What are the other Names for this Condition? (Also known as/Synonyms)

• Cutaneous Leukocytoclastic Vasculitis
• Hypersensitivity Vasculitis (HV)
• Small Vessel Vasculitis of Skin

What is Cutaneous Small-Vessel Vasculitis? (Definition/Background Information)

• Cutaneous Small-Vessel Vasculitis (CSVV) form a group of conditions affecting the blood vessels, which are mostly observed in adults. In this condition, inflammation of the small blood vessels of skin is observed. It can cause damage to various body organs depending on the region affected, due to decreased blood and oxygen supply to the region
• Cutaneous Small-Vessel Vasculitis can cause small or large skin rashes or varying types that present burning or itching sensation and pain. Systemic involvement is seen in some individuals involving the gastrointestinal tract, kidney, and muscles and joints
• The exact cause of the condition is not understood, but it is reportedly immune-mediated. Several autoimmune diseases, viral infections, use of certain medications, and rarely, malignancies are associated with the development of Cutaneous Small-Vessel Vasculitis
• A wide variety of tests and exams may be performed including blood and urine tests, radiological studies, and tissue biopsies, as needed, to understand the involvement of various body organs. However, in many cases, Cutaneous Small-Vessel Vasculitis is diagnosed by excluding other conditions presenting similar signs and symptoms
• An early diagnosis and appropriate treatment of Cutaneous Small-Vessel Vasculitis can lead to a good prognosis. The overall prognosis depends on the severity of the condition, the organs affected, and one’s response to therapy. Individuals with mild skin symptoms and minimal systemic involvement generally have a good prognosis

Who gets Cutaneous Small-Vessel Vasculitis? (Age and Sex Distribution)

• Cutaneous Small-Vessel Vasculitis (CSVV) is reportedly amongst the most common form of vasculitis
• Even though it can affect individuals of a wide age range, it is predominantly observed in older children and adults. CSVV is generally not observed in children below the age of 16 years
• The condition affects both males and females, although some medical literature inform that it is slightly more common in males
• It is observed worldwide and can affect people of all racial and ethnic backgrounds. The condition is more prevalent among Caucasians
• In adults, in USA and UK, the prevalence rate of CSVV is between 10-55 cases per million population

What are the Risk Factors for Cutaneous Small-Vessel Vasculitis? (Predisposing Factors)

The following risk factors have been reported for Cutaneous Small-Vessel Vasculitis (CSVV):

• In 10-15% of the cases, use of certain medications including antibiotics, such as penicillin, non-steroidal anti-inflammatory drugs (NSAIDs), diuretics, and blood pressure medications
• In 15-20% of the cases, infections that affect the upper respiratory tract, longstanding bacterial infections caused by staphylococcus and streptococcus, viral infections including hepatitis B and C, and HIV infection
• In 15-20% of the cases, the involvement of autoimmune disorders, such as Sjögren's syndrome, systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and inflammatory bowel disease (IBD), is noted
• Use of certain food additives; food allergies
• In rare cases, blood-related malignancies
• CSVV is sometimes seen along-with other large blood vessel vasculitis

In nearly 35-55% of the cases, the risk factors are not clearly evident.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Cutaneous Small-Vessel Vasculitis? (Etiology)

• The exact cause of development of Cutaneous Small-Vessel Vasculitis is not known; researchers consider it an immune-mediated disorder
• It may develop as a hypersensitivity reaction to certain stimuli, such as an inflammatory conditions, infection, or medication use, triggering an abnormal immune reaction in the body

Small-Vessel Vasculitis of Skin is not transmitted from one individual to another; the condition is non-contagious.

What are the Signs and Symptoms of Cutaneous Small-Vessel Vasculitis?

The severity of signs and symptoms of Cutaneous Small-Vessel Vasculitis may vary from one individual to another. In some, it may be mild, resulting in a delayed diagnosis of the condition. In other individuals, it may be severe. The signs and symptoms depend on the organs affected and the presence of an underlying cause/trigger, if any.

