Urticarial vasculitis associated with castleman’s disease, the localised form - a case report
Castleman’s disease is a very rare disorder characterized by benign lymph-node hypertrophy. There are described two forms: the localized form, in which only one lymph node is involved, often pauci-symptomatic, treated by surgical excision with full recovery without relapse in almost all cases, and the multicentric or systemic form with general symptoms, polyadenopathy (neck, chest, mediastin, abdomen), organomegaly (liver, spleen) and, with a much less favorable prognosis. The etiology remains unknown, but some recent studies have suggested that the human herpesvirus 8 (HHV 8) can be involved, especially in the multicentric forms.
We present the case of a 60 year-old man who was admitted to our hospital for acute urticaria (with an aspect of vasculitic urticaria) and angioedema, started two weeks before presentation. At the physical examination we observed a submandibular round tumor (8 cm in diameter), firm-elastic to palpation, painless, covered by normal epidermis, which occurred simultaneously with urticaria and increased with time. We have treated the urticaria with antihistamines and pentoxifilin with a good response and we referred the patient to the Otolaryngology department where the tumor was completely excised. The histological examination of the lesion showed angiofollicular lymphoid hyperplasia the hyaline-vascular form, suggesting the Castleman’s disease. The computer tomography revealed no widespread lymphadenopathy, so the diagnosis was Castleman’s disease, the localized form. We periodically evaluate the patient; one year after surgical excision no relapse was noticed.