Rheumatology Case Challenge: A 54-Year-Old Woman With Discolored Toes and Nonhealing Ulcers

Kelsey N. Rigby, DO


April 27, 2023


The patient in this case presented with chronic nonhealing ulcers, subcutaneous nodules, and livedo reticularis. She had no pulmonary, renal, or other systemic symptoms. The differential diagnosis of her presentation includes small vessel vasculitis, polyarteritis nodosa (PAN), embolic disorder, thrombosis, atherosclerosis, fibromuscular dysplasia, catastrophic antiphospholipid syndrome, cryoglobulinemia, pernio (chilblains), vasculitis secondary to connective tissue disease, and adenosine deaminase deficiency.

Tests for ANA and antibodies to ENA, an antiphospholipid antibody panel, complement levels, ANCA screening, a viral hepatitis panel, and tests for cryoglobulins and cold agglutinins were negative in this patient. Imaging of her lower extremities did not reveal any atherosclerosis or large artery or venous abnormality. She had no history of catheterization, making an embolic disorder less likely. Histopathologic examination of a biopsy specimen showed fibrinoid necrosis of the vessel wall of a medium-sized muscular artery in the deep dermis, which was associated with a mixed infiltrate of neutrophils, lymphocytes, and histiocytes. These histologic changes are consistent with a diagnosis of PAN.[1]

In patients with pernio, histopathologic examination shows papillary dermal edema, both superficial and deep, with moderate to dense lymphocytic infiltrate surrounding vessels and endocrine glands.[2] Leukocytoclastic vasculitis is a small-vessel vasculitis, and histopathologic examination reveals neutrophil infiltration in the small-vessel walls. Immunofluorescence should be performed on the tissue sample. In a patient with leukocytoclastic vasculitis, immune deposition is present. Immunoglobulin A deposition is indicative of Henoch-Schonlein purpura.[3]   Raynaud phenomenon is recurrent vasospasm of the fingers and toes that occurs in the setting of cold or stress. It can be primary or secondary. Primary Raynaud disease is characterized by the occurrence of vasospasm without a secondary or associated illness. On physical examination, the results of nail capillaroscopy are normal, and necrosis is absent.[4]


Comments on Medscape are moderated and should be professional in tone and on topic. You must declare any conflicts of interest related to your comments and responses. Please see our Commenting Guide for further information. We reserve the right to remove posts at our sole discretion.