What is Pustular Psoriasis?
Pustular psoriasis is a rare skin condition caused by an overactive immune system. It features small, yellowish blisters filled with fluid (known as pustules) on red, inflamed skin. These blisters can appear on any part of the body and each case often looks different. This condition is a type of psoriasis, a skin disorder that causes red, scaly patches on the skin. The unique feature of pustular psoriasis is that the blisters are filled with white blood cells, specifically neutrophils, but are not infected or dangerous. Unlike the most common type of psoriasis, plaque psoriasis, the skin areas affected by pustular psoriasis may be sensitive and sore.
Pustular psoriasis can be further divided based on the appearance and location of the blisters. Here are different types of pustular psoriasis:
* Widespread types:
* von Zumbusch type: This form involves a widespread rash along with symptoms like fever, joint pain, and others.
* Annular type: This type presents as ring-shaped rashes with blisters along the outer edge.
* Exanthematic type: This type is characterized by a sudden onset of blisters but doesn’t cause other overall health symptoms and usually clears up in a few days.
* Impetigo herpetiformis: This is a type of pustular psoriasis that arises during pregnancy.
* Localized types:
* Acrodermatitis continua of Hallopeau: This form mainly affects the fingers, toes, and areas around the nails.
* Palmoplantar psoriasis: This form mainly affects the palms of the hands and soles of the feet.
What Causes Pustular Psoriasis?
Pustular psoriasis is a type of skin condition that can happen in people who have a certain genetic makeup, and can be set off by specific factors that cause changes in the skin and a build-up of a type of white blood cell known as neutrophils in the outer layer of the skin. People with pustular psoriasis are more likely to have a particular genetic variant known as the HLA-B27 allele than the general population.
For many people, pustular psoriasis seemingly happens for no known reason, but there are certain risk factors that might play a part in causing pustular psoriasis. These include:
– Suddenly stopping the use of certain medication called systemic steroids
– Having skin infections, especially caused by a bacteria known as Staphylococcus aureus
– Having an electrolytes imbalance, particularly low levels of calcium in the blood, a condition known as hypocalcemia (this is notably seen in a subtype of pustular psoriasis known as von Zumbusch)
– Taking certain medications like lithium, iodine, penicillin, and interferon-alpha
– Being pregnant (seen in a specific form of pustular psoriasis known as impetigo herpetiformis)
– Undergoing a type of skin treatment known as phototherapy
– Having certain vaccinations like BCG and H1N1.
Risk Factors and Frequency for Pustular Psoriasis
Pustular psoriasis is an uncommon skin condition that affects both kids and adults. The condition typically shows up at two distinct age ranges. In adults, it’s most common between ages 40 and 50, while in kids, it’s usually seen in infants. This condition is more prevalent in the Asian population than in Caucasians. Both women and men are equally likely to get it. Pustular psoriasis variants make up about 1% of all the cases of the skin condition called psoriasis vulgaris.
Signs and Symptoms of Pustular Psoriasis
Pustular psoriasis is a skin condition that appears as small, yellowish, round sores or pustules on reddened skin. These sores can be scattered or clumped together and can affect certain areas of the body or be spread over a larger area. When someone has this condition, it’s important for a healthcare professional to do a full body skin exam. They’ll check not only the skin, but also the moist parts of the body (like the inside of the mouth) and the nails. They do this to look for more signs of psoriasis and to make sure that the sores aren’t caused by something else.
When pustular psoriasis first appears, it starts out as red spots or raised areas on the skin. These quickly turn into the yellowish sores on a red background that we talked about before. There are different types of pustular psoriasis. The generalized von Zumbusch type can come with other symptoms like fever, joint pain, headaches, and an increase in white blood cells. On the other hand, the exanthematic type shows up as a sudden rash of sores, but doesn’t come with any other symptoms. The annular type usually occurs in kids as ring-shaped sores with pustules along the edge of the ring. Lastly, there are types of pustular psoriasis that only affect certain parts of the body like the fingers, toes, and nailbeds, such as Acrodermatitis continua of Hallopeau, or the palms and soles, like palmoplantar psoriasis.
Testing for Pustular Psoriasis
If you’re suspected to have a particular type of severe psoriasis known as pustular psoriasis, especially the von Zumbusch subtype which can be dangerous, your doctor will need to perform a careful assessment. This will involve a range of laboratory tests.
