Amongst the many vasculitis or blood vessel inflammation related diseases, one of the most significant one or rather the most common skin disorder is the Leukocytoclastic Vasculitis. It is also commonly known as Hypersensitivity Angiitis and Cutaneous Necrotizing Venulitis. Leukocytoclastic Vasculitis is caused mainly due to the inflammation in the small blood vessels, the basic characteristic of this condition is purpura, or purple rashes all over the skin.
Definition of Leukocytoclastic Vasculitis:
Leukocytoclastic Vasculitis is a skin disorder or condition that is caused by the inflammation of the small blood vessels, occurring and affecting any of the blood vessels in the skin – venules, capillaries, lymphatics and arterioles etc.
Signs and Symptoms of Leukocytoclastic Vasculitis:
Leukocytoclastic Vasculitis has some very general or non-specific symptoms that are quite common in almost all types of vasculitis disorders. Sometimes the symptoms are mistaken with other diseases and disorders; however, they are rarely associated with Leukocytoclastic Vasculitis.
The common symptom of Leukocytoclastic Vasculitis is rash like development on the skin. The cause of this is the inflammation in the blood vessels that damage the vessels and make them leaky. The blood vessels thus cause haemorrhage like purple-red appearances on the skin. These haemorrhage like appearances are known as palpable purpura as they are raised lesions. Such raised lesions are seen to be occurring in multiple areas on the legs or other body parts and these discrete, grouped lesions are usually painful and itchy.
Usually these lesions are present for 1 to 4 weeks on the skin. When they are associated with painful, large and ulcerated blisters, it indicates that the inflammation of the blood vessel is severe. At times, the signs of this condition may also be associated with –
- Muscle aches
- Abdominal pain
- Joint pain
- Bloody stool
- Numbness and weakness
If all of these signs and symptoms are persistent, it indicates that it is time to visit the doctor.
Causes of Leukocytoclastic Vasculitis:
There is no fixed and known cause of Leukocytoclastic Vasculitis. Although the causes are largely unknown, it seems that the underlying cause of Leukocytoclastic Vasculitis in most of the cases is autoimmune disorder or immunological abnormalities. When there is an inflammatory change in the walls of the blood vessels, it leads to Leukocytoclastic Vasculitis. Mostly it is the inflammation of the small blood vessel that causes Leukocytoclastic Vasculitis, but different types and sizes of the blood vessels have individual impact on the condition.
The immune cells in the blood are the neutrophils. In autoimmune disorders, these neutrophils are destroyed and the debris is called leukocytoclastic cells. This debris of the neutrophil cells is deposited on the walls of the blood vessels and causes this skin disorder. The disorder can be confined only to the skin, but may also affect the internal body organs such as lungs, heart, kidneys, the gastrointestinal tract and even the central nervous system.
The conditions with which Leukocytoclastic Vasculitis is associated are –
- Allergic reaction to food additives
- Allergic reaction to drugs like non-steroid anti-inflammatory drugs (NSAIDs), antibiotics, Orlista, Letrozole
- Inflammatory bowel disease
- Diseases like Sjögren syndrome and lupus erythematosus
- Infections like bacterial endocarditis
- Malignancy like - Wegener's granulomatosis and Hairy cell leukaemia
- Rheumatoid arthritis
- Collagen diseases
- Churg-Strauss syndrome and Polyarteritis nodosa
Prognosis and Epidemiology of Leukocytoclastic Vasculitis:
Leukocytoclastic Vasculitis is a rare disorder that occurs more in adults than in children. It originates from Spain, where the occurrence has been reportedly 3 per 100,000 for hypersensitivity vasculitis. It does occur in children and is referred to as the Henoch-Schönlein purpura. The rate of occurrence in this case is 1.5 per 100,000. In most of the cases, the prognosis of Leukocytoclastic Vasculitis is excellent, if there is no internal involvement causing the condition and mere treatment of the infection or discontinuation of the drug that is causing the disorder helps in treating the condition within weeks to a month.
