
Differential Diagnosis
- Antiphospholipid antibody syndrome
- Cryoglobulinemia
- Livedoid vasculopathy
- Polyarteritis nodosa
Diagnosis
The correct diagnosis in this case is antiphospholipid antibody syndrome (APS)—an acquired autoimmune disease characterized by the formation of autoantibodies against various phospholipids. These antibodies cause an increased propensity for clotting by interfering with the function of proteins C and S, as well as directly interacting with platelets and the endothelium. APS is commonly attributed to an underlying autoimmune disease, such as systemic lupus erythematosus or less commonly HIV or hepatitis C. Additionally, several medications are associated with APS, including chlorpromazine, hydralazine, and procainamide.
Symptoms vary depending on the organ system involved. The most common thrombotic events occur in the deep venous system, usually in the leg. Respiratory compromise may signal a pulmonary embolism. Obstetric complications include premature delivery, unexplained fetal loss beyond 10 weeks of gestation, or 3 or more episodes of unexplained consecutive spontaneous abortions before 10 weeks of gestation. Neurologic deficits include severe migraine headaches, visual disturbances, and stroke. In rare cases, catastrophic antiphospholipid antibody syndrome can develop, characterized by rapid development of widespread thrombotic disease involving at least 3 organ systems, which can include the skin.
What to Look For
- APS may present with painful cutaneous ulcers and necrosis
- Look for evidence of thrombosis in the organ system affected (for example, leg swelling indicating a deep vein thrombosis or shortness of breath indicating a pulmonary embolism)
Pearls for Urgent Care Management
- In this case, appropriate wound care for the cutaneous ulcer depending on location and depth is needed
- Pain management considering topical anesthetic agents is indicated
- Refer to rheumatology for further evaluation and treatment
- Referral to the emergency department is advisable if evidence of severe disease is observed (eg, deep vein thrombosis, pulmonary embolism, stroke)
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