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Demographic, clinical, and scintigraphic comparison of patients affected by palmoplantar pustulosis and severe acne: a retrospective study

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Abstract

Objective

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome encompasses heterogeneous dermatological manifestations, mainly palmoplantar pustulosis (PPP) and severe acne (SA). This study aims to explore the necessity of stratified management according to skin lesions.

Methods

In a cohort of SAPHO patients, we compared the demographic, clinical, and scintigraphic characteristics of the SAPHO patients whose skin lesion was PPP or SA.

Results

A total of 249 patients were included (227 affected by PPP and 22 affected by SA). Patients with SA were younger at onset (20, interquartile ranges (IQR) 15–30 vs. 37, IQR 30–46 years old; p < 0.001) and enrollment (35, IQR 25–38 vs. 41, IQR 33–50 years old; p = 0.001), and they had a prolonged disease duration (88.5 months, IQR 18.7–216.0 vs. 16.0, IQR 7.0–48.0 months; p < 0.001) and time needed for diagnosis (7.5, IQR 2.0–19.0 vs. 1.0, IQR 1.0–4.0 years; p < 0.001). Adjusted by age, sex, and disease duration, SA was significantly associated with more disease-modifying anti-rheumatic drug (DMARD) use (adjusted odds ratio (OR) 3.72; 95% confidence interval (CI) 1.23, 12.62; p = 0.019) and more sternoclavicular joint involvement (adjusted OR 5.91; 95% CI 1.17, 61.3; p = 0.030) in two separate Firth's logistic regression models.

Conclusion

SAPHO patients affected by PPP or SA as the only skin lesion may have different epidemiologic features, osteoarticular manifestations, and treatment history.

Key Points

• SAPHO patients with PPP or SA were heterogenous in both demographic, clinical, and imaging features.

• SAPHO patients with SA were mainly male and had a significantly younger age and longer duration of symptoms before diagnosis.

• SA in SAPHO patients was significantly associated with more sternoclavicular involvement and more DMARD use history.

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References

  1. Nguyen MT, Borchers A, Selmi C, Naguwa SM, Cheema G, Gershwin ME (2012) The SAPHO syndrome. Semin Arthritis Rheum 42(3):254–265. https://doi.org/10.1016/j.semarthrit.2012.05.006

    Article  PubMed  Google Scholar 

  2. Colina M, Govoni M, Orzincolo C, Trotta F (2009) Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a single center study of a cohort of 71 subjects. Arthritis Rheum 61(6):813–821. https://doi.org/10.1002/art.24540

    Article  PubMed  Google Scholar 

  3. Rukavina I (2015) SAPHO syndrome: a review. J Child Orthop 9(1):19–27. https://doi.org/10.1007/s11832-014-0627-7

    Article  PubMed  PubMed Central  Google Scholar 

  4. Misiak-Galazka M, Wolska H, Rudnicka L (2017) What do we know about palmoplantar pustulosis? J Eur Acad Dermatol Venereol 31(1):38–44. https://doi.org/10.1111/jdv.13846

    Article  CAS  PubMed  Google Scholar 

  5. Pettey AA, Balkrishnan R, Rapp SR, Fleischer AB, Feldman SR (2003) Patients with palmoplantar psoriasis have more physical disability and discomfort than patients with other forms of psoriasis: implications for clinical practice. J Am Acad Dermatol 49(2):271–275

    Article  Google Scholar 

  6. Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A (1987) Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases. Rev Rhum Mal Osteoartic 54(3):187–196

    CAS  PubMed  Google Scholar 

  7. Kahn MF, Khan MA (1994) The SAPHO syndrome. Baillieres Clin Rheumatol 8(2):333–362

    Article  CAS  Google Scholar 

  8. Cao Y, Li C, Yang Q, Wu N, Xu P, Li Y, Shi X, Pan Q, Wu X, Sun X, Zhao Y, Gu Z, Yuan S, Zuo Y, Li L, Zhang W, Hao W, Liu J, Jing H, Zhang W (2019) Three patterns of osteoarticular involvement in SAPHO syndrome: a cluster analysis based on whole body bone scintigraphy of 157 patients. Rheumatology (Oxford, England). doi:https://doi.org/10.1093/rheumatology/key415

    Article  Google Scholar 

  9. Li C, Zhao Y, Zuo Y, Zhou Y, Zhang F, Liu S, Zhu Q, Chen J, Zhang W, Xu W, Gu Z, Li L, Li F, Tao W, Cao Y, Sun X, Jing H, Chen H, Zhang S, Dong Z, Liu J, Shi X, Hao W, Qiu G, Zhang W, Wu N, Wu Z (2019) Efficacy of bisphosphonates in patients with synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a prospective open study. Clin Exp Rheumatol

