Behçet disease (incomplete) and cutaneous polyarteritis nosa

Abstract

A 42-year-old man with a 2-year history of left posterior uveitis and recurrent oral aphthous ulcers presented for evaluation of recurrent, erythematous, subcutaneous, tender nodules on his lower extremities. A biopsy specimen of the nodules showed a medium-sized-vessel vasculitis with a mild and moderate inflammatory cell infiltrate in the septae and lobules of the subcutaneous adipose tissue. These changes were consistent with cutaneous polyarteritis nodosa. Behçet disease is a multisystem inflammatory disorder that includes recurrent oral aphthous ulcers, and at least two of the following features: recurrent genital ulcers, eye lesions, skin lesions, and a positive pathergy test. Behçet disease and cutaneous polyarteritis nodosa have rarely been described in conjunction, and the suggestion has been raised that cutaneous polyarteritis-nodosa-like lesions may actually be a cutaneous marker of Behçet disease.