For example, a complete blood count (CBC) might be conducted; this test measures the cells that make up your blood. Through this, the doctor can check for an abnormally high number of white blood cells, also called leukocytosis, a common sign of infections or other diseases.
They might also test the amount of calcium in your blood with an electrolyte panel. Low levels of calcium, or hypocalcemia, are often seen in patients with severe psoriasis. Along with this, a panel test may be done on your liver. This helps them look for signs of liver problems, such as transaminitis (an increase in certain enzymes that could indicate liver damage).
If you’re a woman that could become pregnant, the doctor will likely order a pregnancy test. This is simply to ensure they have all the information needed to plan your care.
If your condition is hard to diagnose, the doctor might perform a punch biopsy; this procedure involves removing a small sample of skin to examine under a microscope.
Also, if you have any areas of skin that are weeping or oozing, the doctor may take a sample for culture. This will allow them to check for any primary or secondary infections that might need to be treated.
Treatment Options for Pustular Psoriasis
The first step in managing a condition called pustular psoriasis is to identify what’s causing it, which could be something like an infection. Pustular psoriasis is a type of skin condition that causes blisters filled with pus on the skin. The more severe type is known as generalized pustular psoriasis (one subtype is von Zumbusch). It could be so severe that hospital admission may be necessary. Symptoms like fever and joint pain might also show up, and they’d need to be treated with medications that control fever and reduce inflammation.
There are certain medications that are usually the first choice for adults with this condition. These include specific types of drugs known as systemic retinoids, methotrexate, cyclosporine, and infliximab. For children, the preferred medications are acitretin, cyclosporine, methotrexate, and etanercept.
If a patient doesn’t respond to these first-line options, there are second-line treatments that can be considered. If the psoriasis covers large areas of the body, medications like etanercept and adalimumab can be tried. If it’s in more localized areas, creams and ointments like corticosteroids, calcipotriene, and tacrolimus might be more useful. These second-line options can be used by themselves or alongside the first-line treatments. Phototherapy, which involves exposing the skin to ultraviolet light, is another treatment that could be considered. Pregnant people with a severe type of skin condition called impetigo herpetiformis may be advised to give birth early.
Recent research has shown that some newer treatments can be effective as well. These involve using drugs called anakinra, which works by blocking a body protein named IL-1, and IL-36 receptor antagonists. Tocilizumab, an antibody that blocks the IL-6 receptor, may also be effective, especially in difficult cases of pustular psoriasis. It’s always important to remember to discuss with your healthcare provider about these treatment options and their potential risks and benefits.
What else can Pustular Psoriasis be?
When trying to diagnose a skin condition, doctors consider a range of potential illnesses that can cause similar symptoms. These include:
- Acute generalized exanthematous pustulosis
- Drug eruption reaction
- Pemphigus vulgaris
- Pemphigus foliaceous
- Impetigo
- Dermatitis Herpetiformis
- Infected eczema
- Erythroderma
- Acute cutaneous lupus
- Dyshidrotic eczema
- Disseminated herpes simplex virus
What to expect with Pustular Psoriasis
Pustular psoriasis is a long-term skin disease that often results in repeated outbreaks or flare-ups. The goal of treatment is to shorten these flare-ups, prevent further complications, and aim for longer periods without disease symptoms.
There are different types of pustular psoriasis. A specific type called von Zumbusch has a higher risk of causing death due to it affecting the whole body, especially when the patient has other health conditions like cancer, kidney failure or liver failure. For localized types of pustular psoriasis – types that are only present in certain areas of the body – the risk of death is much lower.
Possible Complications When Diagnosed with Pustular Psoriasis
- Low calcium levels in the blood
- Widespread blood infection
- High body temperature
- Liver damage
- Sudden kidney failure
- Poor nutrition
Preventing Pustular Psoriasis
People with psoriasis must be informed to avoid suddenly stopping their steroid medication as it can lead to bouts of a different type of psoriasis, known as pustular psoriasis. Pustular psoriasis is characterized by red, swollen patches on the skin with pus-filled bumps. Also, if they have flaccid lesions – patches of damaged skin that are soft and sagging – these should be treated with appropriate wound care. This care is crucial in preventing more serious problems and additional infections.