  10. Firth D (1993) Bias reduction of maximum likelihood estimates. Biometrika 80(1):27–38. https://doi.org/10.1093/biomet/80.1.27

    Article  Google Scholar 

  11. Heinze G, Schemper M (2002) A solution to the problem of separation in logistic regression. Stat Med 21(16):2409–2419. https://doi.org/10.1002/sim.1047

    Article  PubMed  Google Scholar 

  12. Vittinghoff E, McCulloch CE (2007) Relaxing the rule of ten events per variable in logistic and cox regression. Am J Epidemiol 165(6):710–718. https://doi.org/10.1093/aje/kwk052

    Article  PubMed  Google Scholar 

  13. Hsieh FY, Bloch DA, Larsen MD (1998) A simple method of sample size calculation for linear and logistic regression. Stat Med 17(14):1623–1634. https://doi.org/10.1002/(sici)1097-0258(19980730)17:14<1623::aid-sim871>3.0.co;2-s

    Article  CAS  PubMed  Google Scholar 

  14. Schilling F, Kessler S (2000) SAPHO syndrome: clinico-rheumatologic and radiologic differentiation and classification of a patient sample of 86 cases. Z Rheumatol 59(1):1–28

    Article  CAS  Google Scholar 

  15. Rohekar G, Inman RD (2006) Conundrums in nosology: synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome and spondylarthritis. Arthritis Rheum 55(4):665–669. https://doi.org/10.1002/art.22087

    Article  CAS  PubMed  Google Scholar 

  16. de Waal AC, van de Kerkhof PC (2011) Pustulosis palmoplantaris is a disease distinct from psoriasis. J Dermatol Treat 22(2):102–105. https://doi.org/10.3109/09546631003636817

    Article  Google Scholar 

  17. Moradi Tuchayi S, Makrantonaki E, Ganceviciene R, Dessinioti C, Feldman SR, Zouboulis CC (2015) Acne vulgaris. Nat Rev Dis Primers 1:15029. https://doi.org/10.1038/nrdp.2015.29

    Article  PubMed  Google Scholar 

  18. Zaenglein AL, Graber EM, Thiboutot DM (2012) Chapter 80. Acne vulgaris and acneiform eruptions. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K (eds) Fitzpatrick's dermatology in general medicine, 8e. The McGraw-Hill Companies, New York

    Google Scholar 

  19. Greywal T, Zaenglein AL, Baldwin HE, Bhatia N, Chernoff KA, Del Rosso JQ, Eichenfield LF, Levin MH, Leyden JJ, Thiboutot DM, Webster GF, Friedlander SF (2017) Evidence-based recommendations for the management of acne fulminans and its variants. J Am Acad Dermatol 77(1):109–117. https://doi.org/10.1016/j.jaad.2016.11.028

    Article  PubMed  Google Scholar 

  20. Hayem G, Bouchaud-Chabot A, Benali K, Roux S, Palazzo E, Silbermann-Hoffman O, Kahn MF, Meyer O (1999) SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum 29(3):159–171. https://doi.org/10.1016/s0049-0172(99)80027-4

    Article  CAS  PubMed  Google Scholar 

  21. Okuno H, Watanuki M, Kuwahara Y, Sekiguchi A, Mori Y, Hitachi S, Miura K, Ogura K, Watanabe M, Hosaka M, Hatori M, Itoi E, Sato K (2018) Clinical features and radiological findings of 67 patients with SAPHO syndrome. Mod Rheumatol 28(4):703–708. https://doi.org/10.1080/14397595.2017.1372874

    Article  PubMed  Google Scholar 

  22. Burden AD, Kemmett D (1996) The spectrum of nail involvement in palmoplantar pustulosis. Br J Dermatol 134(6):1079–1082

    Article  CAS  Google Scholar 

  23. Wang TS, Chiu HY, Hong JB, Chan CC, Lin SJ, Tsai TF (2016) Correlation of IL36RN mutation with different clinical features of pustular psoriasis in Chinese patients. Arch Dermatol Res 308(1):55–63. https://doi.org/10.1007/s00403-015-1611-x

    Article  CAS  PubMed  Google Scholar 

  24. Wise CA, Gillum JD, Seidman CE, Lindor NM, Veile R, Bashiardes S, Lovett M (2002) Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder. Hum Mol Genet 11(8):961–969

