PASS syndrome M35.8

PASS is an acronym for Pyoderma gangrenosum, Acne vulgaris, Hidradenitis suppurativa and Ankylosing spondylitis. This constellation is a rare inflammatory disease characterized by a chronic recurrent course of pyoderma gangrenosum, acne vulgaris, hidradenitis suppurativa, and ankylosing spondylitis. 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.

In contrast to other autoinflammatory diseases such as PAPA and PAPASH, no biological or genetic marker has been detected for PASS. In particular, no mutations in the PSTPIP1 gene, could be detected. This raises the suspicion that further mutations in the IL-1 signaling pathway might be causally involved (Leuenberger M et al. 2016).

Therapeutic success has been achieved with the IL-1 receptor antagonist anakinra (Kineret®) and with the IL-17A antagonist secukinumab (Li M et al. 2022). The combination of colchicine and thalidomide was described as effective.

During his disease exacerbation, the patient exhibited febrile episodes along with elevated serum levels of interleukin (IL)-1β. The skin lesions were characterized by sterile neutrophilic infiltrates. The overall symptomatology responded promptly to the IL-1 receptor antagonist anakinra (Kineret®).

1. Antón-Vázquez V et al (2020) Adult-Onset Autoinflammatory Syndromes. J Clin Rheumatol 26: 160-163.
2. Huang J et al (2022) Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome: A case report and literature review. Front Med (Lausanne) 9:856786.
3. Leuenberger M et al (2016) PASS Syndrome: An IL-1-Driven Autoinflammatory Disease. Dermatology 232:254-258.
4. Li M et al. (2022) Secukinumab for PASS syndrome: A new choice for therapeutic challenge? Dermatol Ther 35:e15507.
5. Schwob E et al (2020) PASS: a rare syndrome within the autoinflammatory diseases that still lacks a genetic marker. J Eur Acad Dermatol Venereol 34:e478-e480.