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Vasculitis Update

A summary of vasculitic conditions, with a focus on manifestations other than leg purpura

For dermatologists, vasculitis manifests most frequently as palpable purpura, often on the legs. The authors of a recent review focused on other vasculitis forms. Here are some pearls:

• Giant cell arteritis often presents with headache, fever, jaw or tongue claudication, and scalp tenderness. Nodules, tenderness, and absent pulse over the temporal artery are common. The erythrocyte sedimentation rate, platelet count, and alkaline phosphate levels are high and the red cell count low. Immediate systemic corticosteroids can ward off the dreaded associated blindness.
  
• Takayasu arteritis damages the aorta and its branches, causing headaches, dizziness, hypertension, bruits, asymmetric pulses, and asymmetric blood pressure — but seldom skin disease.
  
• Wegener granulomatosis affects smaller vessels, most often those in the lungs and kidneys. Nosebleeds, nasal pain, nasal crusting, unresolving sinusitis, and propensity to venous thrombosis and embolism are other possibilities. A finding of serum antibodies to proteinase 3 (C-ANCA) is more than 90% specific.
  
• Microscopic polyangiitis shares some features with Wegener granulomatosis but involves skin in about 50% of patients. Other vulnerable organs are kidneys (~80% of patients), lungs (~20%), joints (~70%), gastrointestinal tract (~40%), and peripheral nerves (~20%). Churg-Strauss syndrome is rare but causes asthma, fever, and eosinophilia (absolute eosinophil count >1500). Check for myeloperoxidase-anticytoplasmic antibody (p-ANCA) in serum.
  
• Polyarteritis nodosa causes fever, weight loss, malaise, night sweats, hypertension, and involvement in skin, muscle, bone, nerves, heart, gut, and renal arteries. Angiograms show microaneurysms, stenosis, occlusion, and beading of medium arteries.
  
• Cryoglobulinemic vasculitis is classified separately from other small-vessel leukocytoclastic vaculidities. Blood contains cryoglobulins, and disease occurs in skin (100% of patients), joints (73%), and kidneys (55%).
  
• Kawasaki disease affects children as an acute febrile illness with red mucosae, brawny induration of the extremities (followed by desquamation), and cervical adenopathy (50%–75% of patients). Aneurysms occur in 15%–25% of untreated patients, so prompt diagnosis and treatment are important.
  
• Henoch-Schönlein purpura is another small-vessel vasculitis classified separately from other leukocytoclastic vaculidities. Palpable purpura (100% of patients), arthritis (82%), abdominal pain (63%), nephritis (40%), and gastrointestinal bleeding (33%) are possible symptoms.
  
• Urticarial vasculitis, as the name suggests, produces urticarial wheals. Compared with urticaria, the lesions last longer and are more livid. Wheals hurt rather than itch and leave residual pigmentation after they resolve. Skin biopsy confirms leukocytoclastic vasculitis, and serum complement levels may be depressed or normal.
  

Comment: Nothing too new here, but a nice review and reference. I recommend repeat urinalysis for all patients with leukocytoclastic vasculitis. Diascopy of wheals of urticarial vasculitis (pressing on them with a glass slide or dermoscope) often reveals otherwise occult microscopic purpura.

— Mark V. Dahl, MD

Published in Journal Watch Dermatology July 17, 2009

Citation(s):

Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226.

• Medline abstract (Free)

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