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

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.

Bibliographic Details

Main Authors:	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, Ferreira,Juliana C. O. A., Silva,Marco Felipe Castro, Ferriani,Mariana, Sakamoto,Ana Paula, Ferriani,Virginia Paes Leme, Centeville,Maraísa, Sato,Juliana, Santos,Maria Carolina, Bonfá,Eloisa, Silva,Clovis Artur
Format:	Digital revista
Language:	English
Published:	Sociedade Brasileira de Reumatologia 2019
Online Access:	http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2523-31062019000100201
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