Abstract
ObjectiveTo 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.MethodsPanniculitis 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).ResultsPanniculitis 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.ConclusionsPanniculitis 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.
Highlights
Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs and systems
This study was conducted in 10 pediatric rheumatology services in the state of São Paulo, Brazil including a population of 847 childhood-onset SLE (cSLE) patients [3]
Painful subcutaneous erythematous and indurated nodules were observed in 6/6 Lupus erythematosus panniculitis (LEP) patients, and concomitant painful subcutaneous erythematous plaques in 4/6
Summary
Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs and systems. Mucocutaneous involvement was described as initial manifestation in up to 81% childhood-onset SLE (cSLE) patients and in. Lupus erythematosus panniculitis (LEP) is a rare form of chronic cutaneous lupus erythematosus described from 2 to 5% of adult SLE [4,5,6]. In cSLE, LEP data are limited to few case reports [7,8,9,10]. The objective of the present multicenter cohort study was to evaluate the prevalence of LEP and its possible association with demographic data, clinical manifestations, laboratory abnormalities,. (2019) 59:3 disease activity score, treatment and outcome in a large cSLE population
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