If Leukocytoclastic Vasculitis is caused by systemic corticosteroids and immunosuppressive agents, then these should be avoided completely. In 10% of the cases, the patients suffering from Leukocytoclastic Vasculitis are seen to suffer from chronic conditions. If the disorder is chronic and recurs time and again, the lesions should be treated with nontoxic modalities. However, chronic conditions are rarer and acute situations can be treated, after diagnosing the specific subtype.
In 60% cases, according to clinical research based study reports, Leukocytoclastic Vasculitis is short-lived and have been cured within three months. However, recurrence has also been common in some cases. The results have been better in those cases, where there was no underlying cause identified as a root of this condition. Painless lesions, normal temperature of the lesions and association with Wegener's granulomatosis, polyarteritis nodosa, Churg-Strauss syndrome are risk factors and can be fatal.
Differential Diagnosis of Leukocytoclastic Vasculitis:
For different Leukocytoclastic Vasculitis, there are different diagnostic procedures that are useful in determining the proper condition and cause of it.
Henoch Schönlein Purpura: In this case, the patient has to undergo IgA deposition on immune-fluorescence to confirm a clinical diagnosis. However, IgA positivity is not a sign of a positive confirmation for this condition as it is associated with many other causes.
Sweet Syndrome: In this case a notable amount of papillary dermal oedema is seen to be present and a diffuse neutrophilic infiltrate is also common. However, in some minority cases fibrinoid necrosis is also diagnosed.
Urticarial Vasculitis: Superficial dermal oedema is a prominent condition associated with these cases, along with an absence of dense inflammatory infiltrate. Hence, in most diagnostic tests, there will be less deposition of neutrophilic component.
The other general diagnostic tests include –
- Skin biopsy: This is a less invasive diagnostic test that involves collecting a sample of the inflamed blood vessel, from under the skin through a minor procedure done with local anaesthesia, where the wound is stitched and removed after a week. However, in some cases, it is not useful as the biopsy can collect sample mostly from the epidermis and superficial dermis, but the affected medium–sized arteries and small blood vessels are located in the deep dermis.
- Organ biopsy: If any other organ in the body is involved in the disorder, ultrasound images of the organ may show the condition and diagnose the cause or extent of the disorder. It sometimes is associated with after-diagnosis bleeding and hence, is done under 48 hours hospital monitoring.
Treatment of Leukocytoclastic Vasculitis:
The treatment of Leukocytoclastic Vasculitis is mostly associated with discontinuation of the drugs that is causing the inflammation of the blood vessels, treating the infection that leads to this condition and avoiding the foods and food additives that have been reasons for Leukocytoclastic Vasculitis. With these measures, Leukocytoclastic Vasculitis is treated within weeks and months. Till then, some managerial steps are recommended to help the patient feel comfortable and so that the condition does not worsen and these are –
- Keeping the legs in an elevated condition
- Not stand for long and rest the legs
- Avoid wearing tight fitting clothes
Those, who are suffering from severe Leukocytoclastic Vasculitis, are often recommended these medicines – corticosteroids, dapsone, colchicines, NSAIDs, hydroxychloroquine etc. If the condition is chronic or is associated with systemic functions, then treatment should be different.
Leukocytoclastic Vasculitis and Pregnancy:
There has been no significant studies and clinical research that can tell about the association and impact of the condition on the child in the womb of the pregnant mother, with Leukocytoclastic Vasculitis. The pregnant mothers, who are diagnosed with Leukocytoclastic Vasculitis, have given birth to babies, without any life threat to them. The mothers have had spontaneous labour and normal vaginal delivery. In cases, where the child also had Leukocytoclastic Vasculitis right after birth in the neonatal stage, the lesions disappeared spontaneously in a few days after birth, with no further complications.
Leukocytoclastic Vasculitis is a rare condition and not much information is available about this disorder. However, it is a fact that Leukocytoclastic Vasculitis is quite well treated with no fatality associated with it under normal circumstances.