The general signs and symptoms may include:

• Mild fever
• Headaches
• Bloodshot eyes
• Fatigue
• Loss of weight
• Weakness and malaise
• Pain that may affect the chest, back, or abdomen
• Shortness of breath
• Discomfort

Since the skin is always involved, cutaneous signs and symptoms are initially noted. These may include: 

• Skin eruptions that begin as small, medium, or large-sized rashes of varying types; this varies from one individual to another
• Presence of raised and tender macules or papules may be noted, which are purplish in color
• In some cases, the rashes may be reddish and flat
• The red/purple discoloration occurs from blood leaking into the skin layers due to vasculitis
• On touch or when applying pressure, the lesions do not decrease in color intensity (i.e., turn lighter in tone). This feature is termed “non-blanching”
• The rashes present itchiness, burning sensation, and pain in some individuals. However, in many cases, no pain or itching sensation is felt
• Later, in a few individuals, pus-filled blisters are seen, resembling pimples
• The blisters may break and bleed and cause ulceration
• Most of the skin rashes are present on the lower part of the body, mostly involving the buttocks and upper legs. Sometimes, the skin rashes are noted above the waist too - on the chest, back, and arms
• On the upper body, rashes may be seen in localized patches, which may then cover large areas as the condition progresses
• The skin rashes may develop quickly within 1-2 days and initially cover large skin surfaces on the body. Then, over the next few weeks, the rashes tend to develop slower

Following the skin symptoms, systemic symptoms start that tend to mostly involve the digestive system, kidney, and joints. It is important to note that the combination of signs and symptoms may vary from individual to individual.

Signs and symptoms related to the gastrointestinal (GI) system:

• Nausea and vomiting
• Fatigue and weakness
• Difficulty in swallowing
• Hiccups
• Hoarse voice
• Indigestion; feeling of fullness after eating a small amount of food
• Heartburn or acid reflux (gastroesophageal reflux disease or GERD)
• Loss of appetite
• Stomach or abdominal pain and discomfort
• Abdominal bloating and distension
• Irregular bowel movements
• Passing gas; belching or flatus
• Constipation or diarrhea
• Blood or mucus in stool
• Unintended loss of weight

Signs and symptoms related to the kidney and urinary bladder (genitourinary system):

• Unexplained fever
• Night sweats
• Difficulty in urinating; presence of burning sensation
• Pain while urination, frequent urge to urinate
• Presence of blood in the urine
• Feeling of obstruction while urinating
• Inflammation of the bladder (cystitis)
• Sharp pain in the back or lower abdomen that radiates to the groin
• Chronic pain in the pelvis
• Fluid accumulation in the lower legs (pedal edema)
• Increased blood pressure due to kidney problems

Signs and symptoms related to the muscles and joints (musculoskeletal system):

• Inflammation of the muscles and joints
• Muscle and joint pain, involving one or more joints; mostly the knees and ankles are affected, and sometimes elbows
• Tenderness of the muscles
• Muscle stiffness and spasms
• Swollen joints
• Joint stiffness
• Muscle weakness, especially involving the upper or lower limbs
• Loss of muscle mass
• Muscle contracture
• Difficulty climbing stairs and walking
• Difficulty with other motor skills, including jumping, hopping, and running
• Difficulty in bending or stretching one’s back
• Limited range of motion; decreased joint function
• The condition may involve any joint in the body such as the knee, elbow, shoulder, hip, etc.

The signs and symptoms of the underlying or associated condition, if any, may be additionally noted.

How is Cutaneous Small-Vessel Vasculitis Diagnosed?

A diagnosis of Cutaneous Small-Vessel Vasculitis (CSVV) will usually depend on the system or organ of the body that is affected and the underlying trigger, if any. A skin biopsy is an important consideration towards establishing its diagnosis.

In a vast majority of cases, CSVV is a diagnosis of exclusion. It is also necessary to rule out IgA vasculitis (or Henoch-Schönlein purpura), which is another form of small-sized blood vessel vasculitis.

