MKSAP Quiz: lower-extremity pain and ‘red knots'
Beta-blockers may have benefit in COPD patients
A 25-year-old woman is evaluated for a 2-week history of bilateral lower-extremity pain and skin lesions that she describes as “red knots.” Ibuprofen has not alleviated her discomfort, and she has continued to develop new skin lesions. Six months ago, she developed vulvar ulcers that were negative for herpes simplex virus on a polymerase chain reaction assay; these lesions healed within 3 weeks. Two years ago, she developed uveitis that was treated with prednisolone drops. She also has a 7-year history of Raynaud phenomenon and a long-standing history of recurrent oral ulcers. She has had no recent infections and currently has no vulvar ulcers.
On physical examination, vital signs and cardiopulmonary and abdominal examinations are normal. There is no conjunctival injection. There are two ulcers on her tongue. Cutaneous examination reveals several subcutaneous reddish-colored nodules that are tender to palpation located on the lower extremities bilaterally. There is no synovitis, and range of motion of all joints is full.
Laboratory studies:
A chest radiograph is normal.
Which of the following is the most appropriate treatment for this patient?
A. Leflunomide
B. Penicillin
C. Prednisone
D. Sulfasalazine
MKSAP Answer and Critique
The correct answer is C) Prednisone. This item is available online to MKSAP 15 subscribers in the Rheumatology section, Item 6.
This patient's lower-extremity lesions are consistent with erythema nodosum. She also has oral ulcers, an elevated erythrocyte sedimentation rate, and a history of genital mucosal ulcers and uveitis. This clinical presentation strongly suggests a diagnosis of Behçet disease, and the most appropriate management for this patient is prednisone.
Diagnostic criteria for Behçet disease consist of the presence of oral ulcerations that recur at least three times in 1 year and at least two of the following manifestations: recurrent genital ulcerations, inflammatory eye disease, cutaneous lesions, and positive results on a pathergy test (characterized by a papule developing 48 hours after insertion of a 20-gauge needle intradermally). Patients with Behçet disease also may have central nervous system vasculitis; a nonerosive arthritis that involves the medium and large joints; and elevated markers of inflammation, such as the erythrocyte sedimentation rate. Other manifestations include arterial and venous thromboses, and patients with Behçet disease have a high mortality rate associated with arterial aneurysm rupture.
Treatment of Behçet disease is directed toward the involved organ system. Although erythema nodosum can be treated with NSAIDs, use of these agents has not alleviated this patient's symptoms. Treatment with a corticosteroid such as prednisone is therefore reasonable.
Leflunomide and sulfasalazine are indicated to treat rheumatoid arthritis but are not useful in patients with erythema nodosum or Behçet disease. Rheumatoid arthritis may present with Raynaud phenomenon and subcutaneous nodules. However, rheumatoid nodules typically develop on pressure points and are not tender or erythematous. Rheumatoid arthritis also is characterized by swelling and tenderness in and around the joints and may manifest as joint stiffness, synovial hypertrophy, synovitis, joint effusion, and loss of normal range of motion. These findings are absent in this patient.
Sulfasalazine also is used to treat inflammatory bowel disease, which may be associated with erythema nodosum. However, this patient has no gastrointestinal manifestations.
Key Point
- Treatment in patients with Behçet disease is directed toward the involved organ system.