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Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
Mônica Verdier1, Pedro Anuardo1, Natali Weniger Spelling Gormezano1,2
1Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Brazil.
Insights
Panniculitis is a rare skin manifestation in childhood-onset systemic lupus erythematosus (cSLE), often appearing within three years and causing significant sequelae. Most patients experience mild lupus symptoms alongside this skin condition.
Area of Science:
- Rheumatology
- Dermatology
- Pediatrics
Background:
- Childhood-onset systemic lupus erythematosus (cSLE) is a complex autoimmune disease with diverse clinical presentations.
- Skin manifestations are common in cSLE, but specific conditions like panniculitis are infrequently studied.
- Understanding rare manifestations like panniculitis is crucial for comprehensive patient management.
Purpose of the Study:
- To determine the prevalence of panniculitis in a multicenter cohort of cSLE patients.
- To characterize the clinical, laboratory, and histopathological features of cSLE-associated panniculitis.
- To compare outcomes and associated symptoms between cSLE patients with and without panniculitis.
Main Methods:
- A multicenter cohort study involving cSLE patients.
- Diagnosis of panniculitis based on clinical presentation (painful subcutaneous nodules/plaques) and skin biopsy findings (lobular/mixed panniculitis).
- Statistical analysis, including Bonferroni correction, to compare patient groups.
Main Results:
- Panniculitis was identified in 0.7% (6/847) of cSLE patients.
- Clinical features included painful subcutaneous nodules/plaques, with generalized or localized distribution. Cutaneous hyperpigmentation and atrophy were common (83%).
- cSLE patients with panniculitis showed significantly higher frequencies of constitutional involvement (67% vs. 10%) and leukopenia (67% vs. 7%) compared to those without.
Conclusions:
- Panniculitis is a rare but significant skin manifestation in cSLE, typically emerging within the first three years of disease.
- This condition can lead to considerable sequelae, including skin atrophy and hyperpigmentation.
- Most patients with cSLE-associated panniculitis present with generally mild lupus manifestations.
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.
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