(von Zumbusch) acute generalized pustular psoriasis

Von Zumbusch acute generalized pustular psoriasis (GPP) is a rare and severe form of psoriasis characterized by the widespread development of sterile, pus-filled blisters on a background of reddened skin. This condition is also known as acute generalized pustular psoriasis of von Zumbusch, after the dermatologist Leo Ritter von Zumbusch who first described it in the early 20th century. GPP can occur in individuals with no previous history of psoriasis, although it may also arise in those with existing psoriasis.

Symptoms and Signs

The onset of von Zumbusch acute generalized pustular psoriasis is usually rapid, with patients experiencing fever, chills, severe itching, and an increase in heart rate (tachycardia). This is followed by the appearance of pustules that are typically surrounded by red skin. The pustules may coalesce to form lakes of pus that dry and peel off over a period of days, potentially leading to cycles of pustulation and remission. Other symptoms may include fatigue, muscle weakness, and weight loss. Due to the extensive skin involvement, patients are at risk of secondary infections, dehydration, and in severe cases, systemic inflammatory response syndrome (SIRS) which can be life-threatening.

Causes and Risk Factors

The exact cause of von Zumbusch acute generalized pustular psoriasis is unknown, but it is believed to involve a combination of genetic predisposition and environmental triggers. Triggers may include infections, stress, withdrawal from systemic corticosteroids, and certain medications. Genetic studies have identified mutations in the IL36RN gene, which encodes a protein involved in skin inflammation, as a risk factor for GPP.

Diagnosis

Diagnosis of von Zumbusch acute generalized pustular psoriasis is primarily clinical, based on the appearance of the skin and the presence of characteristic pustules. Laboratory tests may show elevated white blood cell counts and elevated inflammatory markers. Skin biopsy can confirm the diagnosis by revealing spongiform pustules of Kogoj and subcorneal pustules of Munro, which are characteristic of the disease.

Treatment

Treatment of von Zumbusch acute generalized pustular psoriasis aims to manage symptoms and prevent complications. Initial management may include hospitalization for fluid replacement, temperature regulation, and monitoring for signs of infection. Systemic treatments include retinoids (such as acitretin), cyclosporine, methotrexate, and biologic agents targeting specific inflammatory pathways, such as tumor necrosis factor (TNF) inhibitors and interleukin inhibitors. Topical treatments, such as corticosteroids and coal tar, may be used adjunctively but are generally insufficient as monotherapy.

Prognosis

The prognosis of von Zumbusch acute generalized pustular psoriasis varies. With prompt and appropriate treatment, patients can experience remission. However, the condition may recur, and chronic GPP can be challenging to manage. Complications such as secondary infections, dehydration, and systemic inflammation can be life-threatening and require immediate medical attention.

See Also

• Psoriasis
• Pustular psoriasis
• Psoriatic arthritis
• Dermatology