What is Erythrodermic Psoriasis?
Erythrodermic Psoriasis is an extremely aggressive but quite rare form of Psoriasis vulgaris. Approximately 2% of all psoriatic patients tend to develop this form of psoriasis. The primary characteristic feature of Erythrodermic Psoriasis is the presence of an inflammatory peeling rash covering about 75% of the entire body mass. These rashes may at times itch or burn and it tends to spread very aggressively. There are a variety of complications associated with Erythrodermic Psoriasis some of which are extremely serious and even life-threatening.[1,2,3]
The pathophysiology of Erythrodermic Psoriasis is not well understood but the morbidity and the mortality associated with this condition suggests that efforts should be made to better understand the pathophysiology of Erythrodermic Psoriasis to formulate a better treatment option. As of now, Erythrodermic Psoriasis requires a multidisciplinary approach of treatment.[1,2,3]
With regard to drugs, cyclosporine is the frontline medications that have been suggested by the US National Psoriasis Foundation for the treatment of Erythrodermic Psoriasis, especially in unstable cases.[1,2,3]
What Causes Erythrodermic Psoriasis?
As stated, the causes of Erythrodermic Psoriasis are not clearly understood but some researchers are of the opinion that an overactive immune system may be a potential cause. However, it is still not clear why does Erythrodermic Psoriasis develop. The researchers suggest that in people with psoriasis there is an overproduction of T-cells which is a form of white blood cells that fights off bacteria and viruses in the body. These T-cells then attack the healthy skin cells which lead to an overproduction of skin cells seen in the form of rashes along with other symptoms as seen in people with Erythrodermic Psoriasis. This condition aside from affecting the skin can also affect joints, nails, and other parts of the body as well.
The cause of Erythrodermic Psoriasis may not be clear but there are certain factors that can trigger a flare-up of Erythrodermic Psoriasis. These factors include stopping psoriasis medications abruptly or overuse of corticosteroids for treating psoriasis. Certain infections at times may also cause a flare-up of Erythrodermic Psoriasis. Alcohol abuse, allergic reactions, and emotional stress are also some of the factors that can trigger a flare of the symptoms of Erythrodermic Psoriasis.
What are the Symptoms of Erythrodermic Psoriasis?
As Erythrodermic Psoriasis is an aggressive and rapidly progressive condition, the symptoms of this condition are quite intense and severe. In some people, especially those who have had a recent diagnosis of psoriasis, the symptoms are acute and appear all of a sudden. In other people, they tend to come on more gradually over a period of time.
The primary symptoms of Erythrodermic Psoriasis include erythematous skin covering about 75% of the entire body surface area. The rashes seen with Erythrodermic Psoriasis then starts to peel off but instead of in scales or flakes as seen with other forms of psoriasis the shedding is more intense and in sheets. There are widespread pustules and blisters.
The skin looks burnt with a brownish black tinge to the affected area. The person will have an intense sense of itching around the body. There will be pain almost all over the body. The body temperature then starts to fluctuate and the person starts to have an increased heart rate. Since Erythrodermic Psoriasis affects even other body systems there will be additional symptoms experienced to include swelling around the ankles, pain in the joints, fever and chills.
How is Erythrodermic Psoriasis Treated?
Erythrodermic Psoriasis is a condition that is at times quite challenging for physicians to treat especially if the disease has advanced to the extent that complications develop. However, the best treatment strategy for Erythrodermic Psoriasis includes use of both drug therapy as well as topical agents. The treatments will change depending on the severity of the disease and associated complications or other health conditions, if any.
The topical agents that are used for treatment of Erythrodermic Psoriasis include moisturizers and steroid creams that can be applied over the affected area. Oatmeal baths have also been found to be quite effective in treatment of Erythrodermic Psoriasis. In case if there are complications due to Erythrodermic Psoriasis, then IV fluids and electrolytes will be administered to stabilize the patient. The patient will then be given systemic medications that will cover the entire body to treat the complications of Erythrodermic Psoriasis.
With regard to medications, Cyclosporine is the most preferred drug as suggested by the US National Psoriasis Foundation. This is because researchers believe that it is extremely fast reacting and calms down a flare very quickly. Methotrexate is also quite effective in treatment of Erythrodermic Psoriasis but this medication acts quite slowly when compared to cyclosporine. Once stabilized, the patient will then be switched to a combination therapy or a medication called Enbrel which is quite effective for treating Erythrodermic Psoriasis.
Biologic Drugs are also used quite effectively in the treatment of Erythrodermic Psoriasis. The most preferred drug of choice is Humira. These drugs are tumor necrosis factor alpha inhibitors and are beneficial for treatment of various forms of psoriasis including Erythrodermic Psoriasis. In case the patient develops an infection due to Erythrodermic Psoriasis then that will also need to be treated with a course of antibiotics.
In most cases, Erythrodermic Psoriasis requires treatment in an inpatient setting, especially if fluids are lost due to complications from the condition. However, if the patient is not losing fluids and is stable then outpatient treatment may be good enough. It has been seen that people with HIV are at most risk for developing psoriasis and Erythrodermic Psoriasis. In such patients, this condition is very hard to treat. These patients will be given antiretrovirals and ultraviolet phototherapy. Aside from this, the patient will also be given acitretin which has shown some effective ness in treating Erythrodermic Psoriasis in HIV patients.
Can Erythrodermic Psoriasis Be Prevented?
The best way to prevent a flare of Erythrodermic Psoriasis is to avoid triggers that cause it. It is important for people with a known diagnosis of psoriasis to follow the guidelines recommended by their physician. People are at risk for Erythrodermic Psoriasis should avoid any form of stress. They should also be weary of cold weather and windy conditions as it may lead to a flare.
It is also recommended to drink alcohol only in moderation and not to overdo it. Smoking is something that the person should completely abstain from. In some cases, however, the onset of Erythrodermic Psoriasis is so acute that it is impossible to take any preventive measures.
What Is The Prognosis Of Erythrodermic Psoriasis?
In majority of the cases of Erythrodermic Psoriasis, the prognosis is quite good with timely treatments. There is some research data that shows Erythrodermic Psoriasis may have led to life-threatening complications and fatalities in about 5-60% of cases and the most common complications seen with Erythrodermic Psoriasis are septicemia, heart failure and pneumonia. However, with newer treatments and better understanding of the condition, the mortality rate is significantly declining in cases of Erythrodermic Psoriasis.