What is Pustular Psoriasis?
Psoriasis is a skin disorder characterized by scaly, red patches on the skin. Psoriasis can develop anywhere on the body; however, it is most commonly found around the elbows and knees. Psoriasis is not a contagious condition and anyone can suffer from this frustrating skin condition. There are different forms or types of psoriasis one of which is Pustular Psoriasis. Pustular psoriasis, as the name itself suggests, is characterized pus-filled blisters (pustules), which are noninfectious in nature.
Development & Appearance of Pustular Psoriasis
Patient can develop pustular psoriasis in conjunction with other types of psoriasis, such as plaque psoriasis. Pustular psoriasis can develop in single areas, such as hands and feet or it can break all over the body. However, pustular psoriasis is rarely seen on the face. Pustular psoriasis often starts with redness and tenderness developing in a part of the skin. After some hours, there is development of the characteristic large blisters which are filled with noninfectious pus. Ultimately, these blisters turn crusty and brown in appearance. Then there is peeling off of these blisters and the underlying skin appears scaly or shiny.
What are the Types of Pustular Psoriasis?
Pustular psoriasis initially behaves like normal psoriasis where the patient will have patches of red skin and raised scales with burning or itching. After there is development of noninfectious pus filled blisters, then it becomes pustular psoriasis.
Acute & Chronic Pustular Psoriasis: Pustular psoriasis breakouts can be severe and sudden where they are known as acute pustular psoriasis. If the pustular psoriasis breakouts affect the patient multiple times over months or years then this is known as chronic pustular psoriasis. However, there are many patients who have symptoms which resemble both the extreme forms of pustular psoriasis, i.e. acute as well as chronic.
Pustular psoriasis is further classified into different types depending on the patient’s symptoms and the parts of skin covered with blisters.
Von Zumbusch Pustular Psoriasis: This is severest type of pustular psoriasis and is characterized by sudden breakouts, which cover large areas of the patient’s body. There is development of painful, red skin and pus-filled blisters in a couple of days. In Von Zumbusch Pustular psoriasis, patient also suffers fever, chills, itching and also feels tired. Patient may also have nausea and joint pain. Von Zumbusch Pustular Psoriasis can be potentially fatal, so it is important to seek immediate medical attention. Older patients are more prone to suffering from the more severe Von Zumbusch Pustular Psoriasis.
Ring-Shaped or Annular Pustular Psoriasis: This type of pustular psoriasis is relatively milder and the patient may not experience any symptoms except for skin blisters, which are pus-filled, raised rings on the arms, torso and legs. There is healing of the center of the rings before the edges.
Children rarely suffer from psoriasis, but when they develop pustular psoriasis, it is most often ring-shaped pustular psoriasis. Emotional stress greatly increases the risk of flare-ups of pustular psoriasis within a few hours. Steroid creams or ointments are effective in treating ring-shaped pustular psoriasis.
Palmoplantar Pustular Psoriasis: This type of pustular psoriasis is characterized by pus-filled blisters developing on the palms of the patient’s hands and the soles of the patient’s feet. Palmoplantar Pustular Psoriasis can recur many times over a period of months and years. Women are more commonly affected with palmoplantar pustular psoriasis than men. In some patients, there can be inflammation of bones or joints in palmoplantar pustular psoriasis.
Acropustulosis: This is a very rare type of pustular psoriasis characterized by pus-filled blisters which develop on the tips of the fingers and toes and commonly under the nails. Acropustulosis affects the fingers more than toes, and commonly develops after any injury. The blisters in acropustulosis can cause deformity of the nails or the nails can fall off. In severe cases of acropustulosis, the bones of the fingers or toes can change shape and become deformed.
What Are Causes and Risk Factors of Pustular Psoriasis?
The cause of pustular psoriasis is thought to be a combination of genetics and environment, which is also the cause for psoriasis. Factors which can aggravate or increase the risk of pustular psoriasis are: alcohol, smoking, stress and sun exposure. Medications, which can aggravate pustular psoriasis, are: Lithium, oral iodides, NSAIDs, hydroxychloroquine, strong, irritating topical solutions, recombinant interferon-beta injections, interferon alfa and abruptly stopping the use of high-dose systemic steroids. In some patients, the trigger factor for pustular psoriasis is not identified.
What Are the Signs & Symptoms of Pustular Psoriasis?
The generalized form of pustular psoriasis is characterized by the skin becoming tender and fiery red in color. Some patients can develop symptoms such as fever, nausea, headache, chills, pain in the joints, uneasiness and reduced appetite. Then, there is development of clusters of pus filled blisters within a few hours.
The pustules of pustular psoriasis commonly develop on the genital and anal regions and in the skin folds. Pustules can also develop on the face, although this but this is not common. The pus-filled blisters can also develop on the tongue, due to which the patient experiences difficulty in swallowing. The pus-filled blisters can also develop under the nails and cause the nails to come off.
Within 24 hours, the pustules of pustular psoriasis coalesce and form “lakes” of pus, which then dries and peels off in sheets. The surface of the skin underneath appears a smooth and reddish in color on which there can be development of new pustules. Recurrence of such episodes of pustular psoriasis can go on for days to weeks, as a result of which the patient feels extremely uncomfortable and exhausted.
