What is Generalized Pustular Psoriasis?
Pustular psoriasis is a rare and severe type of skin condition where clusters of small blisters or pustules form on the skin. These blisters or pustules don’t contain any infectious bacteria. The symptoms and underlying features of this type of psoriasis are quite intense compared to other types.
The age at which people get generalized pustular psoriasis, what triggers it, how severe it is, and how it develops over time can all vary greatly. As a result, several overlapping types of this skin condition are recognized.
This type of psoriasis is related to another type called plaque psoriasis. Some people with generalized pustular psoriasis may have had episodes of plaque psoriasis before or after they have a generalized pustular psoriasis flare. However, others only experience the generalized pustular psoriasis and never develop plaque psoriasis.
What Causes Generalized Pustular Psoriasis?
There are various things that can trigger or make generalized pustular psoriasis worse. These include:
1. Infections: Both viral and bacterial infections can increase the problems associated with this type of psoriasis. This was noted in 12 of 16 patients with diagnosed generalized pustular psoriasis in one study.
2. Medications: Corticosteroids, a type of medication, are known to aggravate the condition. Stopping the usage of systemic corticosteroid therapy, which is a type of treatment that affects your whole body, can make psoriasis more severe. Topical therapy, i.e., medication applied directly to the skin, using potent corticosteroids has also been identified as a factor. Other drugs such as ciclosporin, terbinafine, propranolol, bupropion, lithium, phenylbutazone, salicylates, and potassium iodide may cause the disease, particularly when stopped. Coal tar and dithranol, substances used in skin treatments, may cause the psoriasis to trigger pustulation, which is the formation of small blister-like bumps, if they are applied without caution when the disease is unstable.
3. Psychological stress: Many people note that their condition gets worse in periods of high stress.
4. Low calcium levels: Generalized pustular psoriasis can result in low calcium levels(hypocalcemia), which in turn can make the condition worse. This can also be the result of low parathyroid hormone(hypoparathyroidism), that doesn’t allow the body to maintain proper calcium levels.
5. Pregnancy: Being pregnant is acknowledged as one of the risk factors that can make the condition worse.
Risk Factors and Frequency for Generalized Pustular Psoriasis
Generalized pustular psoriasis is a rare skin condition. Here’s what we know about who it affects and how:
- It is very uncommon. In France, there are about 0.64 new cases and about 1.76 existing cases per million people each year. In Japan, it affects about 7.46 per million people.
- Most new cases occur between ages 40 and 59, but it can also affect children and teenagers. People who only have generalized pustular psoriasis, and not another type called plaque psoriasis, usually get it at a younger age.
- Women are about twice as likely to have it as men, but this isn’t consistent in all studies.
- People with this condition often also have inflammatory polyarthritis, a type of joint inflammation. In one study, a third of patients had this condition.
- There’s also a common link with metabolic syndrome, a group of conditions that increase the risk of heart disease and type 2 diabetes. In one large study, the patients also had obesity (43%), high blood pressure (26%), abnormal cholesterol levels (26%), and diabetes (24%).
Signs and Symptoms of Generalized Pustular Psoriasis
Pustular psoriasis is a type of skin condition, which usually starts with a burning sensation and pain in the skin, sometimes accompanied by fever and general fatigue. This disease can worsen with time, leading to the development of pus-filled blisters. Some people may even experience joint pains.
Here are some different types of this disease based on symptoms and how they progress:
- Von Zumbusch: This is the most intense form of pustular psoriasis. It was named after Leo von Zumbusch, who first described it in two siblings. The diagnosis involves identifying the physical symptoms, abnormal blood tests, and the presence of a specific type of blister under a microscope. This type may start as plaque psoriasis and later transform into pustular psoriasis because of steroid usage or other environmental factors. It could also start suddenly, usually following an infection. Early symptoms include a burning sensation in the skin, which becomes dry and tender, and these are followed by fever and general bad health. Existing skin issues develop into tiny blisters. Redness of the skin worsens, and blisters spread to other areas. The disease process may resolve within a few days or weeks leading to the skin going back to normal or further inflammation.
- Generalized Pustular Psoriasis in Infancy: This rare condition starts in infancy as discrete areas of redness which become elevated. Blisters appear peripherally on the advancing edge, become dry and shedding in the form of scales as the lesion gradually progresses. There are no accompanying systemic symptoms.
- Impetigo Herpetiformis: This subtype occurs during pregnancy, usually in the third trimester. It begins from flexural surfaces as symmetrical, confluent areas of blistering. The lesions start in the groin region and other folds of the skin, with small pustules appearing on inflamed skin. These spread radially, become dry in the center, or form plaques that may become widespread and leave a reddish-brown pigmentation as they heal. The tongue, oral cavity, and even the esophagus may be involved, with erosions following pustules. The risks of severe complications increase with the severity of the disease.
In 30% of cases, the disease occurs in the first year of life, where infants usually do not show systemic symptoms and may not require any treatment. The disease may be limited to flexure areas like the neck for protracted periods. More severe forms with systemic involvement require active management. In older children, the disease resembles that in adults and may take any pattern.
Testing for Generalized Pustular Psoriasis
There are several lab tests that can assist your doctor in assessing your illness. These include:
– A test to check your erythrocyte sedimentation rate (a measure of inflammation in your body)
– A test to monitor your C-reactive protein levels (another marker of inflammation)
– A test to evaluate your lymphocyte count at the beginning of your illness, which may be unusually low, but soon followed by a high count of a type of white blood cell called polymorphonuclear leukocytes
– Tests to measure the levels of plasma albumin, zinc, and calcium, which may be below normal levels
– A lipid profile test, which may show abnormal levels.
