The pustules arenot contagiousbut are the result of sudden and extremeautoimmuneinflammation.
Von Zumbusch can develop at any age but predominately affects adults over 50.
This article goes over the symptoms and causes of this rare condition.
Symptoms of Von Zumbusch Psoriasis
Von Zumbusch can appear abruptly on the skin.
The symptoms start with widespread areas of discolored skin that become increasingly painful and tender.
On lighter skin tones, the patches may appear to be pink or red.
On darker skin tones, they may look violet.
Within hours, tiny pustules appear, many of which will consolidate into larger blisters.
The pustules can easily rupture when scratched or abraded.
Fever and fatigue are common.
Within 24 to 48 hours, the pustules will dry up, leaving a glazed and smooth appearance.
The affected skin can be extremely itchy and peel away in sheets.
This is not only painful but can lead to the rapid loss of moisture and massivedehydration.
Recurrent flares are not uncommon.
Children rarely get von Zumbusch psoriasis.
What Causes Von Zumbusch Psoriasis?
As an autoimmune disease, psoriasis is characterized by an immune system gone awry.
For reasons poorly understood, theimmune systemwill suddenly regard normal skin cells as harmful and launch aninflammatory attack.
With pustular psoriasis, the autoimmune response is even more severe.
Scientists believe that it is because of a defect in an inflammatory protein known asinterleukin.
What results is a massive burst of inflammation that kills many of thewhite blood cellsinvolved in the autoimmune assault.
The dead cells combined with lymph fluid create what the white, gooey substance recognized aspus.
What Triggers Von Zumbusch Psoriasis?
How Is Von Zumbusch Psoriasis Diagnosed?
There are no lab tests or imaging studies that can definitively diagnose psoriasis.
The diagnosis of Von Zumbusch starts with a physical examination and a review of your medical history.
Your healthcare provider may also want to obtain a pus sample for evaluation in the lab.
These include:
How Do You Treat Von Zumbusch Psoriasis?
Von Zumbusch psoriasis can be life-threatening and requires immediate medical care.
This is most often delivered in a hospital where rehydration and supportive care are provided.
The treatment protocol is similar to that of a severe burn.
To help temper the acute flare, anoral retinoidcalled Soriatane (acitretin) is commonly prescribed.
Retinoids slow the hyperproduction of skin cells and help bring the acute symptoms under control.
Severe cases may require immunosuppressant drugs (methotrexate, cyclosporine) or thebiologic drugRemicade (infliximab).
The blisters can rupture and dry, leaving a flaky crust behind.
Dehydration is a complication of von Zumbusch psoriasis and can become severe.
Like other forms of psoriasis, von Zumbusch psoriasis is an autoimmune disorder.
See a healthcare provider or dermatologist if you think you may have von Zumbusch psoriasis.
Von Zumbusch psoriasis can be life-threatening, but treatments are available.
Prevent flares by taking actions like avoiding triggers.
American Academy of Dermatology Association.Can you get psoriasis if you have skin of color?
a review of clinical features and new therapeutics.Burns.2014;40(4):e35e39.
