Cutaneous Small Vessel Vasculitis

 Cutaneous Small Vessel Vasculitis

Synonyms

·          Cutaneous leukocytoclastic vasculitis

·          Hypersensitivity angiitis/vasculitis variants confined to skin

·          Cutaneous necrotizing venulitis (necrotizing involving the predominantly venules) 

Key Features

·          Palpable purpura, urticaria or ulcers

·          Lesions on ankles/lower legs, dependent areas or pressure points

·          Involves small vessels (i.e. post capillary venules) only

·          Histologically with leukocytoclastic vasculitis (LCV)

·          Extracutaneous involvement occurs, but it is not usual

Cutaneous small vessel vasculitis is the most common type of vasculitis, and it primarily affects cutaneous post capillary venules. 

Epidemiology

Cutaneous small vessel vasculitis (CSVV) is seen in both children and adults.  Mean age of onset ranges from 34 to 49 years of age.  Women are affected more commonly than men, with ratios from 2:1 to 3: 1. 

Pathogenesis

Circulating immune complexes have been identified in a large percentage of patients with CSVV. 

Clinical Features

Typical primary skin lesion of CSVV is palpable purpura with lesions ranging from 1mm to several centimeters.  Early lesions may be macular, but they progress to papules, nodules, vesicles, plaques, bulla or pustules.  Other common findings include livedo reticularis, edema, and urticaria.  Secondary changes include ulceration, necrosis, and post-inflammatory hyperpigmentation.  Lesions most commonly occur on dependent areas, such as ankles and lower legs or other areas prone to stasis.

Local symptoms most commonly include pruritis, pain or burning.  Systemic symptoms include fever, arthralgias, myalgias, and anorexia. Gastrointestinal pain or neurological symptoms raise the suspicion that a cutaneous vasculitis may be associated with a systemic vasculitis.  

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