Atypical Wounds - Vasculitis

Vasculitis is an inflammatory disorder of blood vessels which can ultimately lead to organ damage, including the skin. Treatment of vasculitic wounds can be very tedious due to the intense pain that patients experience, especially in the early stages.

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Vasculitis is an inflammatory disorder of blood vessels, which can ultimately result in organ damage, including the skin. The etiology is often idiopathic—it is a reaction pattern that may be triggered by certain comorbidities including underlying infection, malignancy, medication, and connective tissue diseases such as systemic lupus erythematosus. Circulating immune complexes (antibody/antigen) deposit in the blood vessel walls, causing inflammation that may be segmental or may involve the entire vessel. At the site of inflammation, varying degrees of cellular inflammation and resulting necrosis or scarring occur in one or more layers of the vessel wall, and inflammation in the media of the muscular artery tends to destroy the internal elastic lamina.[1]

Leukocytoclastic vasculitis, a histopathologic term used to describe findings in small-vessel vasculitis, refers to the breakdown of inflammatory cells that leaves small nuclear fragments in and around the vessels. Vasculitic inflammation tends to be transmural, rarely necrotizing, and non-granulomatous. Resolution of the inflammation tends to result in fibrosis and intimal hypertrophy, which in combination with secondary clot formation can narrow the arterial lumen and account for the ischemia or necrosis of the tissue supplied by the affected vessels.[2] Clinical symptoms (ie, tissue loss) depend on the artery or arteries that are involved and the extent of lumen occlusion. Cutaneous vasculitis usually occurs in the lower extremities and feet. Vasculitic syndromes based on the affected vessels are listed in the following table:

Name

Typical vessels involved

Symptoms

Churg-Strauss syndrome

 

Small and medium vessel

3 stages:

  • Airway inflammation, asthma, allergic rhinitis
  • Hypereosinophilia
  • Vasculitis with tissue necrosis

 

Giant cell arteritis

Temporal and cranial arteries

Headaches, temporal pain, visual disturbances, scalp sensitivity, dry cough with respiratory symptoms, fever, upper extremity weakness and sensory changes

Henoch-Schönlein purpura

 

Small vessels

Purpura, arthritis, abdominal pain

(usually in children)

Immune complex–associated vasculitis

 

Small vessels to neurons

Peripheral neuropathy

Microscopic polyangiitis

 

Small vessels to organs

Ischemia, hemorrhage, loss of organ function

Polyarteritis nodosa

 

Small and medium arteries

Subcutaneous nodules or projections of lesions; fever, chills, tachycardia, arthralgia, myositis, motor and sensory neuropathies

Primary angiitis of the CNS

 

Small and medium vessels in the brain and spinal cord

Brain: headache, altered mental status, focal CNS deficits; spinal cord: lower extremity weakness, bladder dysfunction

Takayasu's arteritis.

 

Aorta, aorta branches, pulmonary arteries

Inflammatory phase with flu-like symptoms, pulseless upper extremity, claudication, renal artery disease

Wegener's granulomatosis (granulomatosis with polyangiitis)

 

Small and medium vessels

Organ failure (lung and kidneys), variable including skin, depending on the vessels involved

Note: The trend is to classify the diseases by descriptive names rather than by names referring to the discovering physician.

Adapted from Hamm R.  Why isn’t this wound healing?  In Schiffman M (Ed). Recent Clinical Techniques, Results, and Research in Wounds. Springer;2018.

Clinical presentation of cutaneous vasculitis, which varies depending on the arterial involvement, includes palpable purpura, livedo reticularis, pain, skin lesions with or without nodules, and tissue necrosis. It may present as one large necrotic lesion or several small lesions, but all are full thickness after debridement. Systemic symptoms may also be present and usually relate to kidney, lung, or gastrointestinal tract involvement. On some occasions, signs of vasculitis in other organs (e.g. symptoms of a CVA or lung disease) may appear at the same time that skin lesions appear. One very distinctive characteristic for differential diagnosis from chronic venous wounds is the exquisite pain that occurs with vasculitis, making the initial local treatment very tedious.

Treatment of any vasculitis depends on the etiology, extent, and severity of the disease. For secondary vasculitic disorders, treating the underlying comorbidity (eg, infection, drug use, cancer, or autoimmune disorder) is crucial.  Remission of life- or organ-threatening disorders is induced by using cytotoxic immunosuppressants (e.g., cyclophosphamide) and high-dose corticosteroids, usually for 3–6 months, until remission occurs or until the disease activity is acceptably reduced. Adjusted treatment to maintain remission takes longer, usually 1–2 years. During this period, the goal is to eliminate corticosteroids, reduce the dosage, or use less potent immune-suppressants as long as needed. After tapering or eliminating corticosteroids, methotrexate or azathioprine can be substituted to maintain remission.[3]

Initial treatment of wounds caused by vasculitis is extremely difficult because of the pain. The principles of standard wound care (debride necrotic tissue, treat inflammation and infection, apply moist wound dressings, nurture the edges) are recommended. Topical lidocaine helps reduce pain during treatments, non-contact low-frequency ultrasound helps mobilize cellular activity and interstitial fluids, and compression therapy helps manage the edema that occurs in the lower extremities as a result of the inflammation and decreased mobility. Non-adherent dressings that promote autolysis of the necrotic tissue (eg, X-Cell, Medline, Mundelein, IL) are excellent initially, especially in reducing pain levels with dressing changes. Silicone-backed foam dressings are helpful in absorbing exudate as well as in reducing pain. If the patient is on steroids, local vitamin A can be used to negate the effects of steroids. As the acute inflammation recedes, pain levels decrease, and wound healing progresses to proliferation, treatment can be more aggressive with the goals of full re-epithelialization and return to prior level of function.

 

[1] Lawley TJ, Kubota Y.  Vasculitis. Dermotol Clinic.  1990;8(4):681-687.

[2] Fauci A, Braunwalk E, Isselbacher KT, et al. Harrison’s Principles of Internal Medicine. New York, NY:McGraw-Hill;1998:989-991,1004-1019.

[3] Ntatsake E, Carruther D, Chakravarty K, et al.  BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis.  Rheumatology.  2014;53(12):2306-2309.

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Rose Hamm

Physical Therapy, University of Southern California

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