title: Cutaneous polyarteritis nodosa localized to a region of lymphedema secondary to Streptococcus viridans cellulitis and multiple surgeries.
abstract: 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 15 cm 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 3 months 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.
authors.affiliation: Department of Pathology, Albany Medical College, Albany, NY, USA.
authors.forename: Andrew
authors.initials: A
authors.lastname: Matsumoto
authors.affiliation: Department of Pathology, Albany Medical College, Albany, NY, USA.
authors.forename: Rami N
authors.initials: RN
authors.lastname: Al-Rohil
authors.affiliation: Department of Pathology, Bassett Healthcare Network, Cooperstown, NY, USA.
authors.forename: Marina
authors.initials: M
authors.lastname: Bravin
authors.affiliation: Department of Pathology, Bassett Healthcare Network, Cooperstown, NY, USA.
authors.forename: Lori
authors.initials: L
authors.lastname: Anderson
authors.affiliation: Wadsworth Center, New York State Department Health, Albany, NY, USA.
authors.forename: Danielle
authors.initials: D
authors.lastname: Wroblewski
authors.affiliation: Department of Pathology, Albany Medical College, Albany, NY, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Carlson
mesh_terms: D001327:Autoimmune Diseases / Q000209:etiology / Q000473:pathology*; D002481:Cellulitis / Q000382:microbiology; D005260:Female; D006801:Humans; D008209:Lymphedema / Q000209:etiology / Q000473:pathology; D008875:Middle Aged; D010488:Polyarteritis Nodosa / Q000209:etiology / Q000473:pathology*; D012782:Shoulder / Q000601:surgery; D012867:Skin / Q000473:pathology; D013290:Streptococcal Infections / Q000150:complications; D034363:Viridans Streptococci
keywords: Streptococcus viridans; cellulitis; cutaneous polyarteritis nodosa; localized lymphedema; vasculitis