After there is improvement in the pustules, majority of the symptoms of pustular psoriasis, such as fever and headache, often subside. In some patients, the skin can retain the bright red color followed by the classical plaque form of this disease.
The ring-shaped pustular psoriasis is commonly seen in young children and this type of pustular psoriasis is mostly sub-acute or chronic in nature. The symptoms of ring-shaped pustular psoriasis are relatively mild when compared to the generalized type of pustular psoriasis. There is development of recurrent ring-shaped plaques on the skin. The pustules develop on the edges of the ring. These symptoms commonly develop on the trunk and can also appear on the legs and arms. Eventually there is expansion of the edges and healing in the center area.
- The infantile or juvenile type of pustular psoriasis is also mild without any systemic symptoms. Juvenile type of pustular psoriasis usually resolves on its own.
- Pustular psoriasis which appears on the soles and palms with inflammation of joint or bone (psoriatic arthritis) is usually chronic in nature.
How is Pustular Psoriasis Diagnosed?
The following tests are conducted to diagnose Pustular Psoriasis:
- A complete blood count is done to check the lymphocytes count, which is reduced. Polymorphonuclear leukocytes are another type of leukocyte which can be high.
- The ESR or erythrocyte sedimentation rate is also usually increased, which indicates inflammation.
- Serum chemistry test shows increased plasma globulins and decreased albumin, zinc and calcium.
- A small sample of the contents of the pustule is taken to perform a culture and the result of which is often negative. As there is damage to the skin in pustular psoriasis, there is a risk of infection and doing the cultures is very important.
- In some cases, a skin biopsy is done to confirm the diagnosis of pustular psoriasis.
What is the Treatment for Pustular Psoriasis?
Patients suffering from generalized type of pustular psoriasis may need to be hospitalized for intravenous fluids and bed rest. Application of bland compresses is done to the patient’s skin. Patient is also given oatmeal baths, which help in soothing and healing of the skin affected by pustular psoriasis. This treatment suffices for children suffering with pustular psoriasis.
Treatment for pustular psoriasis consists of two basic types that is, topical therapy which consists of medicines applied to the skin; and systemic therapy which consists of medicines the patient needs to take orally or intravenously. Both the treatments can be used alone or can be used in conjunction.
Topical Treatment for Pustular Psoriasis: These are the medications which are applied directly to the skin. Topical treatment for pustular psoriasis is the first line of treatment. Primary medications used in topical treatment for pustular psoriasis consists of corticosteroids, coal tar, vitamin D-3 derivatives, retinoids or anthralin. There is no single topical medicine, which is effective for all patients suffering from psoriasis. Different topical medicines have different side effects and for this reason, the patient may need to use multiple medications or a combination of them to find out which is the best fit for the patient.
Narrow-band UVB light or Ultraviolet-B (UV-B) for treating Pustular Psoriasis: Ultraviolet-B having wavelengths of 290 to 320 nanometers can also be used for treating pustular psoriasis. This is often used in conjunction with one or more topical treatments.
Systemic Agents: Systemic medicines, such as retinoids can be used initially for treating generalized pustular psoriasis, followed by PUVA treatment. In case of milder and chronic types of pustular psoriasis, light treatment or topical therapy can be tried first. Systemic treatment can also be used in patients with severe pustular psoriasis where it becomes disabling for the patient either psychologically, physically, economically or socially.
What are the Medications used for Treating Pustular Psoriasis?
The aim of medications in treatment of pustular psoriasis is to manage and alleviate the symptoms of pustular psoriasis and to prevent any complications from pustular psoriasis. Systemic medications for treating pustular psoriasis include: Methotrexate, Cyclosporine, Infliximab, Etanercept, Adalimumab and Ustekinumab. In most cases, if the patient has extensive pustular psoriasis then the topical treatment has limited use in the treatment. Topical therapies, which can be used in milder forms of pustular psoriasis, include topical steroids, bland emollients, vitamin D-3 derivatives, anthralin, coal tar and retinoids.
Can Pustular Psoriasis be Prevented?
Pustular Psoriasis be prevented by avoiding its triggers, such as environmental factors like sun exposure, smoking and stress. Avoiding the triggers of pustular psoriasis can help in preventing or minimizing the flare-ups of pustular psoriasis. Alcohol is another risk factor for pustular psoriasis in young to middle-aged males. It is also important to follow a balanced diet to prevent pustular psoriasis or any other disease.
What Is the Prognosis of Pustular Psoriasis?
If treatment is not initiated, especially in the severe forms of pustular psoriasis, then complications can occur, which include:
- Bacterial skin infections.
- Nail loss and hair loss.
- Hypoalbuminemia, this is a condition where the level of albumin in the blood becomes abnormally low due to the loss of blood protein into the tissues.
- Hypocalcemia where the levels of calcium in the blood are extremely low.
- Liver and kidney damage.
- Malabsorption where there is insufficient absorption of the nutrients by the gastrointestinal tract.
- The von Zumbusch type of pustular psoriasis is the severest form of pustular psoriasis where the patient has fever and toxicity and can even cause death. It is important to treat this type of pustular psoriasis during the acute phase. Pustular psoriasis can become a serious condition in patients with compromised cardiopulmonary function and the elderly.
- Generalized pustular psoriasis can sometimes become complicated from acute respiratory distress syndrome.
- Recovery of children with pustular psoriasis is well as long as serious skin infections are avoided.
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