Pustular Psoriasis

Although rare, Pustular Psoriasis is a very serious condition which affects the body both internally and externally. We seek to understand this disease by discussing in depth the various types of pustular psoriasis, their causes, symptoms, and treatments.

This page includes information on the types of pustular psoriasis including generalized pustular psoriasis, and its causes, symptoms, and treatment. We also look at the types of localized pustular psoriasis, including palmo-plantar pustulosis, its symptoms and treatment, and acrodermatitis and its methods of treatment

Types of Pustular Psoriasis

Pustular (pronounced Pus-tew-ler) psoriasis is an uncommon, but potentially very serious type of psoriasis. There are two main types of Pustular Psoriasis: Generalized and Localized. If it widespread over the body it is referred to as Generalized Pustular Psoriasis; if it is limited to just one part of the body, it is referred to as Localized Pustular Psoriasis. There are two types of Localized Pustular Psoriasis: Acropustulosis, which occurs only on the tips of the fingers, and Palmo-plantar pustulosis, which only occurs on the palms of hands and/or the soles of feet.

Generalized Pustular Psoriasis

What is Generalized Pustular Psoriasis?

Generalized Pustular Psoriasis, also known as Von Zumbusch Pustular Psoriasis, is characterized by large, reddened, and painful areas of skin developing suddenly (within a few hours) and randomly on large areas of the body, with pustules, or puss-filled blisters appearing on the skin. These blisters occur in a cyclical pattern - lasting over several days, drying up, peeling, and coming back again. This type of Pustular Psoriasis is very serious, and may require hospitalization for treatment.

Occurring most frequently in typically adults, it can have potentially life-threatening complications for them as chemical imbalances of this type overwork the heart and kidneys. It occurs rarely in children, and they are more likely to have a higher rate of improvement than adults. Because the body relies on normal skin coverage to control its temperature, Generalized Pustular Psoriasis keeps the body from being able to regulate its temperature naturally. In addition, the plaques (or blisters) serve as a means for germs to enter the skin and cause infection, as well as aid in the loss of body fluid. Relapses are common in survivors of the acute phase and its complications. Patients experience remission within days or weeks, and the psoriasis will revert to it's previous state or may develop erythroderma.

Causes of Generalized Pustular Psoriasis

On average, Generalized Pustular Psoriasis has no identifiable cause (idiopathic). However, in some cases, people with this type of psoriasis have a history of the common variety of psoriasis, chronic plaque psoriasis, which is characterized by persistent scaly patches of skin. The following have also been implicated as possible causes of Generalized Pustular Psoriasis:

Drugs, including beta blockers, iodide, salicylates, indomethacin, lithium, and the withdrawal of oral/injected corticosteroids.
Topical preparations, including coal tar, dithranol and strong topical corticosteroids under occlusion.
Infection
Pregnancy

Symptoms of Generalized Pustular Psoriasis

The skin initially becomes dry, red and painful. This may be accompanied by chills, a fever, headache, rapid pulse rate, nausea, muscle weakness, or loss of appetite. Two to three millimeter pustules filled with non- infectious pus appear, concentrated on the flexures and genital areas. These pustules then form pools of pus, which will then dry and peel. The new skin will have a shiny and smooth surface on which new pustules might form. In a pattern of every few days or weeks, these groups of pustules may form, erupt, dry and peel.

Treatment for Generalized Pustular Psoriasis

The treatment of Generalized Pustular Psoriasis is a means to restore the skin’s natural barrier function and chemical balance, prevent additional fluid loss, and restore the body’s stable temperature. Treatment of severe cases requires hospitalization, which may include antibiotics in case of infection, bed rest, topical treatments, rehydration, and avoidance of heat loss. If a person becomes exhausted due to recurring outbreaks, systemic (oral or injected) drugs may be used in treatment. These include:

Acitretin (Soriatane) or methotrexate (prescribed often)
Cyclosporine (Neoral): Primarily used for only severe plaque psoriasis, but successful in few cases of pustular psoriasis
Oral steroids: Contriversial, as withdrawal from oral steroids may trigger generalized Pustular Psoriasis. Prescribed only when other treatments have failed or if the patient is very ill.
PUVA: Used after passing of severe stage

*Systemic treatments in children is generally avoided, as children may improve without these medications.

Localized Pustular Psoriasis

Types of Localized Pustular Psoriasis

Localized Pustular Psoriasis either occurs on the tips of the fingers (acropustulosis) or affects only the palms of hands, soles of feet (palmo-plantar pustulosis). It is very painful, and may keep you from any daily activity upon which you need your hands and feet, including walking and eating.

Palmo-Plantar Pustulosis

In general, Palmo-Plantar Pustulosis occurs in people between 20 and 60 years old, and may be triggered by infection and/or stress. It has also been found to affect females more than males. As with Generalized Pustular Psoriasis, Palmo-Plantar Pustulosis occurs in a cyclical pattern, with new pustules occurring after a period of low-to-no activity.

Symptoms and Signs of Palmo-Plantar Pustular Psoriasis

The "fleshy" areas of the hands and feet (base of thumb and/or sides of heels) develop large, yellowish pustules - about the size of a pencil eraser, or .5 centimeters. These Pustules have a studded pattern over reddened patches of skin, and contain non-infectious pus (white blood cells). Throughout the next 7 to 14 days, the Pustule will become smaller, lose it's yellowish color, and become topped with a brown scale of skin. Usually, the disease becomes much less active for a time after peeling.

How is Palmo-Plantar Pustulosis treated?

Palmo-Plantar Pustulosis often proves stubborn to treat. Topical treatments, such as corticosteroids, are usually prescribed first. PUVA, acitretin (Soriatane), methotrexate or cyclosporine (Neoral) sometimes must be used to clear this form. Combination treatment with PUVA and Soriatane (called RePUVA) may also be effective.

Acrodermatitis

Acrodermatitis is a form of Palmo-Plantar Pustulosis which includes painful, potentially disabling, skin lesions on the tips of fingers and sometimes the tips of the toes. The nails may become deformed, and this type of Palmo-Plantar Pustulosis can change and damage the bone in the affected area.

Treatment for Acrodermatitis

Acropustulosis occasionally starts after the skin is injured or infected. This form has traditionally been hard to treat. Tar preparations under occlusion help some patients. Oral retinoid drugs, such as acitretin (Soriatane), may help clear the lesions and restore the nails. PUVA may also be used.

Although considered to be a rare form of the disease, Pustular Psoriasis proves to be one of the most dangerous forms, due to the fact that is may affect the body both internally and externally. You may find the following links useful in learning more related to this type of psoriasis: