Vasculitis means "inflammation of blood vessels," and generally, it involves arteries. The pathophysiology is still not completely understood, but in most cases, it seems to involve an autoimmune response where the body's immune system attacks its own blood vessels. This is a very simplified explanation, of course, but we do not need to dwell more on the details as wound care practitioners. Be aware, however, that the manifestation of vasculitis on the skin is only what we see on the surface. Usually, the arteries in the other body organs are also affected, and the patient may be very sick indeed. How severe the condition is depends on which size the involved arteries have. Obviously, the larger the arteries involved, the more serious the condition.
In many cases, we do not know what triggers this autoimmune reaction, but in some cases, it can be due to a medication the patient is using. Some conditions like malignancy, Behcet's Disease, and HIV are also associated with vasculitis. Particularly HIV related vasculitis is of relevance to the African continent. Vasculitis is a complex field, and the subgroups have exotic-sounding names like Takayasu arteritis, Kawasaki Disease, Polyarteritis nodosa, ANCA vasculitis, and IgA vasculitis.
There is very little epidemiological data relating to the occurrence of vasculitides in Africa. The condition is probably not rare in Africa- it is under-diagnosed. It is encouraging that there has recently been an increasing number of publications from the African continent about vasculitides. This reflects a growing recognition of these conditions by African physicians, and hopefully, this translates into an improvement in the diagnosis and management of this condition. Genga et al.,2018, have written an overview article about this ( see references)
As wound care practitioners, it is essential that we are aware that this condition exists and that we refer the patient to a specialist center if we suspect vasculitis. Remember: the treatment for vasculitis is always some form of immune-modulating systemic treatment like corticosteroids, methotrexate, azathioprine, cyclophosphamide, or rituximab, amongst others. Corticosteroids are usually still the main form of therapy, but medication choice will depend on the type and severity of the vasculitis and which organs are involved.
Depending on where you work, dermatologists or rheumatologists are often responsible for managing vasculitis disorders. Again, if you have a patient with ulcers suspicious of vasculitis, be aware that many organs can be affected and that the condition can potentially be fatal. Get the patient referred whenever possible. If you are working in a setting where referral to a higher level institution is not an option, you will have to start treatment with, for example, high doses of prednisolone yourself.
The challenging part is that many other conditions in tropical countries can give rise to ulcers that may look very similar to vasculitis ulcers but may need entirely different treatments. Differential diagnoses are tropical ulcers, atypical mycobacterial infections ( Buruli ulcer), leprosy (neuropathic ulceration), cutaneous tuberculosis, deep fungal infections, leishmaniasis, and opportunistic infections in patients with HIV. Pyoderma gangrenosum is another differential diagnosis you will have to keep in mind too.
Figure 1 Ulcers secondary to vasculitis at early stages in three different patients ( the two bottom pictures belong to the same patient). The tell-tale sign that these manifestations could be vasculitis is that multiple ulcerations appeared simultaneously.
Figure 2 Be aware that ulcers secondary to vasculitis can mimic venous or arterial ulcers! When vasculitis ulcers look like those in the images above, it is easy to go right into the trap. Many of us would think that the pictures above were most likely to be mixed ulcers- i.e., both venous and arterial. Many of us would do an ankle-brachial index, start with compression, and maybe refer the patient to a vascular consultation. Some months later, when our treatment plan doesn't seem to work, we may start thinking that these ulcers may have a different etiology altogether.
Both patients in the images above complained about severe pain from the ulcers, which is also an important clue. While arterial or venous ulcers ( or mixed ulcers) often give some degree of pain, it is often not so severe. In vasculitis ulcers, however, pain is usually quite severe. Remember that pyoderma gangrenosum is another important differential diagnosis here!
What are clues that can help us diagnose vasculitis as the cause of the ulcers?:
The patient says that the ulcer(s) suddenly appeared without any form of mechanical irritation.
In most cases, multiple lesions appear simultaneously
The ulcers are usually extremely painful, both at rest and when we try to debride them.
Vasculitis ulcers are often found on the extremities ( usually lower extremities), but they can appear anywhere on the body.
