[Vitiligo in a patient with pachydermoperiostosis: a fortuitous association?]. / Vitiligo em paciente com paquidermoperiostose: uma associação fortuita?

Pachydermoperiostosis (PDP) is a rare hereditary disorder characterized by pachydermia, digital clubbing, arthritis and periosteal hypertrophy. We report the case of a patient with PDP who developed vitiligo. While the association of autoimmune diseases and some dermatological disorders with PDP has been reported before, this appears to be the first described case of vitiligo in association with PDP. The main clinical features of PDP are reviewed and the differential diagnosis is discussed.

[Hemophagocytic Syndrome: a clinical presentation of systemic lupus erythematosus]. / Síndrome hemofagocítica uma apresentação inicial do lúpus eritematoso sistêmico.

Hemophagocytic Syndrome is a clinical condition characterized by the activation of either macrophages or histiocytes with a prominent hemophagocytosis feature in the bone marrow and other reticuloendothelial systems. It leads to the phagocytosis of erythrocytes, leukocytes, platelets, and their precursors. The presence of hemophagocytosis can be associated to infections, malignancies, autoimmune diseases, drugs and a variety of other medical conditions. We report a case of a previously healthy 23 year-old woman that developed hemophagocytosis at the same time that she fulfilled diagnostic criteria for systemic lupus erythematosus. Lupus-related hemophagocytic syndrome is a rare and potentially fatal entity. It offers significant differential diagnosis challenges and requires urgent therapeutic intervention. There are only few cases reported in the literature. In this article, we briefly reviewed what is currently known about this syndrome. However, much is still needed in order to better understand its causes, all the immunopathogenic mechanisms, as well as its clinical aspects.