A  case of Hughes-Stovin syndrome (incomplete Behçet's disease) with extensive arterial involvement : Unmasking the true face of a rare syndrome.

We describe a 35-year-old man with HSS showing classical features of the syndrome in the form of recurrent thrombophlebitis of the lower limb veins, pulmonary arterial aneurysms, and left lower limb ischemia with extensive arterial tree involvement. The patient presented with critical arterial ischemia in the left lower limb together with aortic and left common iliac artery thrombosis, occlusion of the left superficial femoral artery, and occlusion of both lower limb arteries. Urgent vascular surgeries were carried out for limb salvage. Shortly after, the patient started on pulse corticosteroid/cyclophosphamide therapy, followed by monthly cyclophosphamide for 1 year, with much improvement. We discuss arterial involvement in HSS and similarities of HSS and BD regarding thrombotic events. We summarize the current management options of HSS. PMID: 30874932 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Rheumatologie - Category: Rheumatology Authors: Tags: Z Rheumatol Source Type: research