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Does SAPHO syndrome exist in dermatology?
1Department of Dermatology and Allergy, Technical University of Munich, Munich, Germany.
Summary
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.
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.
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