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

• PASH-AS Syndrome
• PASS (Pyoderma Gangrenosum, Acne Conglobata, Suppurative Hidradenitis, and Axial Spondylarthritis) Syndrome
• Pyoderma Gangrenosum, Acne Conglobata, Suppurative Hidradenitis, and Axial Spondylarthritis

What is Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis? (Definition/Background Information)

• Pyoderma Gangrenosum, Acne Conglobata, Suppurative Hidradenitis, and Axial Spondylitis (PASH-AS or PASS Syndrome) is a rare inflammatory disorder. The exact prevalence of PASH-AS Syndrome is presently unknown. The onset of signs and symptoms may be noted during adolescence or later (into teenage or adulthood)
• PASH-AS Syndrome is characterized by the presence of four conditions, namely pyoderma gangrenosum (causing painful and rapidly progressing ulcers), acne conglobata (causing large acne nodules and cysts), suppurative hidradenitis (forming painful lumps or abscesses of the axilla and groin), and ankylosing spondylitis (inflammation and inflexibility of the spine and other joints)
• Pyoderma Gangrenosum, Acne Conglobata, Suppurative Hidradenitis, and Axial Spondylitis is a genetic disorder caused by mutations in gene(s) that is yet to be identified. No linkage to the “expected” PSTPIP1 gene has been found; PSTPIP1 gene involvement is observed in many pyoderma gangrenosum-associated auto-inflammatory syndromes. The inheritance pattern of PASH-AS is also unknown
• Pyoderma Gangrenosum, Acne Conglobata, Suppurative Hidradenitis, and Axial Spondylitis is diagnosed by the presence of the four distinct conditions, a positive family history, 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 and antibiotics, and infliximab or adalimumab, to reduce inflammation and prevent recurrent episodes. Physical therapy, occupational therapy, and psychological support may also help improve the quality of life
• The prognosis of Pyoderma Gangrenosum, Acne Conglobata, Suppurative Hidradenitis, and Axial Spondylitis is generally good with proper treatment. With adequate treatment, most patients can have an improved quality of life. However, without treatment, PASH-AS Syndrome can cause significant discomfort and pain and result in several complications

PASH-AS 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 Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis? (Age and Sex Distribution)

• Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) is a rare disorder of unknown prevalence with onset in adolescence (or later)
• PASH-AS Syndrome affects both males and females
• Worldwide, all racial and ethnic groups may be susceptible to the disorder

What are the Risk Factors for Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis? (Predisposing Factors)

• Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) is believed to be caused by a genetic mutation, and the main risk factor may be a positive family history of the disorder

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 Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis? (Etiology)

Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) is not caused by mutations in the PSTPIP1 gene, as are the other pyoderma gangrenosum-associated auto-inflammatory syndromes.

• The mutations in the undiscovered gene lead to the development of PASH-AS Syndrome
• An association of the IL-1RN gene is proposed but is yet to be proven
• Even though a family history of the disorder may be noted, its inheritance pattern remains unknown

What are the Signs and Symptoms of Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis?

The main signs and symptoms of Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) may include the following:

• Painful, persistent, and rapidly progressing ulcers (pyoderma gangrenosum)
• Acne nodules and cysts on the face, shoulders, and back, with resultant scarring
• Painful draining and scarring nodules and formation of abscesses (suppurative hidradenitis) in the groin and axilla
• Inflammation and stiffness in the spine and other joints (ankylosing spondylitis)

Understanding each component disease will enhance understanding of PASH-AS Syndrome and its consequences.

How is Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis Diagnosed?

Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) is typically 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
• Medical testing that is necessary to diagnose each of the four conditions comprising the syndrome
• Cultures and biopsies of the skin lesions
• Joint fluid studies, if needed
• Radiological imaging studies as required
• Genetic testing, if possible

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 Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis?

Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) can lead to the following complications:

• Skin scarring
• Severe pain
• Severe arthritis leading to immobility of the lower back and neck
• Reduced quality of life

How is Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis Treated?

Treatment for Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis (PASH-AS) typically includes medications such as non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and antibiotics to reduce inflammation and prevent recurrent episodes.

• Infliximab has been reported to be effective
• Adalimumab, combined with sirolimus or methotrexate, has also been effective
• Physical therapy, occupational therapy, and psychological support may help improve one’s quality of life

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

How can Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis be Prevented?

Presently, it is not possible to prevent Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis as the cause is a genetic mutation.

• 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 Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis? (Outcomes/Resolutions)

The prognosis for Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis is generally good with appropriate treatment. 

• With effective 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 Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis, and Ankylosing Spondylitis:

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

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