Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study

• Autor(a) principal: Verdier, Mônica
• Data de Publicação: 2019
• Outros Autores: 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
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UNESP
• Texto Completo: http://dx.doi.org/10.1186/s42358-019-0049-9; http://hdl.handle.net/11449/189684
• topic: Childhood; Lupus erythematosus panniculits; Systemic lupus erythematosus and multicenter study

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.