Small-Vessel Vasculitis

Cutaneous leukocytoclastic vasculitis

Cutaneous idiopatic IgA leukocytoclastic vasculitis presenting as livedo vasculitis.


Clinical Background:

This 42 year old woman had a 2 year history of a macular eruption on the legs accompanied by arthritis of the ankles, constitutional symptoms (weakness) and Raynaud's phenomenon.
Two months before being admitted to our Department, she noticed the appearance of multiple painful ulcers on the lower extremities and patchy areas of erythema on the knees.
At physical examination she showed a purpuric eruption on the legs characterized by small non-palpable macules (2-4mm in diameter), several superficial ulcerations scattered around the ankles and slight livedo reticularis around the knees. The clinical appearance was that of a livedo vasculitis but the cutaneous biopsy disclosed an infiltrate of neutrophils in and around the vessel walls, fibrinoid necrosis and thrombi affecting the small and medium sized vessels of the dermis and overlying areas of necrotic epidermis, consistent with a diagnosis of leukocytoclastic vasculitis. Direct immunofluorescence showed IgA, C3 and C1q in the vessel walls.
Besides the finding of a slight increase in the ESR, CRP and the presence of ANA on HEp-2 cells (homogeneous pattern, low titre), all laboratory investigations were within normal values.
All other clinical investigations failed to disclose any sign of associated connective tissue, systemic or infectious disease or occult malignancy. She was discharged on prednisone (20mg/d) with some improvement.

All contents copyright of the "On-line Archives of Rheumatology", 1996