The ulcers are usually relatively small and with fairly well-defined edges. They can, however, also become large, as shown in figure 2.
It is common to see purplish spots (purpura) on other areas of the skin.
Keep in mind other differential diagnoses like malignant ulcers ( especially basal-cell carcinoma and spinocellular carcinoma) and auto-immune disorders like pyoderma gangrenosum. Also - always question yourself if it could be tropical ulcers, atypical mycobacterial infections ( Buruli ulcer), leprosy (neuropathic ulceration), cutaneous tuberculosis, deep fungal infections, leishmaniasis and opportunistic infections in patients with HIV.
It is very important to take multiple biopsies from the lesions under local anesthesia. These biopsies will quite often not show specific vasculitis signs unless you have a very dedicated pathology lab that is more specialized in this sort of histology. However, the biopsies will be helpful to rule out other conditions.
Treatment of vasculitis ulcers
Again, if you suspect vasculitis as the underlying cause of the ulcers, refer the patient to a higher level of care if this is an option where you work. Always ask the patient if they have started with a new medication, also ask for any traditional medicines the patient may take. Stop any new medications the patient has been taking previous to the outbreak of the ulcerations. Generally, do not attempt to debride the ulcers until the patient has started with anti-inflammatory treatment - usually prednisolone. If you can refer the patient to a specialist center, you should not take biopsies of the ulcers either- this will only delay the onset of correct treatment. Leave the diagnosing and management to the centers with more knowledge of this.
If you are working in Africa, where referral to a higher level of care is not an option for the patient, you may have to initiate the treatment yourself. This is by no means ideal, but we understand that this may be the only option in some situations. If you are convinced that you are dealing with vasculitis, you would start the patient on high doses of systemic corticosteroids and gradually taper down the dosage over a couple of weeks. We will not go into any details here as we primarily want to focus on the wound care aspect of vasculitis.
If you have to do the biopsy yourself, take the biopsies at the edge of the ulcerations - in the transitional area between the ulceration and skin. You can read more about biopsy taking in the separate chapter on this under "practical skills" in the main menu.
Again- try to avoid debriding the ulcers until the anti-inflammatory treatment has been initiated for at least 3-5 days. If you debride the ulcers without having the patient on anti-inflammatory medication, this can aggravate the ulcerations and increase their size! So be patient -even if there is skin necrosis, there is usually no hurry with getting the ulcers debrided. You may not even need to debride them if the ulcers are covered by dry necrosis.
Vasculitis ulcers are a good example of wounds where the treatment has to come from the inside ( anti-inflammatory treatment) - it is less important what we place on the outside of the ulcers. However, our choice of dressings should be such that they are easy to remove without causing the patient more pain. Foam dressings are usually a good choice here. If you have all resources available, then in our opinion, Polymem® foam dressings are an excellent choice here. We have had very good results with this unique foam dressing. It has anti-inflammatory properties, and in many cases, our patients have reported a significant reduction of pain when using Polymem®.
Figure 3 We have seen excellent results when using Polymem® on vasculitis ulcers. Unlike most foam dressings, Polymem® contains active ingredients. Most of our patients have reported a significant reduction in pain when using Polymem®. We have observed that the effect on pain is best when the dressing covers some of the areas with intact skin as well. However, be aware that the dressing itself cannot heal the vasculitis ulcer unless you also provide some systemic treatment. In these cases, the dressing is only a supplement for the treatment.
In very superficial vasculitis ulcers, you may reverse the condition using topical steroids. In most cases, however, you will need at least oral immunomodulating substances, in severe cases, even intravenous medications.
Often the ulcers will respond better when aided with compression therapy. Because many of these patients have considerable pain in their ulcers, compression therapy is not always well tolerated- at least in the start until the immunomodulating treatment has started to show signs of effect. So start very slow with the compression, gradually increasing the compression level as the patient tolerates.
Since vasculitis ulcers are usually quite painful, we also have to address the pain. Vasculitis-related ulcers often do not respond well to usual pain medication, and even opioids may be quite ineffective. Please also refer to our chapter on pain management for more information on this topic.