Von Zumbusch psoriasis is caused by sudden, severe autoimmune inflammation

Von Zumbusch disease or acute generalized pustular psoriasis is a rare and life-threatening type of psoriasis characterized by pus-filled pustules. The pustules are not contagious and generally limited to the hands and feet. 

Von Zumbusch psoriasis can develop at any age but mostly affects adults over age 50. It is caused by sudden, severe autoimmune inflammation.

What are the symptoms of Von Zumbusch disease?

Von Zumbusch psoriasis can develop suddenly and initially appear as widespread red, painful, tender, skin blotches. These blotches are followed by tiny pustules that combine to form large blisters. These pustules rupture easily when scratched. 

Within 24-48 hours, the pustules tend to dry up and leave a smooth, glazed appearance. The skin will be itchy and start to peel off. This can lead to rapid loss of moisture and dehydration.

Symptoms of Von Zumbusch disease include:

• Peeling skin all over the body
• Fever and chills
• Intense itching
• Rapid heart rate
• Shallow breathing
• Dehydration 
• Fatigue
• Headache
• Nausea
• Muscle weakness
• Joint pain
• Weight loss

What causes Von Zumbusch disease?

Generally, psoriasis develops when the immune system is overactive and produces an excess amount of inflammatory agents called cytokines. Cytokines usually help the body fight infections and heal injuries. But with autoimmune diseases, cytokines attack healthy tissue instead of concentrating on fighting off an infection.

Due to the inflammatory attack, skin cell production is accelerated, causing cells to accumulate on the skin surface and form patches of scaly lesions. With pustular psoriasis, the autoimmune response is even more severe. 

Von Zumbusch disease is usually caused due to a defect in an inflammatory protein called interleukin. Generally, there are matched sets of interleukins, with one that promotes inflammation and one that reduces inflammation. Defective interleukin cells can trigger an autoimmune response and result in a massive burst of inflammation.

Some of the triggers that can lead to Von Zumbusch disease include:

• Emotional stress
• Medications such as:
  • Salicylates 
  • Lithium
  • Penicillin
  • Interferon
  • Terbinafine
• Abrupt discontinuation of corticosteroid drugs
• Skin infections (Staphylococcus aureus and Streptococcus epidermidis)
• Excessive sun exposure
• Phototherapy
• Hypocalcemia (low blood calcium)
• Hormonal changes
• Pregnancy, especially during the third trimester
• Bacille Calmette-Guerin vaccination for tuberculosis
• Irritating topical medications (coal tar or anthralin)

Psoriasis causes the top layer of skin cells to become inflamed and grow too quickly and flake off.
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How is Von Zumbusch disease treated?

Because Von Zumbusch disease is a life-threatening disease, it requires immediate medical attention. The aim of the treatment is to:

• Prevent the loss of fluid
• Stabilize body temperature
• Restore the skin’s protective function 
• Correct electrolyte imbalances

Medications commonly used for treating Von Zumbusch disease include:

• Oral retinoids such as acitretin
• Immunosuppressant drugs such as methotrexate
• Biologic drugs such as infliximab
• Antibiotics for suspected infections

Can Von Zumbusch disease be prevented?

Although Von Zumbusch psoriasis cannot necessarily be prevented, you can reduce the risk of recurrence with the following measures:

• Losing weight if you are obese
• Eating healthy by following the Mediterranean or gluten-free diet
• Taking a nutritional supplement such as fish oil or vitamin D
• Limiting alcohol intake
• Quitting smoking
• Getting natural sunlight exposure for 15-20 minutes a day
• Avoiding over-the-counter pain medication such as ibuprofen and naproxen, as they can exacerbate symptoms