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Generalised pustular psoriasis

What is generalised pustular psoriasis?

Generalised pustular psoriasis is a rare form of psoriasis, which presents as widespread pustules on a background of red and tender skin. Widespread patches may occur randomly on any part of the body. It is also known as acute generalised pustular psoriasis of von Zumbusch.

Another form of pustular psoriasis is localised pustular psoriasis, which appears on the hands or feet (palmoplantar pustulosis). This needs to be distinguished from a localised form of generalised pustular psoriasis.

What causes generalised pustular psoriasis?

In most patients no identifiable cause can be found i.e. the generalised pustular psoriasis is idiopathic.

Some people with generalised pustular psoriasis have a preceding history of chronic plaque psoriasis (the common variety in which there are persistent red scaly patches). In many patients no identifiable cause can be found for the altered pattern of disease i.e. the generalised pustular psoriasis is idiopathic. But in some cases the following have been implicated as possible trigger factors:

• Sudden withdrawal of injected or oral corticosteroids
• Drugs such as lithium, salicylates, indomethacin, iodide and some beta-blockers
• Strong, irritating topical preparations such as coal tar, dithranol and strong topical corticosteroids under occlusion
• Infection
• Pregnancy

What are the signs and symptoms?

Initially the skin becomes dry, fiery red and tender. The patient may also have a fever, chills, headache, rapid pulse rate, and loss of appetite, nausea and muscle weakness. Within hours 2-3 mm pustules filled with non-infected pus appear on parts of the body especially the flexures and genital areas. After a day they coalesce to form lakes of pus, which then dry and peel to leave behind a glazed, smooth surface on which new crops of pustules may appear. Successive crops of pustules may appear and erupt every few days or weeks.

Generalised pustular psoriasis

The sudden onset of this condition can be quite alarming. If the patient survives the acute phase and its complications, remission occurs within days or weeks and the psoriasis reverts to its previous state or erythroderma may develop. Relapses are common.

What treatment is available?

Generalised pustular psoriasis can be life threatening so hospitalisation is usually required. The aim is to prevent further fluid loss, stabilise body temperature and restore electrolyte imbalance. Characteristically, there is a low level of calcium in the blood (hypocalcaemia). Other changes on blood testing include low plasma albumin and zinc, high ESR (erythrocyte sedimentation rate), raised neutrophil count, reduced lymphocyte count and raised lactate levels.

Affected areas are treated with bland topical compresses. Antibiotics may be prescribed if infection has occurred. In severe cases or cases where recurrent outbreaks have exhausted the patient, systemic medications are used. These include:

• Oral retinoid such as acitretin, derived from vitamin A
• Sometimes it is necessary to restart corticosteroids, usually temporarily
• Other medications such as methotrexate, colchicine, ciclosporin, tioguanine and hydroxyurea have been used with some success.
• Phototherapy (ultraviolet radiation), especially in combination with oral psoralens (PUVA). This is usually started once the patient has been stabilised on acitretin.

What are the complications?

Death can result from cardiorespiratory failure during the acute eruptive phase so it is very important to treat as early as possible. Elderly patients are at greatest risk. Other complications include:

• Secondary bacterial infection
• Disturbed protein and electrolyte balance, especially low albumin and calcium
• Renal and liver impairment
• Malabsorption of nutrients and therapeutic drugs
• Inflammatory arthritis