These tests provide valuable clues to your doctor about the state of your health and can help guide your treatment plan.
Treatment Options for Generalized Pustular Psoriasis
Generalized pustular psoriasis, a type of skin condition characterized by pus-filled blisters, can be managed with a combination of practical measures and medical treatments.
Practical measures, also known as conservative treatment, include encouraging a high fluid and protein intake and keeping an eye on fluid levels. If there’s an infection, antibiotics can be used for treatment. A medical team may also suggest terminating pregnancy if the life of the mother is at risk. To avoid getting too cold, preventive measures against hypothermia are also necessary.
Topical treatments, or those applied directly to the skin’s surface, can also be beneficial. These can include simple skin care products called emollients, which can moisturize and protect the skin, along with diluted corticosteroids, a type of anti-inflammatory medication that reduces swelling and redness.
Additionally, a range of medications that work internally, or systemic treatments, can be used to manage generalized pustular psoriasis. First-line treatments, which are used when the condition is first diagnosed, can include medicines such as acitretin (a retinoid that encourages skin cell turnover), methotrexate (a medication that suppresses the immune system), and ciclosporin (a drug that also reduces the immune response). However, these medications should not be used during pregnancy due to the potential risks.
If these drugs don’t work or lead to problematic side effects, there are second-line treatments, including infliximab, adalimumab, and etanercept, which are all biologics that work by targeting specific parts of the immune system. In certain special conditions and complications, the corticosteroid prednisolone may also be used.
If a patient isn’t able to tolerate or does not respond to any of the first or second-line treatments, third-line treatments such as anakinra, a drug that blocks the inflammatory response, may be considered. It must be emphasized that all these treatments should only be used under physician supervision, with the selected approach depending on the individual patient’s condition and the potential benefits and risks associated with each medication.
What else can Generalized Pustular Psoriasis be?
The process of diagnosing widespread nonfollicular pustules involves eliminating other possible conditions that cause similar symptoms. This includes conditions such as acute generalized exanthematous pustulosis, subcorneal pustular dermatosis of Sneddon and Wilkinson, and IgA pemphigus.
- Acute generalized exanthematous pustulosis (AGEP): This is a rash that comes with fever and causes skin inflammation and pustules (blister-like bumps filled with pus). It usually gets better after two weeks of stopping whatever caused it – it can be triggered by drugs or infections.
- Subcorneal pustular dermatosis of Sneddon and Wilkinson: This is a skin condition that causes redness and blisters, mainly on the body’s trunk and the areas where skin folds rub against each other. Whether it’s linked to pustular psoriasis is still under question.
- IgA pemphigus: This is a skin disease that can look a lot like generalized pustular psoriasis, but tests that look at the microscopic structure of the skin and the antibodies in the body help tell them apart.
During the acute phase, symptoms like fever, increased white blood cell count, and high markers of inflammation may result in a misdiagnosis of systemic infection. This can sometimes lead to the unnecessary stoppage of immunosuppressive treatment, which could do more harm than help. Also, the condition known as glucagonoma might be suspected because it also often comes with weight loss, inflammation of the tongue (glossitis), and anemia. However, it can be differentiated due to its characteristic migratory necrolytic eruption.
What to expect with Generalized Pustular Psoriasis
Without the right treatment, acute pustular psoriasis could potentially lead to death. However, for slower-developing types, like subacute annular and circinate generalized pustular psoriasis, the outlook is quite good. This is especially true for children suffering from the disease. Furthermore, pustular psoriasis that has a recognisable trigger, like the type that sometimes occurs during pregnancy, typically has an optimistic prognosis.
On the other hand, generalized pustular psoriasis that stems from a condition called acrodermatitis continua of Hallopeau, usually has the worst outlook. In simple terms, acrodermatitis continua of Hallopeau is a rare and severe form of pustular psoriasis affecting the fingers and toes.
Possible Complications When Diagnosed with Generalized Pustular Psoriasis
During the acute phase of generalized pustular psoriasis, the person can feel very sick. The following list details possible complications associated with this disease:
- Acute kidney injury can occur from a severe decrease in blood volume (hypovolemia) and a significant drop in blood circulation to the body’s organs (oligaemia).
- There can be a sudden noticeable drop in albumin, a type of protein in the blood (hypoalbuminaemia) because of the protein leaking into body tissues or due to problems absorbing nutrients from the gut (intestinal malabsorption).
- The drop in albumin can cause a decrease in calcium levels in the body (hypocalcemia).
- Liver enzymes may show abnormal levels suggesting potential issues with liver function.
- A type of liver condition where there is a backup of bile (cholestatic jaundice) can occur as a result of a specific type of liver inflammation (neutrophilic cholangitis).
- In rare cases, a severe lung condition causing breathing difficulties (acute respiratory distress syndrome) can occur.
- The disease process may also be complicated by a staphylococcal infection, a type of bacterial infection.
- If the disease lasts for a long time, it can cause hair loss from all areas of the body.
- Hair loss may continue to occur 2 to 3 months after the peak of the illness as part of a condition known as telogen effluvium.
- Amyloidosis, a condition where an abnormal protein, amyloid, builds up in the organs, is a rare complication that can occur at a later stage.
