Vasculitis, Leukocytoclastic

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ICD Codes

L95.8 – Other vasculitis limited to the skin

709.1 – Vascular disorders of skin


Leukocytoclastic vasculitis (LCV), or cutaneous small vessel vasculitis, describes a heterogeneous group of disorders that are uniformly characterized by purpuric or erythematous papules, vesicles, urticarial lesions, or petechiae. The end-stage phenotype of LCV is an inflammatory process of small blood vessels due to a complex interplay of immune complex deposition, autoantibody production, complement activation, inflammatory cell activation, and mast cell degranulation. From a clinical perspective, different cutaneous features are seen in medium to large vessel vasculitis and include subcutaneous nodules, retiform purpura, ulcers, and livedo reticularis.

LCV can occur in all ages and in both sexes. It is more commonly found in adults, but up to 10% of cases are in the pediatric population. Clinical features include a single eruption of palpable purpuric papules or nodules, vesicles, urticarial plaques, or petechiae cropping up in dependant areas approximately 1 week after an inciting factor. A variety of inciting factors have been identified, including medications (especially antibiotics, NSAIDs, and diuretics), pathogens (hepatitis viruses, HIV, streptococci), foods or food additives, malignancy, inflammatory bowel disease, or collagen vascular diseases. Up to 50% of cases, however, have no identifiable cause and are considered idiopathic.

While the majority of cases are asymptomatic, LCV can be associated with pruritus, pain, or burning. A skin biopsy will greatly aid in confirming the diagnosis. Significantly, the physician will need to differentiate LCV from a systemic vasculitis and should suspect the latter if fever, myalgias, malaise, lymphadenopathy, abdominal pain, melena, hematochezia, diarrhea, hematuria, lower extremity swelling, or paresthesias are noted. The level of systemic involvement will largely influence the prognosis. Over 90% of patients with LCV limited to the skin will experience spontaneous resolution over several weeks to months.

Variants or subcategories of LCV include the following:
  • Henoch-Schönlein purpura – Acute onset of palpable purpura usually seen in children aged younger than 10 years in the lower extremities/buttocks 1-2 weeks after a respiratory infection. Fever, arthralgias, and renal and gastrointestinal involvement are commonly seen. Can occur in adults.
  • Urticarial vasculitis – Recurrent, painful eruptions of urticarial lesions that last for more than 24 hours (assists in differentiating from chronic urticaria) with or without angioedema. Fever, malaise, myalgias, and arthritis are commonly associated. Urticarial vasculitis can be observed with systemic lupus erythematosus, Sjögren's syndrome, and viral infections. Complement levels can predict systemic involvement; normal levels seen with cutaneous limited disease, hypocomplementemic levels seen with arthritis, and gastrointestinal and pulmonary involvement.
  • Erythema elevatum diutinum – Rare; violaceous papules and plaques that favor the extensors symmetrically. Chronic course with spontaneous resolution after 5-10 years.

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