Clinical manifestations

The typical primary skin lesion of CSVV is palpable purpura with lesions ranging in size from

1mm to several centimeters (Figs. 1 and 2). The lesions arise as a simultaneous 'crop', resulting from the exposure to an inciting stimulus. Usually macular in the early stages, they may progress to wide array of lesions including, papules, nodules, vesicles, plaques, bullae, or pustules. Secondary finding include ulceration, necrosis, and post-inflammatory hyperpigmentation (Fig. 3). Other cutaneous findings include livedo reticularis, edema, and urticaria. Lesions most commonly occur on dependent areas, such as ankles and lower legs, or other areas prone to stasis (Ekenstam and Callen, 1984; Martinez-Taboada et al., 1997; Sais et al., 1998; Blanco et al., 1998).

Although normally asymptomatic, local symptoms may include pruritus, pain, or burning. Complaints of systemic symptoms, including fever, arthralgias, myalgias, anorexia, or gastrointestinal pain should raise the suspicion that a cutaneous vasculitis may be associated with a systemic vasculitis. In a study conducted by Ioannidou (2002) renal involvement in patients with CSVV led to the reclassification as Henoch-Schonlein purpura, microscopic polyangiitis, or Wegener's granulomatosus in 29-90 patients.

Cryoglobulinemia and chronic hepatitis C infection have been associated with chronic relapsing CSVV (Martinez-Taboada et al., 1997). The prognosis of isolated CSVV is generally quite good. A case series performed in a private practice by Ekenstam and Callen (1984) reported on 82 patients with CSVV, 51% of which had systemic

Figure 1. Cutaneous small vessel vasculitis. Note the purpura on the anterior aspect of the leg.
Palpable Purpura
Figure 2. Cutaneous small vessel vasculitis. Palpable purpura and early central necrosis on the distal lower extremity. (Courtesy of Dr. Kelly Barham, Wake Forest University School of Medicine, Winston-Salem, NC, USA.)

involvement of one or more systems. Fifty-six percent had acute, 28% had chronic, and 16% had relapsing disease. Two point four percent of patients died which represented only patients with acute extensive systemic disease involvement. More recent studies performed in a referral center confirmed the benign nature of CSVV, however, 10% of patients were found to have systemic vasculitis (Martinez-Taboada et al., 1997).

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