Pyoderma gangrenosum and neutrophilic aortitis preceding the development of rheumatoid arthritis

Background. - We report a case of pyoderma gangrenosum (PG) associated with a complication comprising ascending aortic dissection (neutrophilic aortitis) in a setting of rheumatoid arthritis (RA). Patients and methods. - A 79-year-old female patient was hospitalized in late 2009 for vegetating PG. Treatment with general steroids followed by colchicine and topical steroids resulted in complete healing of skin lesions. During hospitalization, the patient presented dissection of the ascending part of the aorta, for which emergency surgery proved effective. Histological examination of the excised tissue revealed diffuse neutrophilic aortitis. Diagnoses of Takayashu's disease and of lupus were ruled out. A chest CT scan showed interstitial lung disease with mild lymphocytosis in the bronchoalveolar fluid, but with no isolated pathogenic organisms. Relapse of skin lesions occurred 3 and 4 years later, associated with the development of RA, and worsening of the interstitial lung disease was noted in a scan carried out it in 2013, following which stabilization was observed in April 2014. There was no recurrence of the PG lesions. Conclusion. - To our knowledge, no other cases involving association of neutrophilic aortitis with PG and RA has been published to date. The literature describes the emergence of the concept of systemic neutrophilic dermatoses, and this notion is reinforced by the presence of a cutaneous and aortic site of the neutrophilic disease in a single patient. (C) 2015 Elsevier Masson SAS. All rights reserved.