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

• EP (Erythrodermic Psoriasis)

What is Erythrodermic Psoriasis? (Definition/Background Information)

• Erythrodermic Psoriasis (EP) is an uncommon subtype of psoriasis, which is a common chronic inflammatory skin disease that develops from a usually unknown genetic defect. The condition causes the epidermis to grow abnormally, resulting in the formation of skin lesions. Psoriasis can run in families but is not a contagious condition
• Erythrodermic Psoriasis is described as  a severe  form of psoriasis with involvement of nearly 75-80% of the body skin. Apart from severe skin symptoms that include large areas of redness and inflammation, the condition may present with fever and chills, tiredness, and widespread inflammation
• Some of the known triggers of Erythrodermic Psoriasis include alcohol, infections or other severe illnesses,  medications including systemic steroids, sunburn, and stress
• In some cases, Erythrodermic Psoriasis may have a sudden onset and pursue a severe course with systemic involvement of the body, including heart failure that can  be fatal. Other complications include dehydration, hypothermia (low body temperatures), and pedal edema (swollen lower legs)
• It can be difficult to treat this condition; conventional topical applications and most oral medications used for psoriasis are not known to be generally effective. The prognosis of Erythrodermic Psoriasis depends upon its response to treatment

Who gets Erythrodermic Psoriasis? (Age and Sex Distribution)

• Only between 1% to 2.5% of psoriasis cases are reportedly Erythrodermic Psoriasis
• More cases are reported in adults than in children
• Both men and women are affected. No specific gender preference is reported
• The condition affects all races and ethnic groups. Fair-skinned Caucasians have a generally higher prevalence rate, while dark-skinned individuals are affected to a lesser extent

What are the Risk Factors for Erythrodermic Psoriasis? (Predisposing Factors)

The following are the risk factors for Erythrodermic Psoriasis:

• Individuals with prolonged history of psoriasis vulgaris have an increased risk for Erythrodermic Psoriasis
• Psoriasis, in general, has a strong association with genetic factors. Studies have shown a correlation between psoriasis and individuals with certain types of human leukocyte antigens (HLAs). HLAs are gene complexes that form a part of the immune system
• Certain studies have also demonstrated that individuals with close relatives already affected by the condition are at a higher risk for the disease in comparison to the rest of the population. Hence, having a family member with psoriasis is a significant risk factor
• Caucasians, who are fair-skinned, are more susceptible to psoriasis
• HIV-infected patients are at an increased risk
• Use of long-term or high-dose systemic steroids in psoriasis patients
• Alcohol use, sunburn, stress, or severe illness 
• Individuals who are smokers, obese, diabetic, and with heart disease are at an increased risk for developing severe forms of the condition
• The symptoms may get worse during cold season - during fall and winter
• Efalizumab, voluntarily withdrawn from use in 2009, lead to Erythrodermic Psoriasis on discontinuation in some individuals

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 Erythrodermic Psoriasis? (Etiology)

Erythrodermic Psoriasis is a manifestation of severe psoriasis that develops from an abnormal proliferation of epidermal skin cells called keratinocytes. The exact reason behind this development is unknown, but certain factors are contributory:

• There seems to be an association of the skin disorder with a dysfunctional immune system, which results in abnormally rapid growth of epidermal cells
• It is believed that the condition may be affected by certain environmental factors such as low sun and cold temperatures
• It is reported that worsening of psoriasis can result in Erythrodermic Psoriasis

What are the Signs and Symptoms of Erythrodermic Psoriasis?

The onset of signs and symptoms of Erythrodermic Psoriasis can be sudden or gradual when it develops over many days and weeks. Occasionally, the condition can also develop slowly following a decline in pre-existing psoriasis vulgaris.

The signs and symptoms of Erythrodermic Psoriasis may include:

• Large areas of the body (usually over 75% of the body surface) present contiguous red patches, termed erythroderma
• The whole trunk and upper limbs may present red skin
• The affected skin surface is deep red and dry; hair loss may be noted
• Involvement of the palms of the hands, soles of the feet, nails, and scalp may be noted
• Itchiness and irritability
• Other signs and symptoms (not involving skin) include:
  • Fever and chills
  • Fatigue
  • Lethargy
• Erythrodermic Psoriasis has many findings shared with Generalized Pustular Psoriasis, a distinct form of Psoriasis

How is Erythrodermic Psoriasis Diagnosed?

