ABSTRACT
Kaposiform hemangioendothelioma is a rare, locally aggressive or borderline vascular tumor that typically affects infants. It presents as a purpuric cutaneous lesion and may be associated with life-threatening coagulation disorders, such as the Kasabach-Merritt phenomenon. The differential diagnosis can be challenging based on clinical presentation alone. Imaging plays a crucial role in the diagnostic workup, particularly magnetic resonance imaging. We present a case report of a 4-month-old patient with an enlarging vinous cutaneous mass on the thigh and coagulation abnormalities. Magnetic resonance imaging revealed a large, infiltrative, soft-tissue lesion with poorly defined margins and heterogeneous enhancement, that involved all muscle compartments of the thigh and was associated with lymphedema, stranding of the subcutaneous fat and cutaneous thickening. These findings were consistent with kaposiform hemangioendothelioma of the thigh and the diagnosis was confirmed by histopathological characterization.
ABSTRACT
Immunoglobulin G4-related disease (IgG4-RD) is an immunomediated disease that can virtually affect any organ. Despite the pancreas being known as the most frequently involved organ, pulmonary and pleural IgG4-RD is being increasingly reported. The authors present two cases of IgG4-RD diagnosed in the same year, with different presentations and outcomes, in which the lung and pleural involvement were essential for the diagnosis. Recognizing IgG4-RD as a possible cause of chronic pleural effusion and/or thickening and lung abnormalities is important for an early diagnosis and prognosis improvement.
