Erythroderma - A Dermatological Emergency

What Is Erythroderma?

Erythroderma is a condition with widespread skin reddening due to inflammation. It is followed by or associated with peeling off the skin in layers or scales. Hence, it is also known as exfoliative dermatitis. This condition involves more than 90 % of the body surface and often occurs due to underlying inflammatory conditions. The condition can be life-threatening and can cause numerous systemic manifestations.

What Is the Cause of Erythroderma?

The underlying cause of erythroderma needs to be adequately understood. The reason why certain skin conditions escalate into erythroderma in some individuals but not in others is believed to be because of the difference in lymphocytes and keratinocytes, their structure, or their interaction with cytokines and adhesion molecules. It has also been proposed that the turnover rate of epidermal cells can affect erythroderma.

Erythroderma can often be associated with an underlying skin or systemic condition.

1. Some skin conditions are known to cause erythroderma includes:

• Dermatitis: Atopic dermatitis is a common form of dermatitis known to cause erythroderma.

• Drug Eruption: Several drugs can cause a response in the body called drug eruptions which can, in turn, lead to erythroderma.

• Psoriasis: The withdrawal of systemic steroids to treat psoriasis can cause erythroderma.

• Pityriasis Rubra Pilaris - The condition is characterized by red or orange scaly patches on the skin. The symptoms may flare up on exposure to sunlight, infection, or minor trauma.

2. Skin conditions that are lesser known to cause erythroderma include:

• Dermatitis: Several forms of dermatitis, such as contact, allergic or irritant, or stasis dermatitis, can cause erythroderma. In babies, seborrhoeic dermatitis or staphylococcal scalded skin syndrome can cause erythroderma.

• Pemphigus: Blistering skin conditions such as pemphigus and bullous pemphigoid.

• Congenital Ichthyosis Conditions: It is an autosomal recessive congenital disorder of cornification associated with systemic symptoms. The thickness of the stratum corneum causes the skin to be too fragile and itchy.

• Sezary Syndrome: It is a type of blood cancer involving white blood cells. Cancer affects the skin making it extensively itchy.

3. Certain systemic conditions can also cause erythroderma, such as:

• HIV (human immunodeficiency virus) infection.

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• Graft-versus-host disease.

• Cancers of blood components such as leukemia and lymphoma.

What Are the Clinical Features of Erythroderma?

• Erythroderma is a rare skin condition, but it can occur at any age and has no racial predilection.

• Gender preference - Males are three times more affected than females.

• It is often associated with a preexisting skin condition or a systemic condition. However, in about 30 percent of cases, erythroderma can occur without any specific cause.

• Erythrodermic atopic dermatitis affects children and young adults, but other forms are more common in elderly or middle-aged individuals.

How Does the Disease Progress?

Dermatitis, measles-like bumps, psoriasis, or such skin conditions always precede erythroderma. This turns into generalized redness (erythema), which can suddenly become an acute erythroderma condition. Then, for weeks or months, this develops into chronic erythroderma.

Clinical manifestations include:

• It is considered erythroderma when 90 percent or more of the skin is red, edematous, or covered in bumps.

• The classical signs of an inflammatory response can be seen.

• The skin is warm to the touch.

• Itching can occur, which can lead to scratching and, thereby, lichenification.

• Swelling of the eyelids results in ectropion.

• Two to six days after, redness scaling can occur, which can occur in large sheets or flakes.

• Hair loss occurs, which can progress to baldness due to scaling on the scalp.

• Nails can become affected and become ridged, dull, and thickened. In addition, onycholysis can occur or nail shedding.

• Generalized lymphadenopathy or swelling of lymph nodes is also seen.

How to Identify Underlying Systemic Causes?

Some clinical features can help identify the underlying systemic cause:

• Atopic Erythroderma: Here, the oozing of serous liquid can cause the dressings of clothes to stick to the skin, along with an unpleasant odor.

• Pityriasis Rubra Pilaris: Follicular prominence, islands of sparing, and orange color are characteristic features.

• Psoriasis: Scaly plaques are found on elbows and knees.

• Papuloerythroderma of Ofuji: Abdominal creases sparing.

However, some systemic symptoms can either be the cause or the symptom of erythroderma:

• Lymphadenopathy, abnormal liver function, hepatosplenomegaly, and fever; can be a result of malignancy or due to drug hypersensitivity.

• Edema in the legs can be due to high cardiac output or cardiac failure, reduced serum albumin, or due to inflamed skin.

What Are the Complications of Erythroderma?

Erythroderma can have both local and systemic acute, immediate, chronic, or long-term complications.

• Temperature regulation is affected, causing fluid loss by transpiration to maintain homeostasis (adjusting the biological system to the changing external conditions).

• Heat loss leads to a reduction in temperature.

• Loss of fluid causes electrolyte imbalance and dehydration.

• Redness of the skin causes heart failure due to high cardiac output.

• The body can become prone to secondary infections such as cellulitis and impetigo.

• The patient can be prone to pneumonia.

• Long-standing cases of erythroderma can cause changes in pigmentation, such as white or brown patches on the skin.

• Protein loss and increased metabolic rate can cause reduced serum albumin and edema, respectively.

How to Diagnose Erythroderma?

Erythroderma can occur in association with another disease or separately. The diagnosis depends on that.

• Commonly erythroderma is associated with anemia, white blood cell count abnormality, and eosinophilia.

• Reduced serum albumin rate.

• Abnormal liver function.

• C-reactive protein (CRP) may be elevated.

• Sezary syndrome can be suspected if there are more than 20 percent circulating Sezary cells.

• Raised immunoglobulin E (IgE) and polyclonal gamma globulins suggest idiopathic erythroderma.

• If a blistering skin condition of autoimmune origin is suspected, direct immunofluorescence can be performed.

• If the cause is unidentified, a skin biopsy can be performed.

How to Treat Erythroderma?

• Erythroderma can be life-threatening and require immediate medical care. In addition, the patient needs to be monitored to prevent further loss of fluids and electrolytes.

• All unnecessary medications are to be stopped.

• Body temperature and fluid balance are monitored.

• Skin moisture is maintained using wet wraps; topical steroids are applied along with emollients.

• Antibiotics can be administered for bacterial infection, and antihistamines for the itch.

• If the underlying cause is identified, treatment should be made explicitly using topical and systemic steroids for dermatitis. Methotrexate or Acitretin for psoriasis.

How to Prevent Erythroderma?

• Erythroderma, in strict terms, can not be prevented but instead avoided.

• People with a history of drug allergies should stop using that drug.

• Medical records should be well maintained, and drug allergies should be kept in mind while prescribing a new drug.

• The patient should be well informed of their condition and educated about its associated risk.

What Is the Prognosis for Erythroderma?

The prognosis of erythroderma depends on the underlying cause. If it is identified and treated, the condition has a good prognosis. However, if the underlying cause is psoriasis or dermatitis, the condition can recur at any time, even though it can clear with appropriate treatment.

Conclusion:

Erythroderma is the reddening of the skin. It can be a severe and life-threatening condition. One can describe erythroderma as the inflammation of the skin; this describes the clinical manifestations of the disease. Identifying the conditions and monitoring the body temperature and electrolyte balance a crucial in stabilizing the patient.