PsA-PASH M02.3

PsA-PASH is an acronym for psoriatic arthritis, pyoderma gangraenosum, acne and hidradenitis suppurativa. This constellation is a rare coincident inflammatory disease characterized by a chronic recurrent course of pyoderma gangrenosum, acne vulgaris, hidradenitis suppurativa and psoriatic arthritis.

To date, several clinically distinct syndromes of this group of forms have been described in the literature, including:

• PAPA: Pyoderma gangraenosum, acne and pyogenic arthritis.
• PASH: pyoderma gangrenosum, acne and hidradenitis suppurativa
• PASS: Pyoderma gangrenosum, acne, and spondyloarthritis
• PA-PASH: pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa
• PsA-PASH: psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa
• PAC: Pyoderma gangrenosum, acne and ulcerative colitis

Furthermore, the group of autoinflammatory diseases associated with neutrophilic dermatoses includes the SAPHO syndrome(synovitis, acne, pustulosis, hyperostosis, osteitis), which should be considered as a superordinate umbrella syndrome. It is important to raise awareness of the group of autoinflammatory diseases to optimize disease management and ultimately improve patients' quality of life.nd ultimately improve patients' quality of life.

Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne and/or arthritis , are rare inflammatory disorders with a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)-1 signaling leading to sterile neutrophilic inflammation in various organs. The clinical features are recurrent fevers, painful (poly) arthritis, and neutrophilic dermatoses (hidradenitis suppurativa, acne, and pyoderma gangraenosum) accompany.

Elevated systemic inflammatory markers in the blood

The rarity of these syndromes complicates the establishment of evidence-based treatment guidelines. Anti-TNF-α and anti-IL-1a blockade are among the therapeutic approaches that improve skin symptoms and prevent permanent osteoarticular damage. Success has been achieved with the IL-17A inhibitor secukinumab (Nikolakis G et al. 2021) .

Both pyoderma gangrenosum and hidradenitis suppurativa belong to the spectrum of neutrophilic dermatoses and are considered autoinflammatory syndromes. They share similar mechanisms from a pathophysiologic perspective, including neutrophil-rich cutaneous inflammation and overexpression of the interleukin-1 (IL-1) family.

1. Antón-Vázquez V et al (2020) Adult-Onset Autoinflammatory Syndromes. J Clin Rheumatol 26: 160-163.
2. Garcovich S et al (2021) PASH, PAPASH, PsAPASH, and PASS: The autoinflammatory syndromes of hidradenitis suppurativa. Clin Dermatol 39:240-247.
3. Huang J et al (2022) Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome: A case report and literature review. Front Med (Lausanne) 9:856786.
4. Leuenberger M et al (2016) PASS Syndrome: An IL-1-Driven Autoinflammatory Disease. Dermatology 232:254-258.
5. Li M et al. (2022) Secukinumab for PASS syndrome: A new choice for therapeutic challenge? Dermatol Ther 35:e15507.
6. Nikolakis G et al. (2021) Case Report: PsAPSASH syndrome: an alternative phenotype of syndromic hidradenitis suppurativa treated with the IL-17A inhibitor secukinumab. F1000 Res 10:381.
7. Vinkel C et al (2017) Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne. Int J Dermatol 56:811-818.