The Case Challenge series includes difficult-to-diagnose conditions, some of which are not frequently encountered by most clinicians but are nonetheless important to accurately recognize. Test your diagnostic and treatment skills using the following patient scenario and corresponding questions. If you have a case that you would like to suggest for a future Case Challenge, please contact us.
A 54-year-old woman presents to the rheumatology clinic for evaluation of discolored toes and nonhealing ulcers. Her symptoms began approximately 3 years earlier with red and purple discoloration of both feet, which extended proximally to the knees. These changes were more pronounced in the right foot and were exacerbated by cold weather. During the past 2 years, she began to notice small nonhealing ulcers on the tips of her toes. The ulcers were accompanied by pain, sensitivity, numbness, and tingling. She had no other systemic symptoms.
She had previously consulted a vascular surgeon. CT angiography of her chest, abdomen, and pelvis with runoff was unremarkable. An empiric trial of amlodipine and pentoxifylline improved the discoloration of her lower extremities, but her ulcers persisted.
Her local rheumatologist performed an extensive laboratory evaluation, which was notable for an elevated C-reactive protein level of 15.3 mg/L (reference range, < 10.0 mg/L). The erythrocyte sedimentation rate was normal. The evaluation also revealed normal or negative tests for antinuclear antibody (ANA) and antibodies to extractable nuclear antigens (ENA), antiphospholipid antibody panel, complement levels (C3, C4), serum and urine protein electrophoresis, antineutrophil cytoplasmic antibody (ANCA) screening, viral hepatitis panel, and tests for cryoglobulins and cold agglutinins. Genetic testing for adenosine deaminase deficiency was negative. She had previously had a nondiagnostic biopsy of one her ulcers.
The rheumatologist diagnosed livedoid vasculopathy and prescribed a short trial of oral prednisone (40 mg once daily), with partial improvement in her ulcers. This was followed by treatment with methotrexate. Over the next year, the ulcers on the left foot healed, but she continued to have intermittent digital ulcers on the right foot. She also noted the new development of small, nontender nodules on the medial aspect of the right foot.
During the current visit at the rheumatology clinic, a complete review of systems is performed. The patient says she has not had fevers, weight loss, oral ulcers, recurrent sinusitis, shortness of breath, cough, arthralgias, joint swelling, myalgias, muscle weakness, abdominal pain, nausea, vomiting, diarrhea, or melena.
Before her symptoms began about 3 years ago, she had been healthy, with no significant medical history. She denies using tobacco products, alcohol, or illicit drugs. Her family history is negative for autoimmune disease and early vascular disease.
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Cite this: Kelsey N. Rigby. Rheumatology Case Challenge: A 54-Year-Old Woman With Discolored Toes and Nonhealing Ulcers - Medscape - Apr 27, 2023.