What is Exfoliative Dermatitis (Erythroderma)?
Erythroderma, also known as exfoliative dermatitis, was first identified by Von Hebra in 1868. This condition typically presents with widespread redness and peeling, covering more than 90% of the body’s skin. It’s not a disease itself but a skin reaction to various underlying health problems, like psoriasis or eczema, or a response to certain medications. While some experts think it’s not fatal, there’s a chance that erythroderma could be life-threatening. That’s why it’s so important to correctly identify this skin condition, figure out what’s causing it, and manage it appropriately.
What Causes Exfoliative Dermatitis (Erythroderma)?
There are several factors that could cause erythroderma, a condition where the skin becomes red and inflamed. These factors often fall into categories like existing skin conditions, certain medications, and cancers. However, sometimes it could happen for no apparent reason.
There’s a rare birth condition called bullous congenital ichthyosiform erythroderma, or simply epidermolytic hyperkeratosis, that can cause erythroderma.
The most frequent cause of erythroderma are certain pre-existing skin conditions. Among them, psoriasis is considered the most common, being behind roughly half of all erythroderma cases according to some studies. Eczema is also a well-known cause and in some studies, it has been pointed out as the main cause of erythroderma. There are other less common skin conditions that can cause erythroderma, like pityriasis rubra pilaris, acquired ichthyosis, cutaneous lupus, scabies, bullous pemphigoid, pemphigus foliaceus, actinic dermatoses, and actinic keratosis.
Some specific medications can trigger erythroderma too. These medicines include certain epilepsy medications like phenytoin, carbamazepine, and phenobarbital, certain antibiotics like sulfonamides, penicillins, and vancomycin, lithium, and allopurinol and others.
Erythroderma could also occur due to certain cancers. Approximately 1% of erythroderma patients have an underlying form of cancer. Cancers such as cutaneous T-cell lymphoma like mycosis fungoides and Sezary syndrome, B-cell chronic lymphocytic leukemia, and organ cancers such as stomach, esophagus, colon, liver, prostate, and lung cancer can have a strong link with erythroderma.
Risk Factors and Frequency for Exfoliative Dermatitis (Erythroderma)
The exact number of people affected by exfoliative dermatitis in the U.S. and globally is not known. Some research studies suggest various rates. For instance, a study in India found that out of 100,000 patients visiting dermatology clinics, 35 had erythroderma, which represents 0.035%. Another study done in Portugal found that almost 12% of patients hospitalized had erythroderma. Yet, another piece of research proposes a rate of 1 to 2 patients per 100,000 suffering from the condition.
- Erythroderma, another term for exfoliative dermatitis, tends to affect men more than women, with a ratio ranging from 2 to 4 men for every woman affected.
- The common age of onset for this skin condition is typically between 40 and 60 years old.
Signs and Symptoms of Exfoliative Dermatitis (Erythroderma)
Erythroderma is a skin condition marked by red patches that merge to cover nearly all of the skin, followed by the development of a white or yellow scale. The skin could look shiny and thin, and sometimes patients might feel that their skin is tight due to increasing thickness and swelling. Itching is a common symptom in nearly all people with erythroderma, and over half of the patients might also experience fever. In addition, some patients might show signs of enlarged lymph nodes, an enlarged spleen, or an enlarged liver, which could indicate a drug reaction or a serious condition like cancer.
Moreover, some patients might also experience hair loss, and changes to the nails, such as:
- Thickening of skin under the nail
- Nail detachment
- Ridges on nails
- Dry or brittle nails
- Nail shedding
Additionally, signs of the cause of the erythroderma might be present. For instance, in patients with psoriasis, they may have psoriasis-like patches, or in patients with pityriasis rubra pilaris, signs of a thickened and rough skin condition on the palms of the hands and the soles of the feet may be seen.
Testing for Exfoliative Dermatitis (Erythroderma)
Erythroderma is a condition that a doctor identifies by observing your skin, but they often use lab tests and tissue samples (histopathology) to confirm their suspicion and figure out the cause. A type of test called direct immunofluorescence might be used to check for autoimmune skin or connective tissue diseases.
In many cases, the lab results don’t point to a specific condition. However, two tests, ESR (Erythrocyte Sedimentation Rate) and CRP (C-reactive protein), are usually high in most patients with erythroderma. Some patients may also show signs of anemia (lower than normal number of red blood cells), leukocytosis (increase in the white blood cells), an increase in eosinophils (type of white blood cells), and abnormal levels of proteins in the blood.