The diagnosis may involve the following tests and examinations:

• Complete physical examination and a thorough medical history
• Assessment of the presenting signs and symptoms

Tests and procedures based on the different body part/organ or system that is affected may include any of the following.

• Blood and urine tests related to overall inflammatory and autoimmune activity:
  • Complete blood count (CBC) with differential: This measures the red blood cell count and hemoglobin levels in blood
  • Erythrocyte sedimentation rate (ESR): ESR is a blood test that can help the healthcare provider identify any inflammatory activity within the body
  • C-reactive protein (CRP) test: This blood test measures the amount of a protein, called C-reactive protein, in blood. It also provides information on the inflammation condition of the body
  • Anti-nuclear antibody (ANA) test: ANA test identifies the antinuclear antibodies within the blood
  • Basic metabolic panel tests
  • Anti-streptolysin O (ASO) titer test, if a streptococcal infection is the suspected trigger
  • Specific antibodies test
  • Protein electrophoresis and immunofixation electrophoresis tests
  • Complement blood test
  • Cryoglobulin blood test
  • HIV antibody blood test
  • Urine tests for protein and presence of blood
  • Urinalysis

Tests and procedures related to the skin and underlying tissue (integumentary system):

• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Punch biopsy of skin for immunofluorescence studies, especially direct immunofluorescence studies. The immunofluorescence deposits can be performed with fluorescein-labeled antibodies against IgG, IgM, IgA, and C3
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Tests and procedures related to the gastrointestinal (GI) system:

• Liver function tests
• Viral hepatitis panel
• Imaging studies that may include:
  • X-ray of chest or abdomen
  • Ultrasound studies of the chest or abdomen
  • CT scan of chest or abdomen
  • MRI studies of abdominal organs
  • Gastrointestinal angiographic studies
  • Magnetic resonance cholangiopancreatography (MRCP): It is a special MRI exam of the hepatobiliary and pancreatic systems
• Upper GI endoscopy: An endoscopic procedure is performed using an instrument called an endoscope, which consists of a thin tube and a camera. Using this technique, the radiologist can have a thorough examination of the insides of the upper GI tract
• Colonoscopy: A colonoscopy is a test that allows the physician to look at the inner lining of the colon and rectum. A typical colonoscopy involves using a thin, flexible tube (called a colonoscope), with an attached video camera, to view the colon and rectum
• Exploratory laparoscopy (diagnostic laparoscopy) may be required, if gastrointestinal symptoms are present. In this procedure, the abdomen is examined using a minimally-invasive technique, and a tissue biopsy and tissue for culture obtained
• Tissue biopsy: A biopsy of the affected region is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Tests and procedures related to the kidney and urinary bladder (genitourinary system):

• Diagnostic tests may include the following:
  • Urinalysis
  • Blood tests including blood urea nitrogen (BUN) and creatinine levels
  • Kidney function tests
• Radiological studies that may include:
  • Abdominal X-ray
  • CT scan of the abdomen
  • Ultrasound scan of the kidneys
  • MRI scan of the kidney
  • Intravenous pyelogram (IVP): A dye is injected into the blood vessels and the image of kidney structure is obtained
  • Angiography of the abdominal region
• Ureteroscopy: Endoscopic study of the upper urinary tract using an endoscope inserted through the urethra
• Cystoscopy: During a cystoscopy, a narrow tube called a cystoscope is inserted to look directly into the bladder. A local anesthetic is usually administered, in order to make the examination more comfortable

Invasive diagnostic procedures such as:

• Laparoscopy: A special device is inserted through a small hole into the abdomen, to visually examine it. If necessary, a tissue sample is obtained for further analysis. Exploration of the abdomen using a laparoscope is called ‘exploratory laparoscopy’
• Laparotomy: The abdomen is opened through an incision for examination, and if required, a biopsy sample obtained. Exploration of the abdomen using laparotomy procedure is called ‘exploratory laparotomy’

Although the above modalities can be used to make the initial diagnosis, a tissue biopsy may be required to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways, and they include:

• Fine needle aspiration (FNA) biopsy: An FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the affected region. Hence, an FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
• Core biopsy of kidney
• Open biopsy of kidney

A tissue biopsy is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis.

• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation.
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely electron microscopic studies to assist in the diagnosis

Tests and procedures related to the muscles and joints (musculoskeletal system):

• Laboratory tests, which may include:
  • Creatine kinase test: It is used to measure the amount of creatine kinase in blood. The enzyme creatine kinase is released by the muscle and into the bloodstream, when any deterioration has occurred
  • Rheumatoid factor blood test
  • Cyclic citrullinated peptide antibody blood test (anti-CCP)
• Electromyogram (EMG): Electromyographic studies to determine the region of muscle that is affected
• Radiological imaging studies of the affected region such as:
  • X-ray studies
  • CT scans
  • MRI scans
  • Ultrasound imaging: An ultrasound imaging equipment uses high-frequency sound waves to generate a more detailed image of affected region
  • Musculoskeletal angiographic studies
• Fine needle aspiration (FNA) of joint fluid: Occasionally, fluid may accumulate around a joint, which results in pain and disability. Analysis of this joint will provide clues regarding the cause of joint fluid accumulation
• Muscle biopsy: A biopsy of the affected muscle tissue is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Other diagnostic examinations may be performed based on the affected region/part of the body and the nature of the underlying condition.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Cutaneous Small-Vessel Vasculitis?

The complications of Cutaneous Small-Vessel Vasculitis that develop depend on the underlying condition, the organs affected, severity of the signs and symptoms, and one’s response to treatment.

Complications associated with the skin and underlying tissue (integumentary system) may include:

• Emotional stress due to cosmetic concerns
• Bleeding and ulceration may lead to secondary bacterial or fungal infections
• Severe pain and itchiness of skin leading to acute discomfort and irritation, including sleep disruption
• Permanent scarring, especially when severe ulceration takes place

Complications associated with the gastrointestinal (GI) system may include:

• Inability to eat
• Weight loss and malnutrition
• GI tract bleeding
• Perforation of the GI tract
• Stricture formation of the food pipe

Complications associated with the kidney and urinary bladder (genitourinary system) may include:

• Severe pain in the back or lower part of the abdomen
• Obstruction of the urinary bladder
• Blockage of the urinary tract may lead to kidney damage
• Hydronephrosis of kidneys (swollen kidneys)
• Erectile dysfunction
• Urinary incontinence (involuntary dripping or leakage of urine)
• Severe cases may result in kidney damage and kidney failure, but it is rarely observed

Complications associated with the muscles and joints (musculoskeletal system) may include:

• Chronic pain and weakness of the joint affecting the quality of life
• Formation of scar tissue in the muscle and joint tissue
• Immobilization due to loss of movement
• Permanent disability

Treatment complications: Some complications are known to take place with treatment; in some cases, due to treatment also. Immunosuppressive therapy that is used to treat Small-Vessel Vasculitis of Skin may lead to certain complications that include:

• Low blood pressure
• Hyperkalemia or high levels of potassium in blood
• Tremors
• Diabetes mellitus
• Osteoporosis causing decreased bone density
• Decreased muscle function
• Long-term kidney dysfunction
• Elevated risk for malignancy
• Decrease in body immunity to fight infections

How is Cutaneous Small-Vessel Vasculitis Treated?

The treatment of Cutaneous Small-Vessel Vasculitis (CSVV) depends upon a variety of factors including:

• Severity of the signs and symptoms
• Regions of the body affected and the level of functioning of the affected organs
• Overall health status of the individual and his/her age
• Underlying condition triggering CSVV

The main treatment of Cutaneous Small-Vessel Vasculitis includes treating the underlying cause. If a clearly identifiable cause is not known, then the primary role of treatment is to reduce inflammation. Often, a multidisciplinary team of specialists that include general practitioners, rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists, dermatologists, ophthalmologists, among others, may be involved in the treatment.

