Ehlers-Danlos syndrome (EDS) refers to a heterogeneous group of heritable connective tissue disorder characterized by joint hypermobility, increase in skin elasticity and fragility, and vascular lesions. There are multiple clinical subtypes but distinction may be difficult due to a considerable overlap in phenotypic findings. Most patients with cerebrovascular complications have type IV (vascular) EDS.

We report a case of EDS with multifocal vascular complications in a 58 year old male who presented with intractable headache, and a six month history of significant weight loss and abdominal pain. MRI of brain and MRA of carotid, vertebral and abdominal vessels revealed dissections involving the left internal carotid artery (ICA) and bilateral vertebral artery, and stenosis of right renal and proximal coeliac arteries. Small left medial temporal ischaemic foci and a right renal infarction were identified. Small aneurysms of left intracavernous ICA and right anterior cerebral artery were also noted. Findings were reconfirmed on vascular ultrasound and CT angiogram. Differential diagnosis included vasculitides , fibromuscular dysplasia , segmental arterial mediolysis , Marfan’s syndrome, and pseudoxanthoma elasticum. Vasculitides were ruled out due to normal inflammatory and vasculitic markers. Patient did not fulfill the Ghent diagnostic nosology for Marfan’s syndrome.

The diagnosis of EDS was made based on the clinical findings of joint hypermobility , skin hyperelasticity and fragility along with the vascular changes in setting of a strong family history. The weight loss was considered to be the result of mesenteric ischaemia. The patient was managed by anticoagulation and strict control of blood pressure.