What is erythrodermic psoriasis?
Psoriasis is a chronic, inflammatory skin disorder that causes plaques—or thick, scaly accumulations of skin cells. It affects about 2% of the population. Erythrodermic psoriasis is a rare and dangerous type of psoriasis. It affects up to 3% of people with psoriasis.
Erythrodermic psoriasis causes a widespread, red, itchy rash that resembles burned skin. The rash affects most of the body, covering at least 75% of the skin’s surface. The skin can slough off in large sheets, damaging the skin’s protective function. Without an intact skin barrier, fever, hypothermia, and other symptoms may develop. It may also occur in association with pustular psoriasis and cause pus-filled blisters.
A sudden erythrodermic psoriasis flare can be the first sign of psoriasis. Or it can develop slowly in someone who already has plaque psoriasis. This gradual form of the disease tends to be more stable and have a better prognosis. While the exact cause is not well understood, there are known triggers. This includes stress, infection, severe sunburn, and certain medications. People who develop the disease are usually males in their 50s.
Erythrodermic psoriasis is a medical emergency. The damaged skin barrier can lead to severe fluid, protein and electrolyte imbalances. Pneumonia, sepsis, heart failure, and death are also possible. Seek immediate medical care (call 911) for symptoms of erythrodermic psoriasis. Treatment may require hospitalization to restore fluid and electrolyte balance. Antibiotics and biologic therapy may also be necessary. For less severe cases, treatment may include prescription medicines, topical treatments, and wet dressings to protect the remaining layers of skin.
What are the symptoms of erythrodermic psoriasis?
In people with plaque psoriasis, erythrodermic psoriasis tends to develop gradually. Psoriasis symptoms may worsen and not respond to treatment. Symptoms overlap with the plaques, which remain distinct. The other form of erythrodermic psoriasis comes on very quickly. It commonly affects people with psoriatic arthritis and lacks skin plaques.
The main symptom of erythrodermic psoriasis is a red rash covering at least 75% of the skin surface. The rash gives the skin the appearance of being burned. It can also be severely itchy and painful. Other symptoms can include:
Fast heart rate and muscle weakness
Fever, chills, and general ill feeling
Hair loss and nail changes
Joint pain, muscle pain, and enlarged lymph nodes
Skin shedding or sloughing over large areas
Serious symptoms that might indicate a life-threatening condition
Erythrodermic psoriasis can be life threatening. Seek immediate medical care (call 911) if you, or someone you are with, have any symptoms of erythrodermic psoriasis.
What causes erythrodermic psoriasis?
Doctors do not fully understand what causes erythrodermic psoriasis. Research suggests it may be related to an imbalance in immune cells called helper T cells. These cells modulate the body’s immune response by activating other parts of the immune system.
Most people who develop the disease already have psoriasis. And in more than half the cases, there is an identifiable trigger for erythrodermic psoriasis.
Known triggers include:
Medications, including antimalarials, lithium, and trimethoprim/sulfamethoxazole (Bactrim, Septra)
Rebound phenomenon after abruptly stopping systemic psoriasis treatment, including steroids
Severe sunburn or skin injury or trauma, including allergic drug-induced rash (Koebner phenomenon)
What are the risk factors for erythrodermic psoriasis?
Like the cause of erythrodermic psoriasis, not much is known about its risk factors. Small population health studies have found it happens more commonly in males. In fact, men appear to be three times as likely to develop the disease compared to women. It also occurs at an average age of 53.7 years.
Reducing your risk of erythrodermic psoriasis
Because the cause is not well understood, it may not be possible to prevent erythrodermic psoriasis. You may be able to lower your risk of developing it by avoiding known triggers. If you have psoriasis or psoriatic arthritis, talk with your doctor about your risk of erythrodermic psoriasis. Ask about ways you can protect yourself and what to watch for.
How is erythrodermic psoriasis treated?
Treatment of erythrodermic psoriasis often requires hospitalization for severe cases. The first step is restoring fluid and electrolyte balance and stabilizing the body and vital organs like the heart. Then, doctors can treat the disease itself, usually with a combination of treatments.
Treatments can include:
Antibiotics to prevent infection
Biologics, including adalimumab (Humira), etanercept (Enbrel), golimumab (Simponi), infliximab (Remicade), ixekizumab (Taltz), and ustekinumab (Stelara)
Systemic medications, including acitretin (Soriatane), cyclosporin (Gengraf, Neoral, Sandimmune), methotrexate (Rheumatrex, Trexall), and mycophenolate mofetil (Cellcept)
Topical treatments, including wet dressings, oatmeal baths, moisturizers, and corticosteroid ointments
Combination therapy is usually the most effective. Doctors may also prescribe pain medicines, anti-itch medicines, and sleep aids.
What are the potential complications of erythrodermic psoriasis?
Erythrodermic psoriasis is potentially life threatening due to complications. The extreme damage to the skin barrier can result in severe fluid, protein and electrolyte imbalances. Common causes of death include heart failure, pneumonia and sepsis. The risk of death is higher in elderly people who develop complications.
The two subtypes of erythrodermic psoriasis have slightly different outlooks. Plaque psoriasis patients who develop erythrodermic psoriasis gradually tend to have a favorable prognosis. The disease course for these people is relatively stable. When erythrodermic psoriasis develops rapidly in the absence of plaques, the prognosis is not as good. The disease course is more unstable, progresses rapidly, and relapses.