Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrevention

Table of ContentsView All

View All

Table of Contents

Symptoms

Causes

Diagnosis

Treatment

Prevention

This article goes over the symptoms and causes of this rare condition. It also explains how healthcare providers diagnose and treat von Zumbusch psoriasis and what you can do to prevent it.

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.

Symptoms of severe dehydration can include:

If not treated immediately, severe dehydration can lead to cerebral edema (brain swelling), seizures, shock,acute kidney failure,cardiac failure, coma, and death.

According to a 2016 review in the journalPsoriasis,no less than 65% of adults with pustular psoriasis will have had a prior diagnosis of plaque psoriasis. Recurrent flares are not uncommon.

Children rarely get von Zumbusch psoriasis. When they do, it is often their first psoriatic event and tends to be less severe than what is seen with adults.

What Causes Von Zumbusch Psoriasis?

If one of the two molecules is defective, it can leave inflammation on “high” with no way to turn it off. 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?

The underlying mechanisms of psoriasis have yet to be identified, but scientists believe that a combination ofgenetics(that predispose you to the disease) and environmental triggers (that actualize the disease) is involved.

With von Zumbusch psoriasis, there are certain specific triggers known to incite a flare:

Despite the plethora of common triggers, most cases of pustular psoriasis areidiopathic, meaning of no known cause.

Medications That Can Trigger 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.

Although the appearance of pustules may be a giveaway, the healthcare provider will look for other clues to confirm the suspicion, including a prior history of plaque psoriasis, a family history of autoimmune disease, or recent exposure to specific medications.

If the results are inconclusive, adifferential diagnosiswould be performed to differentiate von Zumbusch psoriasis from other diseases with similar features. These include:

How Psoriasis Is Diagnosed

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.

The aims of treatment are to:

In addition tointravenous (IV) fluids, topical, oral, or IV antibiotics may be used to prevent secondary infections.

Supportive therapy with cool compresses and saline or oatmeal baths can help soothe the skin and gently remove (debride) dead skin.

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).

Once the acute crisis has passed, psoralen and ultraviolet A (PUVA)—a type of phototherapy—may be used to bring the condition into sustained remission.

How Psoriasis Is Treated

Prevention of Von Zumbusch Psoriasis

There is really no way to prevent von Zumbusch psoriasis given that so little is known about the underlying causes. However, if you have had a previous flare, you are at risk of recurrence and need to take preventive action. This should include:

10 Psoriasis Self-Care Practices You Can Do at Home

Summary

2 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Dermatology Association.Can you get psoriasis if you have skin of color?Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B.Pustular psoriasis: pathophysiology and current treatment perspectives.Psoriasis (Auckl). 2016;6:131-144. doi:10.2147/PTT.S98954Additional ReadingVarman K, Namias N, Schulman C, et al.Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. a review of clinical features and new therapeutics.Burns.2014;40(4):e35–e39. doi:10.1016/j.burns.2014.01.003

2 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Dermatology Association.Can you get psoriasis if you have skin of color?Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B.Pustular psoriasis: pathophysiology and current treatment perspectives.Psoriasis (Auckl). 2016;6:131-144. doi:10.2147/PTT.S98954Additional ReadingVarman K, Namias N, Schulman C, et al.Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. a review of clinical features and new therapeutics.Burns.2014;40(4):e35–e39. doi:10.1016/j.burns.2014.01.003

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

American Academy of Dermatology Association.Can you get psoriasis if you have skin of color?Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B.Pustular psoriasis: pathophysiology and current treatment perspectives.Psoriasis (Auckl). 2016;6:131-144. doi:10.2147/PTT.S98954

American Academy of Dermatology Association.Can you get psoriasis if you have skin of color?

Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B.Pustular psoriasis: pathophysiology and current treatment perspectives.Psoriasis (Auckl). 2016;6:131-144. doi:10.2147/PTT.S98954

Varman K, Namias N, Schulman C, et al.Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. a review of clinical features and new therapeutics.Burns.2014;40(4):e35–e39. doi:10.1016/j.burns.2014.01.003

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