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

• PsAPASH (Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa)
• PsAPASH Syndrome

What is Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa? (Definition/Background Information)

• Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PsAPASH) is a rare and severe inflammatory disorder that affects the skin and joints. The disorder typically develops in those with psoriasis, a chronic skin condition characterized by thick, red, scaly patches of skin
• PsAPASH is a multifactorial disorder of unknown cause. The disorder is believed to be associated with an abnormal immune response. The primary risk factor for Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa is psoriasis. Other risk factors include being overweight, smoking, and having a positive family history of the disorder or psoriasis
• The signs and symptoms include joint pain and swelling, skin lesions (psoriasis), painful and rapidly progressing ulcers (pyoderma gangrenosum), severe acne with nodules and cysts on the face, neck, and torso (acne conglobata), and inflammation and abscess formation in the armpits, groin, and other areas where sweat glands are located (hidradenitis suppurativa)
• Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa is diagnosed by the presence of the four distinct conditions, a positive family history of psoriasis, skin lesion culture, and biopsy, imaging scans, and genetic testing, where possible
• The treatment typically includes medications, such as non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, immunosuppressants, and biological agents to reduce inflammation and prevent recurrent episodes. Surgery may be required in some severe cases to manage the skin conditions
• The prognosis of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa may vary from individual to individual, and depends on its severity, one’s response to treatment, and the presence of any complications. With adequate treatment, most individuals can have an improved quality of life

PsAPASH Syndrome is one of the six pyoderma gangrenosum-associated auto-inflammatory syndromes (PGAAIS). This family of diseases includes PAPA syndrome, PASH syndrome, PAPASH syndrome, PsAPASH syndrome, PASH-AS Syndrome (or PASS Syndrome), and PAC syndrome.

Who gets Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa? (Age and Sex Distribution)

• The exact incidence of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PsAPASH) is unknown, but it is a rare disorder
• The syndrome affects both males and females
• Worldwide, all racial and ethnic groups may be susceptible to the disorder

What are the Risk Factors for Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa? (Predisposing Factors)

The main risk factor for Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PsAPASH) is psoriasis. Other risk factors may include:

• Being overweight
• Smoking history
• Having a family history of psoriasis or PsAPASH

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 Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa? (Etiology)

The cause of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PsAPASH) is not fully understood, but it is thought to be related to an abnormal immune response.

• It is a multifactorial disorder, and the exact cause of PsAPASH Syndrome is unknown
• Presently, no specific genetic cause has been discovered/identified

PsAPASH Syndrome typically develops in individuals with psoriatic arthritis. Psoriasis is a chronic skin condition characterized by thick, red, scaly patches of skin. It can be seen in association with psoriatic arthritis, which causes joint destruction.

What are the Signs and Symptoms of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa?

The signs and symptoms of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa (PsAPASH) include:

• Joint pain and swelling of psoriatic arthritis
• Skin lesions of psoriasis
• Painful and rapidly progressing skin ulcers (pyoderma gangrenosum)
• Nodules and cysts of severe acne
• Inflammation and abscess formation in the armpits, groin, and other areas where sweat glands are located (due to hidradenitis suppurativa)
• Other symptoms may include eye inflammation, fever, and fatigue

Understanding each component disease will enhance understanding of PsAPASH Syndrome and its consequences.

How is Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa Diagnosed?

Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa is diagnosed based on a combination of the following:

• Clinical examination and assessment of presenting signs and symptoms; the presence of the four distinct conditions is noted
• Evaluation of family history of the condition (or of psoriasis)
• Blood tests to measure the levels of inflammatory markers
• Cultures and biopsies of the skin lesions
• Joint fluid studies
• Radiological imaging studies (X-rays) as required
• Genetic testing to confirm the presence of the HLA-B27 gene.

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 Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa?

Complications of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa may include the following:

• Joint damage from psoriatic arthritis
• Scarring caused by pyoderma gangrenosum, acne, or hidradenitis suppurativa
• Significant disability
• Overall, decreased quality of life

How is Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa Treated?

Treatment for Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa typically includes medications to reduce inflammation and prevent recurrent episodes such as the following:

• Non-steroidal anti-inflammatory drugs (NSAIDs)
• Corticosteroids
• Immunosuppressants
• Oral retinoids
• Biological agents, such as adalimumab with or without methotrexate, have been reported to be successful

Surgical treatment might be recommended to improve affected joints, repair acne scars, remove scar tracts of hidradenitis suppurativa, or aid in the healing of pyoderma gangrenosum.

Smoking cigarettes is proven to worsen hidradenitis suppurativa (HS). Discontinuing smoking is recommended to improve the course of HS.

How can Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa be Prevented?

Presently, it is not possible to prevent Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa as the cause is a combination of genetic and environmental factors.

• Genetic counseling and testing may be recommended for individuals with a family history of the disorder and couples planning to have children
• However, early diagnosis and appropriate treatment can help manage the symptoms and prevent complications

What is the Prognosis of Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa? (Outcomes/Resolutions)

The prognosis for Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa varies and depends on the severity of the disorder, the individual’s response to treatment, and the presence of any complications.

• With suitable treatment, most patients can have an improved quality of life
• However, if left untreated, the disorder can cause significant discomfort and pain

Additional and Relevant Useful Information for Psoriatic Arthritis, Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa:

The following link is a useful resource for information on other rare conditions on DoveMed:

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