CARD 14 Mediated Pustular Psoriasis

CARD 14 Mediated Pustular Psoriasis

Article
Skin Care
Diseases & Conditions
+1
Contributed byLester Fahrner, MD+1 moreSep 30, 2023

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

  • CAMP Psoriasis
  • CARD 14-Associated Psoriasis
  • Familial Psoriasis (PSORS2)

What is CARD 14 Mediated Pustular Psoriasis? (Definition/Background Information)

  • CARD 14 Mediated Pustular Psoriasis (CAMP Psoriasis) is a rare and severe form of pustular psoriasis characterized by the presence of pustules and erythema. The pustules are generally small and are filled with white blood cells without bacterial involvement. CAMP Psoriasis is a rare well-defined genetic subset of Generalized Pustular Psoriasis (GPP)
  • CARD 14 Mediated Pustular Psoriasis is a rare form of psoriasis generally seen in adults, although it can affect individuals of any age range. A positive family history of psoriasis and having a mutation in the CARD14 gene are the main risk factors for developing CAMP Psoriasis
  • The exact cause of CARD 14 Mediated Pustular Psoriasis is not known, but it is related to genetic and environmental factors. This form of pustular psoriasis is related to mutations in the CARD14 gene, which is responsible for the regulation of the IL-1 pathway
  • The signs and symptoms of CARD 14 Mediated Pustular Psoriasis include the sudden development of pustules on the skin, often accompanied by fever, chills, and a rapid heartbeat. Other symptoms may include redness and scaling of the skin
  • The condition is diagnosed by a dermatologist based on the appearance of the skin and a physical examination. A skin biopsy may also be performed to confirm the diagnosis. Genetic testing will be used to confirm the diagnosis of PSOR2 from other, non-specific forms of pustular psoriasis, and pustular psoriasis (15)
  • The complications of CARD 14 Mediated Pustular Psoriasis can include dehydration, electrolyte imbalances, and secondary infections. In severe cases, the condition can lead to sepsis and even necessitate hospitalization
  • The treatment typically involves using IL-1 inhibitors, such as anakinra and canakinumab, as well as topical medications such as corticosteroids and coal tar. In some cases, oral retinoids, phototherapy, or biologic therapy may be recommended
  • Currently, there are no known measures to prevent CARD 14 Mediated Pustular Psoriasis. However, avoiding triggers and managing stress can help reduce the risk of flare-ups
  • The prognosis for individuals with CARD 14 Mediated Pustular Psoriasis is generally good with early diagnosis and treatment. However, the condition can be chronic and may recur

Who gets CARD 14 Mediated Pustular Psoriasis? (Age and Sex Distribution)

  • CARD 14 Mediated Pustular Psoriasis is a rare form of pustular psoriasis. It is seen in adults, but the onset can be during infancy or childhood
  • Most reported cases have had European or Asian ancestry
  • Patients have been reported from various geographical regions such as the US, EU, Canada, Haiti, and Taiwan

What are the Risk Factors for CARD 14 Mediated Pustular Psoriasis? (Predisposing Factors)

The main risk factors for developing CARD 14 Mediated Pustular Psoriasis include:

  • Having a family history of psoriasis
  • Having a mutation in the CARD14 gene

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 CARD 14 Mediated Pustular Psoriasis? (Etiology)

The exact cause of CARD 14 Mediated Pustular Psoriasis is unknown, but it is related to genetic and environmental factors.

  • It is mediated by the activation of IL-1 beta and IL-1 receptor antagonist (IL-1Ra). 
  • This type of pustular psoriasis is related to mutations in the CARD14 gene, which is responsible for regulating the IL-1 pathway

What are the Signs and Symptoms of CARD 14 Mediated Pustular Psoriasis?

The signs and symptoms of CARD 14 Mediated Pustular Psoriasis include:

  • Sudden development of pustules on the skin, which are often accompanied by fever, chills, and a rapid heartbeat
  • Redness of the skin
  • Scaling of the skin

How is CARD 14 Mediated Pustular Psoriasis Diagnosed?

CARD 14 Mediated Pustular Psoriasis is diagnosed by a dermatologist based on the appearance of the skin and a physical examination.

  • A skin biopsy may also be performed to confirm the diagnosis
  • Genetic testing can confirm the abnormality of the CARD 14 gene to differentiate the patient’s disease from pustular psoriasis with other intrinsic causes, such as pustular psoriasis (15), and the majority of cases with as-yet-undefined defect

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 CARD 14 Mediated Pustular Psoriasis?

Complications of CARD 14 Mediated Pustular Psoriasis can include:

  • Dehydration
  • Electrolyte imbalances
  • Secondary infections
  • The distress and discomfort of significant skin disease
  • Sepsis
  • Needing hospitalization in severe cases
  • Recurrence of the condition

How is CARD 14 Mediated Pustular Psoriasis Treated?

  • The treatment of CARD 14 Mediated Pustular Psoriasis typically involves the use of IL-1 inhibitors, such as anakinra and canakinumab
  • Topical medications, such as corticosteroids and coal tar, may be prescribed by the healthcare professional
  • In some cases, oral retinoids, phototherapy, or biologic therapy may be recommended
  • Spesolimab-sbzo is an intravenous treatment developed specifically for Generalized Pustular Psoriasis. It is FDA approved for use in adults over 18 years of age

How can CARD 14 Mediated Pustular Psoriasis be Prevented?

  • Presently, there are no known measures to prevent CARD 14 Mediated Pustular Psoriasis
  • However, avoiding triggers and managing stress can help reduce the risk of flare-ups

What is the Prognosis of CARD 14 Mediated Pustular Psoriasis? (Outcomes/Resolutions)

  • The prognosis for individuals with CARD 14 Mediated Pustular Psoriasis is generally good with early diagnosis and treatment
  • However, the condition can be chronic and may recur

Additional and Relevant Useful Information for CARD 14 Mediated Pustular Psoriasis:

The following link is a useful resource for information on psoriasis:

https://www.dovemed.com/diseases-conditions/psoriasis/

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On the Article

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team
Lester Fahrner, MD picture
Author

Lester Fahrner, MD

Chief Medical Officer, DoveMed Team

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