What are the other Names for this Condition? (Also known as/Synonyms)

• Generalised Pustular Psoriasis
• GPP (Generalized Pustular Psoriasis)

What is Generalized Pustular Psoriasis? (Definition/Background Information)

• Generalized Pustular Psoriasis (GPP) is a severe and sometimes life-threatening form of psoriasis, a common chronic inflammatory skin disease. Psoriasis occurs due to a genetic defect, which causes the skin to grow faster than normal, usually resulting in white scaly patches and plaques on the skin
• In Generalized Pustular Psoriasis, the affected individuals experience episodes of red, swollen, and tender skin with widespread pus-containing blisters (pustules) all over their body. These are accompanied by fever and chills, headaches, appetite loss, nausea, and tiredness
• The cause of formation of Generalized Pustular Psoriasis is unknown in a majority of cases. However, in some, it may be caused by mutations involving the IL36RN gene. In such cases, GPP may be inherited in an autosomal recessive manner. CARD 14 Mediated Pustular Psoriasis is a rare well-defined  genetic subset of GPP, usually with onset in childhood
• Since Generalized Pustular Psoriasis can be a medical emergency, it normally necessitates hospitalization and appropriate management, using oral and topical agents, skin-softeners, and steroid creams. Systemic therapy is usually required. In some individuals, the problem can resolve after a few days or weeks. Nevertheless, the condition is known to recur

Who gets Generalized Pustular Psoriasis? (Age and Sex Distribution)

• Generalized Pustular Psoriasis is a rare disorder that generally affects adults
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Generalized Pustular Psoriasis? (Predisposing Factors)

The risk factors for Generalized Pustular Psoriasis may include:

• Preexistent psoriasis
• A family history of the condition, since some cases of the disorder may be inherited
• Sudden withdrawal of topical, oral, or injected corticosteroids
• Use of certain drugs such as aspirin and beta blockers
• Infections
• Pregnancy, in rare cases

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Generalized Pustular Psoriasis? (Etiology)

In many cases, the cause of Generalized Pustular Psoriasis may not be known, and the condition develops sporadically. Less frequently, Generalized Pustular Psoriasis may be caused by:

• Sudden withdrawal of corticosteroids (oral, injected, or strong topical)
• Withdrawal from efalizumab (brand name Raptiva) an injectable psoriasis medication no longer marketed, commonly caused a pustular flare of psoriasis
• Use of medications such as:
  • Aspirin
  • Some beta blockers including atenolol, cetamolol, metoprolol, nadolol, oxprenolol, practolol, propranolol, and timolol
  • Iodide containing medicines
  • Lithium
  • Non steroidal anti-inflammatory drugs, such as indomethacin
• Mutation(s) in the IL36RN gene, inherited in an autosomal recessive manner, are known to be causative

Autosomal recessive inheritance: Autosomal recessive conditions are traits or disorders that occur when two copies of an abnormal gene have been inherited on a non-sex chromosome. If both parents have an autosomal recessive condition, there is a 100% likelihood of passing on the mutated genes to their children. If, however, only one mutant copy of the gene is inherited, the individual will be a carrier of the condition, but will not be present with any symptoms. Children born to two carriers, have a 25% chance of being homozygous dominant (unaffected), a 50% chance of being heterozygous (carrier), and a 25% chance of being homozygous recessive (affected).

What are the Signs and Symptoms of Generalized Pustular Psoriasis?

The signs and symptoms of Generalized Pustular Psoriasis may vary among affected individuals in type and severity. These may include:

• Reddish discoloration of skin
• Pus-filled blisters (pustules) all over the body
• Fever and chills
• Headache
• Rapid heartbeat
• Nausea and loss of appetite
• Weakness of muscles
• Furrowed tongue
• Nail abnormalities

How is Generalized Pustular Psoriasis Diagnosed?

Generalized Pustular Psoriasis is diagnosed on the basis of the following information:

• Complete physical examination and thorough medical history evaluation
• Assessment of the presenting signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Generalized Pustular Psoriasis?

The complications of Generalized Pustular Psoriasis may include:

• Anemia
• Electrolyte imbalance
• Malabsorption of nutrients and drugs
• Kidney and liver malfunction
• Secondary bacterial infections
• Erythroderma (reddening of skin) following remission
• Relapse

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Generalized Pustular Psoriasis Treated?

The treatment for Generalized Pustular Psoriasis (GPP) is tailored to reduce fluid loss, restore electrolyte balance, and limit secondary infections. It generally requires hospitalization. The treatment methods may include:

• Application of low potency corticosteroids to affected areas
• Immune-suppressant drugs such as cyclosporin
• Oral retinoid medication acitretin
• Antibiotics to address infection
• Tumor necrosis factor (TNF) blockers, such as adalimumab, infliximab, and etanercept
• Interleukin or interleukin receptor antagonists, such as ustekinumab and anakinra
• Administration of colchicine medication
• Chemotherapeutic agents such as methotrexate
• Systemic corticosteroids, as necessary
• Spesolimab-sbzo is an intravenous treatment developed specifically for GPP. It is FDA approved for use in adults over 18 years of age

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

How can Generalized Pustular Psoriasis be Prevented?

Generalized Pustular Psoriasis (GPP) may not be preventable if the cause is genetic.

• If an individual already has psoriasis and develops early signs and symptoms of GPP, seeking immediate medical attention may help arrest a progression of the condition
• Similarly, those who are under medication(s) that has the potential to trigger GPP may benefit from getting educated on identifying triggers, so that they can recognize the signs and symptoms promptly

What is the Prognosis of Generalized Pustular Psoriasis? (Outcomes/Resolutions)

The prognosis of Generalized Pustular Psoriasis is dependent upon the severity of the signs and symptoms and associated complications, if any.

• In most cases, remission is reported to occur within a few days or weeks, although the condition can recur
• Generalized Pustular Psoriasis can be life-threatening, and hospitalization is generally necessary

Additional and Relevant Useful Information for Generalized Pustular Psoriasis:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/