Treatment Options for Exfoliative Dermatitis (Erythroderma)
Erythroderma is a severe skin condition that can be life-threatening and might lead to a hospital stay. Treating this condition mainly involves consistent monitoring and ensuring that your body’s metabolic and circulatory systems are functioning well. To combat the symptoms, resting in bed, proper wound care, including warm baths, wet dressings, and gentle use of medicated creams and simple skin moisturizers can be beneficial. Additionally, oral antihistamines might help with discomfort. If there are signs of an additional infection, antibiotic treatment is required. It’s critical that the root cause of erythroderma is identified and treated accordingly.
For those with idiopathic erythroderma, meaning the root cause is unknown, treatments may include moisturizers, oral or topical steroids, methotrexate (a medication used to manage severe skin diseases), azathioprine (a medication used to decrease your immune system’s response), PUVA (a type of ultraviolet radiation therapy), or other treatment options depending on the patient’s reaction to these treatments.
What else can Exfoliative Dermatitis (Erythroderma) be?
Erythroderma is a skin condition that can be a symptom of many potential causes, such as skin diseases, medications, cancers, or unknown reasons. The exact cause can be identified through patient history, clinical observations, skin tests, such as biopsy and immune fluorescent tests, and laboratory tests. Other conditions that could be responsible instead of Erythroderma and should be considered are:
- Acanthosis nigricans
- Acute complications of sarcoidosis
- Cutaneous T-cell lymphoma
- Dermatologic manifestations of graft versus host disease
- Allergic contact dermatitis
- Bullous pemphigoid
- Familial benign pemphigus (Hailey-Hailey disease)
- Irritant contact dermatitis
- Lichen planus
- Pityriasis rubra pilaris
- Plaque psoriasis
- Reactive arthritis
- Pediatric atopic dermatitis
- Pemphigus foliaceus
- Seborrheic dermatitis
- Stasis dermatitis
Any of these conditions can cause skin changes similar to Erythroderma, so it’s important for doctors to rule them out when making a diagnosis.
What to expect with Exfoliative Dermatitis (Erythroderma)
The way a person responds to treatment can differ based on what’s causing their condition. For instance, erythroderma, a skin condition that results in redness and scaling, varies in response depending on its cause.
If the erythroderma is caused by a drug, it usually gets better quickly once the drug is stopped and timely treatment is provided. Whereas, erythroderma caused by a primary skin disease may take a longer time to improve.
On the other hand, erythroderma due to a cancer could worsen over time. How it progresses and its timeframe can be closely tied to the outcome of the underlying cancer and how well it responds to its own treatment.
Lastly, if the cause of the erythroderma can’t be determined (idiopathic), the course it might take can be unpredictable. People with idiopathic erythroderma may experience periods where their condition worsens or improves (relapse and remission).
Possible Complications When Diagnosed with Exfoliative Dermatitis (Erythroderma)
Exfoliative dermatitis used to be associated with a death rate of anywhere from 4% to 64%. However, these figures are now outdated due to advancements in healthcare and treatment techniques. There are now several case reports that suggest a far lower death rate, from 0% to just 6%. These studies propose that erythroderma, another term for exfoliative dermatitis, isn’t generally a significant threat to life. Another report also notes that for those with non-cancerous forms of erythroderma, the risk of death is minimal.
However, the situation is different for elderly patients who experience complications such as infections, fluid/electrolyte imbalance, or heart failure – these factors can increase their risk of death. The most common causes of death in patients with erythroderma are heart failure, septicemia (infection in the blood), and pneumonia.
Common Complications:
- Infections
- Fluid/electrolyte imbalance
- Heart failure
- Septicemia
- Pneumonia
Preventing Exfoliative Dermatitis (Erythroderma)
For people who have experienced a severe skin reaction known as erythroderma due to a certain medication, it’s crucial to avoid that specific medication in the future. If you have a chronic skin condition, it’s important to manage it properly and regularly check in with your doctor, to lower the chance of the severe reaction happening again. Those with conditions like psoriatic arthritis, atopic dermatitis, or other conditions that improve with steroid medication should be aware that suddenly stopping these types of medicines could trigger a severe skin reaction.
People who have an underlying cancer should make sure to get regular care and keep in regular contact with their cancer doctors (oncologists).
It’s important to have realistic expectations about your recovery. The timescale for seeing improvement can vary from person to person. Also, be aware that in rare cases, the skin reaction can lead to longer-term changes. These can include changes in the color of the skin (either darker or lighter), changes to your fingernails and toenails, and the formation of thickened scar tissue (keloids), among other things.
