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Palmoplantar pustulosis with arthropathy and related inflammatory osteocutaneous conditions require precise classification. Re-evaluating historical groupings like SAPHO syndrome is crucial for understanding complex, multigenic inflammatory disorders.

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Area of Science:

  • Rheumatology
  • Dermatology
  • Genetics

Background:

  • Historically, conditions like palmoplantar pustulosis (PPP) with sternocostoclavicular arthropathy, chronic recurrent multifocal osteomyelitis, acne fulminans, and hidradenitis suppurativa have been described separately.
  • The SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) syndrome was introduced in 1987 to group inflammatory osteocutaneous diseases with unclear pathogenesis.
  • Recent understanding suggests reclassifying certain conditions, such as differentiating hidradenitis suppurativa from acne and recognizing PPP with psoriasis as psoriatic arthropathy.

Purpose of the Study:

  • To review the historical context and evolving understanding of inflammatory osteocutaneous diseases.
  • To propose a shift from 'lumping' diverse conditions under broad syndromes to 'splitting' them for clearer etiological and pathophysiological investigation.
  • To advocate for a more precise classification approach in light of advancements in high-throughput sequencing.

Main Methods:

  • Historical literature review of described osteocutaneous inflammatory conditions.
  • Analysis of diagnostic criteria and proposed classifications over time.
  • Consideration of current dermatological and rheumatological classifications.

Main Results:

  • Palmoplantar pustulosis with sternocostoclavicular arthropathy (Sasaki syndrome) is distinct from broader SAPHO syndrome when other skin manifestations are absent.
  • Hidradenitis suppurativa's pathogenesis aligns with folliculitis, not acne.
  • Palmoplantar pustulosis co-occurring with psoriasis vulgaris is better classified as psoriatic arthropathy.

Conclusions:

  • A move towards 'splitting' rather than 'lumping' clinical findings is recommended for complex inflammatory disorders.
  • Precise classification is essential for deciphering the multigenic and complex inflammatory nature of these conditions.
  • Advancements in genetic sequencing support a more granular approach to understanding these diseases.