Takayasu arteritis with pyoderma gangrenosum: case reports and literature review

Abstract

Abstract Background Takayasu arteritis is a rare, chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum is a skin complication that is a neutrophilic dermatosis characterized by destructive, necrotizing and noninfective skin infiltration. However, there are no related records on these conditions in Chinese patients. Case presentation We reported two Chinese female patients presenting with pyoderma gangrenosum associated with Takayasu arteritis. Pyoderma gangrenosum preceded Takayasu arteritis in both patients. Their skin lesions were diagnosed as pyoderma gangrenosum through skin biopsy and relieved after treating with steroids and immunosuppressants. During the follow-up, both patients developed symptoms caused by vascular stenosis and occlusion, such as dizziness and weakness of upper limb. The results of aortic angiography revealed multiple large arteries narrowed and blocked. According to the criteria of the American College of Rheumatology, the vasculitis in both patients were classified as Takayasu arteritis. Since there was scant evidence of active inflammation and the skin lesions were stable, neither of them was given strong immnosuppressive therapy. The PubMed database was also searched and 16 related well-documented cases of Takayasu with pyoderma gangrenosum were reviewed and summarized. Conclusions Pyoderma gangrenosum could occur at any stage of the Takayasu arteritis disease process. No correlation was found between the location of the skin lesions and the clinical severity and scope of Takayasu arteritis. It is important to remember the rare possibility of Takayasu arteritis in patients with skin lesions indicative of pyoderma gangrenosum of unknown aetiology. Obtaining the relevant history and regular monitoring of the arteries are necessary.