Erythrodermic Psoriasis is easily recognized, and a healthcare provider may diagnose the condition on the basis of a thorough physical examination. A diagnosis of Erythrodermic Psoriasis would involve the following tests and exams:

• Complete physical examination and comprehensive medical history (including evaluation of family history of the condition)
• Bacterial skin cultures
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• X-rays, CT, and MRI scan of the affected joint can help reveal the extent of damage due to inflammation in individuals with psoriatic arthritis
• Skin biopsy: A skin tissue biopsy of the lesions may be 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 microscope findings, the pathologist arrives at a definitive diagnosis

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 Erythrodermic Psoriasis?

Erythrodermic Psoriasis can cause a variety of complications, and these include:

• The lesions may develop bacterial or fungal infections; the condition is typically associated with infections by the bacterium Staphylococcus aureus
• Arthritis, causing destruction of the joint
• Dehydration: There may be loss of body fluids through the skin, resulting in fluid and electrolyte imbalance
• Hypoalbuminemia, from protein and iron loss from skin sloughing
• Pedal edema, which is the swelling of lower limbs from fluid loss into the lower leg and other dependent tissues
• Hypothermia or low body temperatures, which can be a medical emergency
• Heart failure
• Due to its chronic nature, some individuals may undergo severe emotional issues and psychological distress that may require counseling
• Severely affected quality of personal life and academic/professional life
• Medication side effects that may be severe in some

Severe cases of Erythrodermic Psoriasis can result in death.

How is Erythrodermic Psoriasis Treated?

The treatment of Erythrodermic Psoriasis is generally difficult, and there are no established guidelines for treating the condition. It requires immediate and emergency treatment in some cases.

It is very important to avoid certain medications and treatment measures that can cause a deterioration of the condition (such as phototherapy, oral corticosteroids, or coal tar preparations). However, the treatment plan is best left in the hands of a dermatology expert.

The following treatment methods are used to manage Erythrodermic Psoriasis:

• Hospitalization, including administering intravenous fluids to restore electrolyte balances
• Use of wet dressings with mild creams around the red skin areas
• Nutritional support
• Use of immunosuppressive drugs, such as cyclosporine and methotrexate
• A combination therapy using other drugs such as infliximab and acitretin
• Use of biologic agents that include TNF-alpha inhibitors, adalimumab, and IL12/23 inhibitors
• Symptomatic treatment, such as managing infections using antibiotics as needed

Medications can have significant side effects. Besides, since the medications are often needed for a long duration of time, it is recommended that combinations of medications be used to reduce side effects. This is known as rotational therapy.

How can Erythrodermic Psoriasis be Prevented?

Currently, there are no preventative measures available for Erythrodermic Psoriasis since it is a genetic condition. Ensuring proper self-care and regular healthcare visits are helpful in mitigating the severity and discomfort.

• Ensuring consistent and adequate treatment of psoriasis vulgaris can help avoid Erythrodermic Psoriasis, in some cases
• Withdrawal of medications used in psoriasis should be planned and follow medical guidelines
• Avoiding triggers that exacerbate the condition can help with treatment
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• If there is a family history of the condition, then genetic counseling will help assess risks before planning for a child

Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic conditions.

What is the Prognosis of Erythrodermic Psoriasis? (Outcomes/Resolutions)

• The prognosis for Erythrodermic Psoriasis depends on the extent of the condition and severity of signs and symptoms
• The prognosis may vary from one to another and may be established only on an individual basis. In severe cases, the prognosis can be poor, and Erythrodermic Psoriasis can result in fatalities
• With aggressive and consistent treatment, the outcomes may be improved

In any case, psoriasis lasts a lifetime, and so, a close monitoring of the symptoms with appropriate treatment will help decrease the burden of the disease.

Additional and Relevant Useful Information for Erythrodermic Psoriasis:

• There is no evidence to prove that oily foods and chocolate-based products have an influence on the development of this condition
• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken to avoid strong soaps and chemicals that could potentially worsen the condition
• The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help prevent the condition from getting worse