Generalized pustular psoriasis
What is it?
Generalized pustular psoriasis, is a rare type of psoriasis characterized by white, pus-filled blisters (pustules). The pustules are not contagious but are the result of sudden and extreme autoimmune inflammation. It differs from the two other types of pustular psoriasis, which are generally limited to the hands or feet, and is considered far more serious.
Von Zumbusch can develop at any age but predominately affects adults over 50. The outbreak of symptoms, referred to as a flare, may be triggered by any number of things, including medications and stress. Diagnosed by a physical exam and the evaluation of a pus sample, Von Zumbusch almost always requires treatment in a hospital.
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Generalized pustular psoriasis.
Additional names
This group contains additional names:
- Von Zumbusch psoriasis
Signs & symptoms
Von Zumbusch can appear abruptly on the skin. The symptoms start with widespread areas of reddened skin that become increasingly painful and tender. 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 massive dehydration.
Symptoms of severe dehydration can include:
* Extreme thirst
* Dry mouth
* Headaches
* Reduced urination
* Dark-colored urine
* Fatigue
* Sleepiness
* Dizziness
* Confusion
* Muscle weakness
* Rapid heart rate
* Rapid, shallow breathing
* Fever and chills
* Leg swelling
* Unconsciousness
If not treated immediately, severe dehydration can lead to cerebral edema (brain swelling), seizures, shock, acute kidney failure, cardiac failure, coma, and death.
Diagnosis
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, a differential diagnosis would be performed to differentiate Von Zumbusch psoriasis from other diseases with similar features. These include:
* Impetigo
* Dermatitis herpetiformis
* Bacterial septicemia
* Secondary infection of generalized atopic dermatitis or seborrheic dermatitis
* Immunoglobulin A (IgA) pemphigus, an autoimmune blistering disorder
Treatment
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:
* Prevent fluid loss
* Stabilize the body's temperature
* Restore the skin's chemical balance
In addition to intravenous (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, an oral retinoid called 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 the biologic drug 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.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
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