Objective: We report a case of a patient with a cutaneous polyarteritis nodosa who developed dysautonomia and review this association's literature.

Case report: A 75-year-old male patient was admitted with a recent history of recurrent ulcers on his lower limbs. A skin biopsy showed leukocytoclastic vasculitis and fibrinoid necrosis in arterioles. Antinuclear antibodies were positive in a titer of 1:320, with other autoantibodies being negative. A cutaneous polyarthritis nodosa (cPAN) diagnosis was determined based on cutaneous ulcers, compatible histopathology, and the exclusion of systemic manifestations. Methotrexate, folic acid, colchicine, and one single dose of betamethasone depot were administered with a good response, gradually healing the ulcers. However, after two years, the patient felt some episodes of syncope. Orthostatic hypotension (110x80 mmHg standing and 50x30 mmHg in supine position) was noted, and a clinical diagnosis of dysautonomia was determined. Fludrocortisone was started, and the patient felt better.

Conclusions: This patient illustrates the first case of cutaneous polyarteritis nodosa, which evolved to pre-syncope and syncope episodes due to dysautonomia. The possibility of cPAN evolving into a systemic condition is discussed.