Burn Center Management of Severe Necrotic Cutaneous Polyarteritis Nodosa in a Patient With a History of Thymoma

Abstract

Cutaneous polyarteritis nodosa (cPAN) is a rare, necrotizing vasculitis involving the small- and medium-sized vessels of the dermis and subcutaneous tissues. We report a severe case of cPAN in a patient with an atypical presentation of extensive bilateral lower extremity ulcerations with full-thickness necrosis managed at a burn center. A 70-year-old female with a three-month history of necrotizing cPAN to the bilateral lower extremities underwent surgical excision and autografting at an outside hospital. Postoperatively, she had total graft loss and was begun on prednisone. In the outpatient setting, she was tapered off prednisone and subsequently began to experience an acceleration of the disease process. She was then transferred to our regional burn center for bilateral escharotomy and management of her non-healing, tender, necrotic wounds with distinctive black-brown eschar. One year later, the patient's wounds continue to decrease in size and heal with her daily regimen of 15 mg of prednisone, 50 mg of cyclophosphamide, and topical silver sulfadiazine application. With the increasing volume of non-burn wound admissions to burn centers primarily of dermatologic etiology, it becomes crucial for burn specialists to familiarize themselves with severe presentations of vasculitides, including cPAN. Further research is necessary to understand the atypical manifestations of this disease for more timely diagnosis and treatment.

Keywords: autoimmune; cpan; cutaneous polyarteritis nodosa; gangrene; necrosis; polyarteritis nodosa; thymoma; ulceration.

Figure 1. Bilateral (a) right and (b) left lower extremity necrotic, ulcerating wounds with black-brown eschar.

Figure 2. (a) H&E stain, x4 magnification, necrotizing vasculitis involving all the layers of the vascular walls of the medium and small-sized arteries. (b) H&E stain, x4 magnification, overlying necrotic epidermis.

H&E: Hematoxylin and Eosin