Pyoderma Gangrenosum as a Presenting Feature of Takayasu Arteritis

Abstract

Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta and its major branches. The disease has a female preponderance, and it presents with a wide variety of symptoms including skin manifestations, mainly ulcerative nodules, pyoderma gangrenosum, and erythema nodosum-like lesions. We report a case of a 50-year-old female who presented to the outpatient department with multiple ulcerative lesions over both upper extremities and chest. On physical examination, the patient had pulseless upper limbs. Laboratory investigations revealed positive antinuclear antibodies (ANA) and raised inflammatory markers. CT angiography of the aorta showed thickened aortic arch with the obliterated lumen of the left common carotid and left subclavian arteries. A biopsy of the skin lesion revealed surface ulceration and densely inflamed granulation tissue with a fibroblastic proliferation of deeper tissues. The patient had three out of six features of the American College of Rheumatology 1990 (ACR-1990) criteria for the classification of TA and was diagnosed with TA associated with pyoderma gangrenosum. The patient was managed with steroids and immunosuppressants along with gentle wound debridement with grafting of skin wounds. Since TA has varying presentations, its diagnosis is often challenging and requires a combined approach including clinical signs and symptoms, as well as laboratory and radiological workup. The disease also requires longterm follow-up due to its remitting and relapsing course.