    Article  CAS  Google Scholar 

  25. Ferguson PJ, Lokuta MA, El-Shanti HI, Muhle L, Bing X, Huttenlocher A (2008) Neutrophil dysfunction in a family with a SAPHO syndrome-like phenotype. Arthritis Rheum 58(10):3264–3269. https://doi.org/10.1002/art.23942

    Article  CAS  PubMed  Google Scholar 

  26. Del Castillo FJ, Ruiz-Palmero L, Hurtado-Nédélec M, Caniego T, Kahn MF, Gómez-Rosas E, Meyer O, Chollet-Martin S, Hayem G (2014) THU0015 genetic analyses of candidate genes for Sapho syndrome. Ann Rheum Dis 72(Suppl 3):A169.162–A169A169. https://doi.org/10.1136/annrheumdis-2013-eular.543

    Article  Google Scholar 

  27. Li C, Xu H, Wang B (2018) Is SAPHO syndrome linked to PASH syndrome and hidradenitis suppurativa by nicastrin mutation? A case report. J Rheumatol 45(11):1605–1607. https://doi.org/10.3899/jrheum.171007

    Article  PubMed  Google Scholar 

  28. Olivieri I, Padula A, Ciancio G, Salvarani C, Niccoli L, Cantini F (2002) Successful treatment of SAPHO syndrome with infliximab: report of two cases. Ann Rheum Dis 61(4):375–376

    Article  CAS  Google Scholar 

  29. Massara A, Cavazzini PL, Trotta F (2006) In SAPHO syndrome anti-TNF-alpha therapy may induce persistent amelioration of osteoarticular complaints, but may exacerbate cutaneous manifestations. Rheumatology (Oxford) 45(6):730–733. https://doi.org/10.1093/rheumatology/kei221

    Article  CAS  Google Scholar 

  30. Li C, Wu X, Cao Y, Zeng Y, Zhang W, Zhang S, Liu Y, Jin H, Zhang W, Li L (2019) Paradoxical skin lesions induced by anti-TNF-α agents in SAPHO syndrome. Clin Rheumatol 38(1):53–61. https://doi.org/10.1007/s10067-018-4083-5

    Article  PubMed  Google Scholar 

  31. Govoni M, Colina M, Massara A, Trotta F (2009) SAPHO syndrome and infections. Autoimmun Rev 8(3):256–259. https://doi.org/10.1016/j.autrev.2008.07.030

    Article  CAS  PubMed  Google Scholar 

  32. Fitz-Gibbon S, Tomida S, Chiu BH, Nguyen L, Du C, Liu M, Elashoff D, Erfe MC, Loncaric A, Kim J, Modlin RL, Miller JF, Sodergren E, Craft N, Weinstock GM, Li H (2013) Propionibacterium acnes strain populations in the human skin microbiome associated with acne. J Invest Dermatol 133(9):2152–2160. https://doi.org/10.1038/jid.2013.21

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Merola JF (2018) SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. In: Post TW (ed) UpToDate. UpToDate in Waltham, MA

  34. Assmann G, Kueck O, Kirchhoff T, Rosenthal H, Voswinkel J, Pfreundschuh M, Zeidler H, Wagner AD (2009) Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation: an interventional study. Arthritis Res Ther 11(5):R140. https://doi.org/10.1186/ar2812

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Acknowledgements

We authors would like to thank Dr. Dingding Zhang, Central Research Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, and Peking Union Medical College, for his statistical advice.

We authors appreciate the patients recruited in this study.

Funding

This research was funded by National Natural Science Foundation of China (81822030), Chinese Academy of Medical Sciences (CAMS) Initiative Fund for Medical Sciences (2016-I2M-3-003), Chinese Academy of Medical Sciences (CAMS) Initiative for Innovative Medicine (2017-I2M-3-001), Capital Medical Research and Development Fund (2016-4-40112), and National Key Research and Development Program of China (2016YFC0901500).

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Correspondence to Qiyi Li or Li Li.

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The study was approved by the ethics committee of Peking Union Medical College Hospital (ethics documents number: ZS-944) and complied with the 1964 Helsinki declaration ethical standards.

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Li, Y., Li, C., Wu, N. et al. Demographic, clinical, and scintigraphic comparison of patients affected by palmoplantar pustulosis and severe acne: a retrospective study. Clin Rheumatol 39, 1989–1996 (2020). https://doi.org/10.1007/s10067-019-04904-8

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