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

• PASH (Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome)
• PASH Syndrome

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

• Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome (PASH) is a rare disorder that is characterized by the presence of three inflammatory skin conditions - pyoderma gangrenosum, severe acne (acne conglobata), and suppurative hidradenitis
  • Pyoderma gangrenosum is a skin condition characterized by the development of painful and rapidly progressing ulcers affecting almost any surface of the skin
  • Acne is a common skin condition characterized by the presence of pustules and cysts, with blackheads and whiteheads on the face, neck, chest, and back. Acne conglobata is a more severe variant with deep nodules and abscesses
  • Suppurative hidradenitis (or hidradenitis suppurativa) is a chronic skin condition characterized by the development of painful lumps or abscesses in the armpits, groin, and under the breasts
• The genetic defect in Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is not yet identified (as of late 2022). However, a positive family history of the disorder may be a primary risk factor for PASH syndrome 
• The onset of PASH Syndrome is generally noted in older children. The disorder is typically diagnosed based on a combination of clinical examination, blood tests to measure the levels of inflammatory markers, and a biopsy of the pyoderma gangrenosum and hidradenitis suppurativa if indicated
• The treatment of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is typically symptomatic and supportive and may include medications, such as non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and antibiotics, to reduce inflammation and prevent recurrent episodes
• The prognosis is generally good with adequate treatment, and most individuals with Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome can have an improved quality of life. However, without proper treatment, PASH Syndrome may cause complications such as recurrent and persistent skin and chronic systemic inflammation

PAPASH 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, PASS syndrome, and PAC syndrome.

Who gets Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome? (Age and Sex Distribution)

• Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is a rare disorder with onset during or after adolescence
• PAPASH 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, and Suppurative Hidradenitis Syndrome? (Predisposing Factors)

• The primary risk factor for Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is having a 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, and Suppurative Hidradenitis Syndrome? (Etiology)

• Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome (PASH Syndrome) is a genetic disorder of unknown genetic cause; the gene(s) undergoing mutation is not discovered yet
• The abnormal functioning of the inflammatory system leads to chronic skin diseases resulting in severe lesions/ulcers on the skin

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

The signs and symptoms of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome (PASH Syndrome) may include:

• Pyoderma gangrenosum: Formation of large painful ulcers on the legs and other body regions
• Acne conglobata: A severe form of acne that results in the formation of nodules in the body; mostly in the face and neck region
• Suppurative hidradenitis (hidradenitis suppurativa): Chronic and longstanding skin condition characterized by recurrent boil-like lumps at locations where skins rub against each other such as the groin, buttocks, under breasts, and armpits
• The onset of signs and symptoms may be noted in adolescents and older children

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

How is Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome Diagnosed?

Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is typically diagnosed based on a combination of the following:

• Clinical examination and assessment of presenting signs and symptoms
• Evaluation of family history of the condition
• Blood tests to measure the levels of inflammatory markers
• Cultures and biopsies of the skin lesions

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, and Suppurative Hidradenitis Syndrome?

Complications of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome may include:

• Recurrent skin inflammation and ulceration, with pain, in the areas of hidradenitis suppurative and pyoderma gangrenosum
• Scarring of the skin affected by severe acne on the face, shoulders, chest, and back region
• Overall, decreased quality of life

How is Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome Treated?

Treatment for Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is typically symptomatic and supportive and may include medications to reduce inflammation and prevent recurrent episodes such as:

• Non-steroidal anti-inflammatory drugs (NSAIDs)
• Corticosteroids
• Isotretinoin, an oral retinoid medication for acne conglobata
• Dapsone, tetracycline-class, clofazimine, clindamycin, rifampicin, and other antibiotics
• Azathioprine and cyclosporin have been reported to be effective
• Infliximab and anakinra have been found to be useful

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

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

Presently, it is not possible to prevent Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome 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, and Suppurative Hidradenitis Syndrome? (Outcomes/Resolutions)

The prognosis of Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome is generally good with suitable treatment.

• With appropriate treatment, most individuals are known to 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, and Suppurative Hidradenitis Syndrome:

• Suppurative hidradenitis is much more commonly referred to as hidradenitis suppurativa. The order is changed in Pyoderma Gangrenosum, Acne, and Suppurative Hidradenitis Syndrome to make it more easily remembered and pronounced

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

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