Symptoms of chronic hives usually only affect the skin. However, some people report other symptoms, such as fatigue and headache. For other people, hives are a sign of another condition that affects the whole body. Conditions that affect the entire body are called systemic conditions.
Systemic conditions that include hives are not common. They have certain features that distinguish them from chronic hives. Hives caused by systemic conditions are often difficult to treat. Because of this, hives typically become chronic (long-term) for people with these conditions.
Urticarial vasculitis
Urticarial vasculitis (UV) is a rare condition where inflammation of blood vessels restricts blood flow. Between 5 and 20 percent of people with chronic hives have UV. Most people with UV are women over age 30.1,2
UV mostly affects small vessels of the skin. This causes hives that itch and burn. But other organs and tissues may also be affected. UV often has no known cause but may involve an autoimmune reaction. It has also been linked to drug reactions, infections, and cancer.3
Hives caused by UV differ from chronic hives. Doctors suspect UV when individual hives:2
- Are painful
- Last longer than 24 to 36 hours
- Cause skin discoloration
- Are accompanied by fever, arthritis, joint pain, or weight changes
When UV only affects the skin (cutaneous UV), antihistamines and anti-inflammatory drugs relieve symptoms. When it affects multiple organs (systemic UV), steroids and other drugs may be needed.1
Mastocytosis
Mastocytosis is a rare condition where too many mast cells build up in your body. Mast cells are white blood cells that help your immune system protect you from disease. About 1 in 10,000 people have mastocytosis.4,5
When mast cells build up in only the skin, it is called cutaneous mastocytosis. When they build up in multiple organs, it is called systemic mastocytosis. Adults tend to have systemic forms, while children tend to have cutaneous forms.6
When triggered, mast cells release chemicals that cause symptoms similar to an allergic reaction. This can be severe and lead to anaphylaxis, a severe, life-threatening reaction. Symptoms vary in intensity and depend on which organs are affected. Other common symptoms include:4,5
- Flushing, itching, or hives
- Stomach pain, diarrhea, nausea, or vomiting
- Bleeding disorders
- Bone and muscle pain
- Enlarged liver or spleen
Doctors diagnose mastocytosis using your medical history, blood and urine tests, and skin biopsies. They may test for genetic mutations linked to mastocytosis. Treatment includes avoiding triggers and treatments such as:4
- Antihistamines
- Cromolyn sodium
- Ketotifen
- Glucocorticoids
- Omalizumab (Xolair®)
- Imatinib mesylate (Gleevec®), midostaurin (Rydapt®), or avapritinib (Ayvakit™) in special cases
- Stem cell transplant for people with mast cell leukemia
Autoimmune conditions
Autoimmune conditions happen when your immune system mistakenly attacks healthy cells. Hives may be a sign of certain autoimmune conditions. These conditions include:2
- Systemic lupus erythematosus (SLE)
- Rheumatoid arthritis
- Sjögren syndrome
- Celiac disease
- Thyroid diseases, such as Graves’ disease and Hashimoto’s thyroiditis
Your doctor may perform blood and skin tests to diagnose autoimmune hives. Treatment depends on the severity of symptoms. Doctors will usually suggest antihistamines first. They can then suggest other drugs if antihistamines do not control symptoms.2
Cutaneous small vessel vasculitis
Cutaneous small vessel vasculitis (CSVV) is a very rare disease characterized by inflammation of blood vessels. This restricts blood flow and damages organs, usually the skin. Between 40 and 50 people out of every 1 million adults are diagnosed with CSVV every year.2,7
Common symptoms of CSVV include:8
- Purple or reddish rash over legs, buttocks, or torso
- Hives, blisters, and open sores or wounds
Doctors diagnose CSVV using your medical history, blood and urine tests, and skin biopsies. An allergic drug reaction or infection typically triggers CSVV. Symptoms usually go away once the drug is stopped or infection is treated. Most cases go away within weeks to months. Some cases can be chronic and need treatment.8
Cancer
The link between chronic hives and cancer is unclear. Experts do not recommend testing for cancer unless there are other signs of the condition.2
There is some evidence that people with chronic hives have a higher risk of cancer. A few case studies have found cancer as an underlying cause of chronic hives. In these cases, treating cancer also relieved hives.9-11