The PASH syndrome (pyoderma gangrenosum, acne, and hidradenitis suppurativa / acne inversa) has recently been described in few unrelated patients as a new entity within the spectrum of hidradenitis suppurativa / acne inversa syndromes. PASH syndrome is similar to PAPA syndrome but differs insofar as it lacks the associated arthritis and has a different genetic basis, since no mutations have yet been detected in PASH syndrome.

  • Specific challenges associated with the recognition of the condition

Clinical features may help to make a diagnosis.

  • Specific challenges associated with the diagnosis

No genetic test widely available yet. Mutations have not been detected in all cases.

  • Specific challenges associated with the treatment

No drug registered. No clinical studies performed.

  • Specific challenges associated with care of these patients over their lifespan – Quality of life issues – Gaps accross the care

Skin scarring due to severe acne, pyoderma gangraenosum and hidradenitis suppurativa / acne inversa is prominent.

Incidence : unknown

Prevalence : unknown

 

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