or without neuritis, neuritis alone, increase in lesion size and shift from paucibacillary to multibacillary. The incidence of poor clinical outcome was calculated by person month and with the Kaplan Meier methods. 61,8 percent of the participants were females, mean age 32.2 and 67,2 percent had borderline tuberculoid (BT) or tuberculoid forms. TIR was the predominant event; shift from paucibacillary to multibacillary was rare. 92 percent of the volunteers shown no events during the first year, the same occurring to 80,6 percent of them after 3 years of clinical monitoring. The probability of remaining event free was highest among those 40 years old or younger. Poor outcome predominated among BT patients. Extended monitoring of SSL PB leprosy cases under minimal therapy provided valuable case management information for reference centers.

Coorte multicêntrica de pacientes com hanseníase tratada com terapiade dose única: resultados de três anos de seguimento e perspectiva emsáude pública no Brasil / SINGLE LESION LEPROSY PACIENTS MULTICENTRIC COHORT TREATED WITH SINGLE DOSE DRUG THERAPY: FINDINGS ON THREE-YEAR FOLLOW-UP AND PUBLIC HEALTH PERSPECTIVE IN BRAZIL

Single skin lesion, paucibacillary (SSL-PB) leprosy is considered an early diseasemanifestation. This study evaluated the clinical outcome of a cohort of 259 newlydiagnosed SSL-PB treated with one dose of rifampicin, ofloxacin, minocycline (ROM)and followed-up for three-years. Patients were recruited from the North, Central Westand Southeast regions in Brazil (1997-2001). The result expected with ROM therapywas disappearance or the reduction of lesion size. Manifestations that required additional intervention were considered as poor clinical outcome: type-1 reaction (T1R) with orwithout neuritis, neuritis alone, increase in lesion size and shift from paucibacillary tomultibacillary. The incidence of poor clinical outcome was calculated by personmonthand with the Kaplan-Meier methods. 61.8% of the participants were females,mean age 32.2, and 67.2% had borderline tuberculoid (BT) or tuberculoid forms. T1Rwas the predominant event; shift from paucibacillary to multibacillary was rare. 92.0%of the volunteers shown no events during the first year, the same occurring to 80.6%of them after 3 years of clinical monitoring. The probability of remaining event-freewas highest among those 40 years old or younger. Poor outcome predominated amongBT patients. Extended monitoring of SSL-PB leprosy cases under minimal therapyprovided valuable case management information for reference centers.

Lesão única paucibacilar (SSL-PB) é considerada manifestação clínica inicial dahanseníase. Este estudo avaliou resultado clínico de coorte de 259 pacientes SSL-PBrecém-diagnosticados, tratados com esquema de dose única Rifampicina, Ofloxacina,Minociclina (ROM) e acompanhados por 3 anos (1997-2001) nas regiões Norte,Centro-Oeste e Sudeste. O resultado esperado do tratamento ROM compreendedesaparecimento ou diminuição da lesão. O desfecho foi definido como qualquerevento clínico com indicação de terapia adicional: reação tipo 1 (T1R) com ou semneurite, neurite, aumento de tamanho de lesão e mudança de paucibacilar paramultibacilar. Estas manifestações foram consideradas eventos clínicos desfavoráveis,calculados por densidade de incidência (pessoa-tempo) e por Kaplan-Meier. 61,8%dos participantes eram mulheres (32,2 média idade), 67,2% borderline-tuberculoide(BT) e tuberculoide. T1R foi o desfecho predominante; mudança de paucibacilar paramultibacilar foi rara. 92,0% não apresentaram eventos desfavoráveis no primeiro anoe 80,6% ao final de três anos de monitoramento clínico. Participantes com idade d?40 anos tiveram maior probabilidade de permanecerem sem evento e evolução clínicadesfavorável predominou entre pacientes BT. Monitoramento prolongado de hanseníaselesão-única PB tratados com esquema mínimo forneceu dados importantes sobremanejo clínico para os centros de referência.

Single lesion leprosy pacients multicentric cohort treated with single dose drug therapy: findings on three-year follow-up and public health perspective in Brazil

Single skin lesion, paucibacillary (SSL-PB) leprosy is considered and early disease manifesation. This study the clinical outcome of a cohort of 259 newly diagnosed SSL-PB treated with one dose of rifampicin, ofloxacin, minocycline (ROM) and followed-up for three-years. Patients were recruited from the North, Central West and Southeast regions in Brazil (1997-2001). The result expected with ROM therapy was disappearance or the reduction of lesion size. Manifestation that required additional intervention were considered as poor clinical outcome: type-1 reaction (T1R) with or without neuritis alone, increase in lesion size and shift from paucibacillary to multibacillary. The incidence of poor clinical outcome was calculated by person-month and with the Kaplan-Meier methods. 61.8% of the participants were females, mean age 32.2, and 67,2% had borderline tuberculoid (BT) or tuberculoid forms. T1R was the predominant event; shift from paucibacillary to multibacillaru was rare. 92.0% of the volunteers shown no events during the first year, the same occurring to 80.6% of them after 3 years of clinical monitoring. The probability of remaining event-free was highest among those 40 years old or younger. Poor outcome predominated among BT patients. Extended monitoring of SSL-PB leprosy cases under minimal therapy provided valuable case management information for reference centers.