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