The treatment measures may include:

• Nearly 50% of the cases arise as a result of hypersensitivity to an offending stimulus, hence removal of the stimulus is often sufficient treatment for the illness. This may include:
  • Discontinuing the medication responsible for CSVV
  • For infections, use of antiviral agents and antibiotics
  • For food allergies, avoid consumption of the causative foods; use of anti-allergic medication and antihistamines
  • Use of non-steroidal anti-inflammatory medications for autoimmune disorders
• Symptomatic treatment for skin rashes may include the following:
  • Since the lower body and legs are mostly affected, avoid standing for long periods (helps with joint pain too)
  • Elevation of legs and use of compressive stockings are recommended
  • Use of topical steroids for itching and pain
  • Oral corticosteroid therapy
  • Wound care for ulceration
  • If there is no relief within a month’s time, then more intensive treatment with antibiotics, such as dapsone, and oral colchicine is considered
  • If the lesions are more persistent, then a short course of high-dose therapy with immunosuppressive medications (methotrexate and azathioprine) may be needed
• A combination of medications may be used for controlling the symptoms, especially when systemic involvement (GI tract and kidneys) is noted
• Corticosteroid therapy, for severe and/or long-term CSVV
• Immunosuppressive medications may be necessary the condition is resistant to corticosteroids
• Biologic agents using specific monoclonal antibodies to reduce inflammation
• In cases with severe symptoms, or when the affected individuals do not respond to the above-mentioned treatments, plasma exchange (plasmapheresis) or intravenous immunoglobulin (IVIG) therapy may be considered

The treatment also depends on whether there are recurring episodes of the condition. The goal of the treatment is to control the inflammation in order to achieve remission (a state where no signs and symptoms are noted) and maintenance therapy to prevent episodes of relapses.

How can Cutaneous Small-Vessel Vasculitis be Prevented?

Currently, there are no methods available to prevent Cutaneous Small-Vessel Vasculitis (CSVV). An early diagnosis and prompt treatment can help decrease the burden of the condition.

• Undertaking early and appropriate treatment of the underlying infection or autoimmune disorder (potential risk factors) may help in lowering one’s risk for CSVV and is an important consideration
• Drugs that cause the condition may be discontinued or alternative medication prescribed
• Avoidance of food items that one is potentially allergic to (which acts as a trigger for CSVV)
• Treating and managing the underlying condition that triggered the initial episode helps in preventing subsequent episodes
• Maintaining a healthy lifestyle with good eating habits, adequate physical activities, and avoidance of smoking or drinking is beneficial
• Patients are required to be vigilant during the remission period and report any new symptoms promptly to the healthcare provider

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to the possibility of recurrence/relapse. Often several years of active vigilance is necessary.

What is the Prognosis of Cutaneous Small-Vessel Vasculitis? (Outcomes/Resolutions)

• The prognosis of Cutaneous Small-Vessel Vasculitis depends upon a variety of factors including the:
  • Severity of the condition; degree of damage to the vital organs and body systems
  • Age and overall health of the individual
  • Underlying triggering factor
  • One’s response to treatment: Individuals who respond to treatment usually have a better prognosis than those who do not respond well to treatment
  • Whether it is an initial episode or a recurring episode
• The overall prognosis for mild cases of Cutaneous Small-Vessel Vasculitis is good, especially if it is mostly confined to the skin. Most skin symptoms resolve within 3 weeks following treatment, even though scarring may be present (seen as flat spots with dark pigmentation)
• In case of CSVV that arises due to an underlying infection or autoimmune disorder, the prognosis depends on the severity of the underlying condition and its resolution following treatment
• In case of extensive CSVV with severe systemic symptoms, the skin rashes can persist for a long time, and there is a higher risk for complications. Also, the condition can become chronic and relapsing, which can affect the overall quality of life

Even with treatment, Cutaneous Small-Vessel Vasculitis can relapse. If blood in cough or blood in urine is observed, then it is extremely important to contact the healthcare provider right away.

Additional and Relevant Useful Information for Cutaneous Small-Vessel Vasculitis:

The following article link will help you understand other Vein & Vascular Health:

https://www.dovemed.com/health-topics/vein-and-vascular-center/