Page 413 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Dermatologic Aspects of Systemic Vasculitis 387

vasculitis was found overall about one-half of their 348 patient cohort of Pagnoux and
coworkers [45] with PAN including, 57.8% of 225 HBV-seronegative and in 35% of those
HBV-seropositive. Interestingly, they also found on multivariate analysis that the presence of
cutaneous manifestations at diagnosis, especially nodules, was associated with a higher risk of
relapse [45]. Agard and colleagues [22] reported cutaneous involvement as the initial finding
in 11% of 36 patients with MPA and PAN.

Figure 17. Severe cutaneous polyarteritis nodosa with secondary livedoid vasculopathy. Reproduced
courtesy of Henry Foong, MD, Ipoh, Malaysia).

Cutaneous PAN (CPAN), an indolent form of PAN confined to the skin, is suggested in
patients that present with tender nodules, livedo vasculopathy, livedo racemosa, ulcers, acral
gangrene and neuropathy [1, 4, 46]. Deep punch and incisional biopsy demonstrates
neutrophilic muscular vessel vasculitis at arterial branch points located at the dermal-subcutis
junction or within the subcutis. Macular arteritis [47-50] and lymphocytic thrombophilic
arteritis [51] are related variants of CPAN that represent latent or late evolutionary stages of
the disease and manifest lymphocytic vasculitis [47, 52]. Nodular vasculitis or erythema
induratum of Bazin, a lobular panniculitis and vasculitis of venules and septal veins [53] can
be mistaken for CPAN [1, 4, 46]. Their differentiation is possible by considering the relative
silhouette and patterns of elastic tissue distribution [54, 55] such that in contrast to the
rounded contour of arteries, venous vessels have oval silhouettes with few or no elastic fibers.

Large Vessel Vasculitis

Giant Cell Arteritis

This disorder which occurs in fair-skinned Caucasian individuals of older age females
presents with headache, jaw claudication, visual and neurological disturbances resulting from

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