Inflammatory Vasculitis

Recommendations

Identify and Treat the Cause
1 Take a careful history (venous/ arterial characteristics, other diagnoses, infection, medication, coexisting diseases, factors that may impair wound healing) Level of Evidence
Not Assessed
2 Investigate suspected vasculitis to determine a specific diagnosis. Level of Evidence
Not Assessed
3 Perform a basic investigation, which includes a detailed history and physical examination; biopsy of a new lesion for pathology, Gram staining, immunofluorescence, and culture and sensitivity; and laboratory investigations to identify systemic involvement and other conditions. Level of Evidence
Not Assessed
4 Manage vasculitic ulcers with cause-specific therapy where possible, systemic immunosuppression, pain management and local wound care. Level of Evidence
Not Assessed
5 For nonhealable or maintenance wounds, provide support, pain control and modified local care (conservative debridement, bacterial and moisture reduction) Level of Evidence
Not Assessed


Address patient-centered Concerns
6 Communicate (patients, family, caregivers) to establish a social support system with realistic expectations for healing and to prevent leg ulcer recurrences. Level of Evidence
Not Assessed
7 Assess / Control pain and optimize activities of daily living. Level of Evidence
Not Assessed


Provide Local Wound Care
8 Assess and document the wound at regular intervals. Level of Evidence
Not Assessed
9 Optimize local wound care: debridement, inflammation / infection control, and moisture balance. Consider biopsy, if appropriate, active (including biologicals) & adjunctive therapies if the wound is not healing at the expected rate. Level of Evidence
Not Assessed


Provide Organizational Support
10 Consult appropriate disciplines to maximize healing (e.g. mobility and nutrition). Level of Evidence
Not Assessed


Background

Vasculitis, or inflammation of the blood vessel wall, often presents with one or more cutaneous signs, such as petechiae, purpura, blisters, deep nodules, ulcers and livedo. Systemic signs and symptoms may or may not accompany the cutaneous manifestations. Demographic factors often point to specific underlying vasculitic syndromes, as many may be more common in specific age or ethnic groups.

Leucocytoclastic vasculitis (LCV) describes palpable purpura, or raised vasculitic purpura on the legs. LCV is a cutaneous small-vessel vasculitis that may be limited to disease of the skin or accompanied by involvement of one or more organ systems. Approximately half of cases are idiopathic, and the remainder are associated with a variety of disorders, including infection, drug reaction, collagen vascular diseases, autoimmune disorders and malignancy. Infectious agents include bacteria (especially β-hemolytic Streptococcus), viruses (especially hepatitis C virus, which may be associated with cryoglobinemia), fungi, and parasites. Severe and disabling pain accompanies most LCV ulcers when cryoglobins are present, and pain management is a treatment priority in this situation. Large-vessel vasculitides are more likely to ulcerate than LCV but are less common. Vasculitis may also be associated with neuropathy, with development of a neuropathic ulcer.

Clinical features may suggest involvement of a specific size of vessel and specific systemic conditions:
♦ Palpable purpura is usually associated with vasculitis involving postcapillary venules. It may be seen with any vasculitis except giant-cell arteritis and Takayasu’s arteritis.
♦ Skin ulcers are usually associated with involvement of arterioles and small arteries. Polyarteritis, Churg-Strauss vasculitis, Wegener’s granulomatosis and hypersensitivity vasculitis are the most common causes.
♦ Gangrene in an extremity is associated with small-to-medium arteries and often related to polyarteritis, Churg-Strauss vasculitis or Wegener’s granulomatosis.
♦ Neuropathic ulcer usually involves small-artery vasculitis, most often due to polyarteritis, Churg-Strauss vasculitis, Wegener’s granulomatosis or cryoglobinemia.

The clinical approach to suspected vasculitis includes a detailed history and physical examination; biopsy of a new lesion for pathology, Gram staining, immunofluorescence, and culture and sensitivity; and laboratory investigations to determine organ involvement and the presence of other conditions.

Treatment of cutaneous vasculitis depends on the severity of cutaneous disease and any cause identified, the location and size of vessel involved, and the presence and severity of systemic disease. Generally, however, systemic immunosuppression is required to manage cases that are not self-limiting. Management of pain associated with vessel infarction is also an important consideration. Effective treatment of ulcers should recognize the increased fragility of the skin due to the purpura and skin breakdown and the presence of pain.

References

Essential Publications
1 Inflammatory Vasculitis – Iloprost Quality Indicator
 Type:
Veale DJ, Muir AH, Morley KD, Belch JJF. Treatment of Vasculitic Leg Ulcers in Connective Tissue Disease with Iloprost. Clinical Rheumatology 1995; 14(2): 187-190.
This study suggests that iloprost may be used as an adjunctive therapy for vasculitic leg ulcers. The three patients with Rheumatoid Arthritis and one patient with overlap connective tissue disease showed rapid improvement with healing of the vasculitic lesions and leg ulcers on iloprost therapy (all >50% reduction in ulcer diameter). There are many factors which determine the rate of healing of vasculitic leg ulcers including immunosuppressive therapy, concomitant treatment of infection, improved blood flow, bed rest, general health and nutrition.
2 Inflammatory Vasculitis – VAC Therapy Quality Indicator
 Type:
Zutt M, Haas E, Kruger U, Distler M, Neumann C. Successful Use of Vacuum-Assisted Closure Therapy for Leg Ulcers Caused by Occluding Vasculopathy and Inflammatory Vascular Diseases – A Case Series. Dermatology 2007; 214: 319-324.
This study shows that VAC is an excellent and effective alternative in the treatment of therapy-resistant chronic wounds caused by vasculopathy. It results in pain reduction in all of the five cases described.
3 Inflammatory Vasculitis – Hypercoagulability Quality Indicator
 Type:
Mekkes JR, Loots M, Van Der Wal A, Bos JD. Increased incidence of hypercoagulability in patients in leg ulcers caused by leukocytoclastic vasculitis. J Am Acad Dermatol 2004; 50: 104-7.
The purpose of the study was to investigate if patients with vasculitis ulcers have an increased incidence of hypercoagulability. Hypercoagulable disorders were found in 7 of 13(53%) patients with leg ulcers caused by leukocytoclastic vasculitis. The authors recommend that all patients with severe skin necrosis caused by histologically confirmed vasculitis be screened routinely for hypercoagulable disorders.


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