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.

Case 1

A 7 - year - old Caucasian girl presented with a 3-month history of nodules on her thighs and buttocks. During a one-month period, the lesions gradually increased in size and number. They became indurate and tender. Similar nodules appeared on forearms after 4 months. The lesions gradually healed with a depressed scar. On physical examination, we found many indurate areas 10-40 mm in diameter .The surface of some of them was erythematous and scaly. The lesions were not ulcerated and did not have a discharge [figure 1]. On examination, she was afebrile and healthy, organs such as heart, lungs, abdomen, joints and muscles were normal. Laboratory evaluation findings including complete blood cell count, liver and renal function test values, ESR, CRP,VDRL, BUN, Creatinin, complement were all normal .The titers for FANA, Anti ds DNA, Anti Sm, Anti Scl-70, Anti -RNP, Anti-Ro, Anti-La, Anti -Jo1, Anti Phospholipid Ab, Anti cardiolipin Ab, c- ANCA, p- ANCA were negative. A skin biopsy was taken [figures 2]

Lupus eritematoso: formas infrecuentes / Lupus erythematosus: uncommon manifestations

La piel está comprometida en la mayoría de los pacientes con lupus eritematoso. Las lesiones cutáneas más frecuentes son el rash malar, la fotosensibilidad, la alopecia, lupus discoide, lesiones en mucosas, pigmentación, vasculitis, fenómeno de Raynaud y urticaria. Menos frecuentemente los pacientes con lupus pueden desarrollar variantes clínicas inusuales tales como lupus eritematoso ampollar, lupus pernio, lupus eritematoso simil eritema gyrate y lesiones simil-eritema multiforme (síndrome de Rowell). Presentamos cinco pacientes con estas manifestaciones poco frecuentes. El reconocimiento de esta presentación es necesaria para un diagnóstico y tratamiento precoz y adecuado

Lupus eritematoso sistémico de inicio infantil con manifestaciones de lupus profundo y alteraciones de la función pulmonar / Systemic lupus erythematosus of early appearance with manifestations of profund and pulmonary function involvement: study of one patient and review of the literature

Paciente femenina con lupus eritematoso sistémico y alteraciones de la función pulmonar que desarrolló manifestaciones cutáneas de lupus profundo. No se encontró en la literatura pediátrica revisada esta asociación. Se trató con esteroides de dosis bajas, cloroquina y ciclofosfamida con lo que desaparecieron todas las manifestaciones. La frecuencia de paniculitis lúpica en niños admitidos en el Instituto Nacional de Pediátria de 1970 a 1991 es del 1.4 por ciento, similar al 2 por ciento descrito en adultos