Cutaneous Polyarteritis Nodosa Localized to a Region of Lymphedema Secondary to Streptococcus viridans Cellulitis and Multiple Surgeries

Cutaneous polyarteritis nodosa (CPAN) is a chronic, indolent, single organ arteritis that generally presents with lower extremity nodules and/or livedo racemosa, accompanied by malaise and arthralgias. CPAN is often triggered by infection, commonly Group A streptococcal species, and is considered an autoimmune reaction. Scarring from surgery and obliterative lymphangiitis from bacterial cellulitis are the causes of lymphedema. Lymphedematous skin is predisposed to autoimmune disorders. Herein we report a 53-year-old woman who developed CPAN restricted to a localized area of the right upper arm-shoulder that had undergone multiple surgeries, complicated by episodes of Streptococcus viridans cellulitis. Clinically, a 15cm diameter plaque exhibited violaceous, reticulate margins, subtle papules and nodules and central livedo racemosa. Biopsy showed numerous foci of arteritis in active, subacute and reparative stages. In addition, a broad zone of fibrosis replaced the deep dermis-subcutis zone and harbored numerous dilated lymphatic vessels scar lymphedema. Treatment consisted of high potency topical corticosteroids under occlusion; remission after 3months therapy and follow-up. CPAN primarily affects the lower legs, a region of frequently affected by phlebolymphedema. This report of CPAN localized to an area of scar lymphedema underscores the importance of lymphatic function in the pathogenesis of CPAN.