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.

A study of the clinico-histopathological features of erythematous tender nodules predominantly involving the extremities.

Background: Erythematous tender nodules predominantly involving extremities are frequently encountered in dermatology and rheumatology practice. They are diagnosed based on distinct clinical and histopathological features. However, in clinical situations, considerable overlap is observed that poses a diagnostic challenge. We undertook a study on clinico-histological patterns of inflammatory nodules over extremities. Methods: After detailed history and examination, a preliminary clinical diagnosis was made in 43 cases, followed by skin biopsy from representative nodules. Histological diagnosis made was correlated with clinical features. Results: Of 43 cases, a single clinical diagnosis was made in 25 (58.5%) cases while in the remaining cases more than one diagnosis was considered. On correlating with the histopathological diagnosis, concordance was observed in 51% cases while the remaining showed either histological discordance with clinical diagnosis (14% cases) or were kept in the undecided category (35% cases). Conclusion: Considerable clinico-histological overlap was observed in inflammatory nodules over extremities. Histopathology alone was not helpful in differentiating one entity from another at all times since variable histo-pathological patterns were seen.

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]

Systemic lupus erythematosus presenting as lupus panniculitis

Systemic lupus erythematosus [SLE] is an autoimmune disease that presents in diverse ways. Panniculitis is not a common presentation of SLE. Its overall incidence is estimated to be 1-3%, occurring predominantly in women. The lesions have predilection for the fatty areas of body such as shoulders, hips, thighs and face. We present here a case of a young female who visited us with the complaint of soft tissue swellings over buttocks, shins, arms and face. The swellings appeared to be ordinary abscesses at first sight. She also had history of arthralgia and low grade fever. Laboratory examination demonstrated high litres of antinucleal antigen and anti-double-stranded DNA antibodies in serum which led us to suspect lupus panniculitis. Histopathology revealed features of lobular panniculitis with lymphohistiocytic infiltrate deep in the subcutis. We report this case because this is a rare presentation of lupus erythematosus

Paniculitis Lúpica / Lupus panniculitis

La Paniculitis Lúpica es un proceso inflamatorio crónico y recurrente del tejido adiposo subcutaneo, en forma de nódulos o placas induradas, asociado o no manifestaciones cutáneas o sistémicas del lupus eritematoso, con histopatología característica. Presentamos el caso de una paciente mujer sin antecedente de lupus eritematoso, con paniculitis extensa de pared abdominal asociada a dolor que llevan a postración y con signos de lupus sistémico.

Lupus panniculitis is an inflammatory, chronic and recurrent process of the subcutaneous fat tissue in form of nodules or indurates plaques, associated or not to cutaneous or systemic manifestations of lupus erythematosus, with relatively characteristic histopathology. We present the case of a woman patient without antecedent of lupus erythematosus, with widespread panniculitis of abdominal wall associated to pain that take to prostration and with signs of systemic lupus.

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