RESUMO
OBJECTIVE: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. METHODS: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). RESULTS: Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0-11.4) vs. 2.83(0-11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. CONCLUSIONS: Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Paniculite de Lúpus Eritematoso/etiologia , Adolescente , Idade de Início , Brasil/epidemiologia , Criança , Estudos de Coortes , Feminino , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Paniculite de Lúpus Eritematoso/tratamento farmacológico , Paniculite de Lúpus Eritematoso/epidemiologia , Paniculite de Lúpus Eritematoso/patologia , Prevalência , Adulto JovemRESUMO
Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). Results: Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0-11.4) vs. 2.83(0-11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions: Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.
Assuntos
Adolescente , Criança , Feminino , Humanos , Masculino , Adulto Jovem , Paniculite de Lúpus Eritematoso/etiologia , Lúpus Eritematoso Sistêmico/complicações , Brasil/epidemiologia , Paniculite de Lúpus Eritematoso/patologia , Paniculite de Lúpus Eritematoso/tratamento farmacológico , Paniculite de Lúpus Eritematoso/epidemiologia , Prevalência , Estudos de Coortes , Idade de Início , Lúpus Eritematoso Sistêmico/epidemiologiaRESUMO
La piel está comprometida en la mayoría de los pacientes con lupus eritematoso. Las lesiones cutáneas más frecuentes son el rash malar, la fotosensibilidad, la alopecia, lupus discoide, lesiones en mucosas, pigmentación, vasculitis, fenómeno de Raynaud y urticaria. Menos frecuentemente los pacientes con lupus pueden desarrollar variantes clínicas inusuales tales como lupus eritematoso ampollar, lupus pernio, lupus eritematoso simil eritema gyrate y lesiones simil-eritema multiforme (síndrome de Rowell). Presentamos cinco pacientes con estas manifestaciones poco frecuentes. El reconocimiento de esta presentación es necesaria para un diagnóstico y tratamiento precoz y adecuado
Assuntos
Humanos , Feminino , Adolescente , Adulto , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Pérnio/fisiopatologia , Lúpus Eritematoso Cutâneo/classificação , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/patologia , Paniculite de Lúpus Eritematoso/tratamento farmacológico , Paniculite de Lúpus Eritematoso/patologiaRESUMO
La piel está comprometida en la mayoría de los pacientes con lupus eritematoso. Las lesiones cutáneas más frecuentes son el rash malar, la fotosensibilidad, la alopecia, lupus discoide, lesiones en mucosas, pigmentación, vasculitis, fenómeno de Raynaud y urticaria. Menos frecuentemente los pacientes con lupus pueden desarrollar variantes clínicas inusuales tales como lupus eritematoso ampollar, lupus pernio, lupus eritematoso simil eritema gyrate y lesiones simil-eritema multiforme (síndrome de Rowell). Presentamos cinco pacientes con estas manifestaciones poco frecuentes. El reconocimiento de esta presentación es necesaria para un diagnóstico y tratamiento precoz y adecuado (AU)
Assuntos
Humanos , Feminino , Adolescente , Adulto , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/patologia , Lúpus Eritematoso Cutâneo/classificação , Paniculite de Lúpus Eritematoso/patologia , Paniculite de Lúpus Eritematoso/tratamento farmacológico , Pérnio/fisiopatologiaRESUMO
Paciente femenina con lupus eritematoso sistémico y alteraciones de la función pulmonar que desarrolló manifestaciones cutáneas de lupus profundo. No se encontró en la literatura pediátrica revisada esta asociación. Se trató con esteroides de dosis bajas, cloroquina y ciclofosfamida con lo que desaparecieron todas las manifestaciones. La frecuencia de paniculitis lúpica en niños admitidos en el Instituto Nacional de Pediátria de 1970 a 1991 es del 1.4 por ciento, similar al 2 por ciento descrito en adultos