Atypical presentation of systemic lupus erythematosus in a 16-year-old boy: Case report.

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease, characterized by a clear female predominance and a major clinical polymorphism. Some initial presentations, particularly in children and adolescents are exceptional. Our objective was to report an unusual initial presentation of SLE in a male adolescent.

Observation: We reported the case of a 16-year- old tunisian boy who was referred to the hematology emergency department because of a hemorrhagic syndrome. The evolution was marked by pancytopenia, prolonged fever and an anasarca with recurrent cardiac tamponade. Explorations concluded to SLE. The patient was started on pulse therapy of dexamethasone followed by immunoglobulin. He was also provided with pericardiocentesis with a favorable outcome.

Conclusion: SLE is characterized by clinical polymorphism, that's why diagnosis and management of complications could be difficult. SLE should be considered in differential diagnosis of many disorders, such as pancytopenia and cardiac tamponade.