Author: Vanessa Ngan, Staff Writer, 2003. Updated by Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, September 2014.
Generalised pustular psoriasis is a rare and serious skin disorder that presents with flares of widespread sterile pustules on a background of red and tender skin. It is also known as acute generalised pustular psoriasis of von Zumbusch.
Related pustular disorders include:
Generalised pustular psoriasis is an inflammatory skin disease. Recent research suggests generalised pustular psoriasis is distinct from psoriasis, with a different pattern of immune activation. Generalised pustular psoriasis has been associated with abnormalities in the cytokine (messenger protein) interleukin -36-receptor-antagonist signalling. This is due to recessive IL36RN gene mutations. These mutations are also found in some patients with AGEP, palmoplantar pustulosis and acrodermatitis continua of Hallopeau.
About 10% of patients with generalised pustular psoriasis have a preceding history of psoriasis, in which there are persistent, circumscribed, red and scaly plaques. In these patients, CARD14 gene gain-of-function abnormalities have been reported.
Possible trigger factors for flares of generalised pustular psoriasis may include:
Generalised pustular psoriasis sometimes arises in pregnancy. Previously known as impetigo herpetiformis, this name is no longer favoured because the eruption has nothing to do with either impetigo (a bacterial infection) or herpes simplex (a viral infection).
Strong, irritating topical preparations such as coal tar, dithranol and withdrawal of strong topical corticosteroids can lead to localised areas of pustulosis, often associated with existing plaques of psoriasis.
Generalised pustular psoriasis is characterised by recurrent acute flares.
Flares of generalised pustular psoriasis may result in:
Remission occurs within days or weeks and the skin reverts to its previous state or erythroderma may develop. Relapses are common.
Generalised pustular psoriasis is often suspected clinically by experienced dermatologists. However, the following tests are usually undertaken to confirm the diagnosis and to identify potential complications.
Death can result from cardiorespiratory failure during the acute eruptive phase of generalised pustular psoriasis, so it is essential to treat it as early as possible. Older patients are at highest risk. Other complications include:
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. Specialist care is essential.
Systemic medications may include:
Anti-IL36 monoclonal antibodies are being evaluated for their effectiveness in generalised pustular psoriasis.
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