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129 Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
  1. L Campos1,
  2. M Verdier2,
  3. P Anuardo2,
  4. N Gormezano2,
  5. R Romiti3,
  6. N Aikawa4,
  7. R Pereira4,
  8. M Terreri5,
  9. C Magalhaes6,
  10. J Ferreira2,
  11. M Silva2,
  12. M Ferriani2,
  13. A Sakamoto5,
  14. V Ferriani7,
  15. M Centeville8,
  16. J Sato6,
  17. M Santos9,
  18. E Bonfa4 and
  19. C Silva2
  1. 1SAO PAULO, Brazil
  2. 2Children’s Institute- Faculdade de Medicina da Universidade de São Paulo, Paediatric Rheumatology Unit, Sao Paulo, Brazil
  3. 3Faculdade de Medicina da Universidade de São Paulo, Division of Dermatology, Sao Paulo, Brazil
  4. 4Faculdade de Medicina da Universidade de São Paulo, Division of Rheumatology, Sao Paulo, Brazil
  5. 5Universidade Federal de São Paulo, Paediatric Rheumatology Unit, Sao Paulo, Brazil
  6. 6São Paulo State University UNESP – Faculdade de Medicina de Botucatu, Paediatric Rheumatology Unit, Sao Paulo, Brazil
  7. 7Ribeirão Preto Medical School – University of São Paulo, Paediatric Rheumatology Unit, Sao Paulo, Brazil
  8. 8São Paulo State University of Campinas UNICAMP, Paediatric Rheumatology Unit, Sao Paulo, Brazil
  9. 9Irmandade da Santa Casa de Misericórdia de São Paulo, Paediatric Department, Sao Paulo, Brazil

Abstract

Background and Aims 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. Generalised distribution was evidenced in 3/6 and localised in upper limbs in 2/6 and face in 1/6. 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), leukopenia (67% vs. 7%, p=0.002) and median C-reactive protein (10.5 vs. 0.5 mg/L, p=0.001). 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.

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