Autor(es):
Verdier, Mônica ; Anuardo, Pedro ; Gormezano, Natali Weniger Spelling ; Romiti, Ricardo ; Campos, Lucia Maria Arruda ; Aikawa, Nadia Emi ; Pereira, Rosa Maria Rodrigues ; Terreri, Maria Teresa ; Magalhães, Claudia Saad [UNESP] ; Ferreira, Juliana C O A ; Silva, Marco Felipe Castro ; Ferriani, Mariana ; Sakamoto, Ana Paula ; Ferriani, Virginia Paes Leme ; Centeville, Maraísa [UNESP] ; Sato, Juliana [UNESP] ; Santos, Maria Carolina ; Bonfá, Eloisa ; Silva, Clovis Artur
Data: 2019
Identificador Persistente: http://hdl.handle.net/11449/189684
Origem: Oasisbr
Assunto(s): Childhood; Lupus erythematosus panniculits; Systemic lupus erythematosus and multicenter study
Descrição
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Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
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.
Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Brazil
Division of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César, São Paulo, SP, 05403-000, Brazil
Division of Dermatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Brazil
Pediatric Rheumatology Unit Universidade Federal de São Paulo
São Paulo State University (UNESP) - Faculdade de Medicina de Botucatu
Pediatric Rheumatology Unit Ribeirão Preto Medical School - University of São Paulo
São Paulo State University of Campinas (UNICAMP)
Irmandade da Santa Casa de Misericórdia de São Paulo
São Paulo State University (UNESP) - Faculdade de Medicina de Botucatu
São Paulo State University of Campinas (UNICAMP)
CNPq: 303422/2015-7 to CAS
