Púrpura palpável como manifestação clínica inicial do lúpus eritematoso sistêmico juvenil mimetizando púrpura de Henoch-Schönlein Palpable purpura as the initial clinical manifestation of juvenile systemic lupus erythematosus similar to Henoch-Schönlein purpura

Objective: To report five cases of palpable purpura as the initial manifestation of juvenile systemic lupus erythematosus, similar to Henoch-Schonlein purpura. Case reports: From 1983 to 2004, 4.502 patients were followed at the Pediatric Rheumatology Unit of ICr-HC-FMUSP and the diagnosis of juvenile systemic lupus erythematosus was made in 211 (4.7%). Among them, five (2.4%) had palpable purpura not related to thrombocytopenia, painless and not pruritic, with an initial diagnosis of Henoch-Schonlein purpura. All patients were adolescents and four were female. The presence of other clinical manifestations and laboratory abnormalities occurred in these patients, namely: malar rash, oral ulceration, erythema of the soles, Raynaud phenomenon, pericarditis, leucopenia and limphocytopenia. All the patients had a positive antinuclear antibody with titers higher than 1/360, anti-DNA was observed in three, anti-Sm in one and antiphospholipid antibodies in two. The five patients presented nephritis with urinary sediment abnormalities and/or proteinuria higher than 0.5g/day, but only two had severe renal disease (diffuse proliferative glomerulonephritis) and required corticosteroids and pulse therapy with cyclophosphamide. Comments: The recognition of juvenile systemic lupus erythematosus by the pediatricians is very important, since this severe diseases associated to significant morbidity and mortality and needs early treatment. The finding of palpable purpura in adolescents, particularly in females, along with other manifestations not compatible with Henoch-Schonlein purpura indicates the need to determine auto-antibodies to exclude the diagnosis of systemic lupus.