Page 398 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

372 Rami N. Al-Rohil and J. Andew Carlson

Figure 3. Large purpuric lesions with irregular borders affecting the left wrist of a patient with
idiopathic systemic vasculitis.

Table 2. Clinical manifestations of vasculitis based on vessel size affected.

Large vessel Medium vessel Small vessel

Limb claudication Subcutaneous nodules Purpura, infiltrated / elevated

Nodular erythema erythema

Asymmetric blood pressure Ulcers Urticaria

Absence of pulses Livedo reticularis Vesiculobullous lesions

Aortic dilation Pitted palmar/digital scars Splinter Hemorrhages

Bruits Digital Gangrene Scleritis, episcleritis, uveitis

Mononeuritis Palisaded neutrophilic

granulomatous dermatitis*

Aneurysms Glomerulonephritis

Infarct Gastric colic

Hypertension (renal Pulmonary hemorrhage

artery)

Constitutional symptoms: fever, weight loss, malaise, arthralgia and arthritis are common to vasculitic
syndromes of all vessel sizes. *Extravascular necrotizing granuloma. Small vessel neutrophilic

vasculitis is frequently seen in the vicinity of granulomas and necrosis. Adapted from [15].

The type of cutaneous lesions closely correlates with the size of vessel affected by
vasculitis. For example, in cutaneous LCV, immune complexes (IC) deposition and
inflammation mainly target post-capillary venules giving rise to small palpable purpura
(Figure 2). Inflammation that targets arterioles and arteries results in large purpuric lesions
with irregular borders (Figure 3) since these vessels supply multiple dermal papillae. Ulcers,
nodules, pitted scars, and livedo reticularis are associated with arterial muscular vessel
involvement, which is localized to the dermal-subcutis interface or within the subcutis [4].
Table 2 reviews the size of vessel affected with corresponding clinical presentation. The 2012
Revised Chapel Hill Consensus Conference (CHCC) [5] serves as a guide for the nosology
and categorization of diverse forms of vasculitis based upon the vessels involved. The
Pediatric Rheumatology European Society (PRES) and the European League against

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