SAPHO Syndrome from Hidradenitis Suppurativa Veesta Falahati, Msc, MD and Paul B

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JGIM CLINICAL PRACTICE Clinical Images SAPHO Syndrome from Hidradenitis Suppurativa Veesta Falahati, MSc, MD and Paul B. Aronowitz, MD Department of Internal Medicine, University of California, Davis Medical Center, Sacramento, CA, USA. KEY WORDS: clinical image; dermatology; diagnosis; rheumatology. SAPHO syndrome is a poorly understood inflammatory – disorder thought to be an autoimmune reaction provoked by J Gen Intern Med 35(4):1307 8 2 DOI: 10.1007/s11606-019-05131-2 an indolent inflammatory process (in this case, HS). The © Society of General Internal Medicine 2020 acronym SAPHO represents the variable presence of synovitis, acne, pustulosis, hyperostosis, and osteitis seen in this syndrome.3 The most common skin lesion is palmoplantar pustulosis. Osteoarticular manifestations include osteitis, hyperostosis synovitis, arthropathy, and enthesopathy.3 Involve- 36-year-old man with 10 years of hidradenitis ment of bone and joints of the anterior chest wall is felt to be A suppurativa (HS) presented with worsened HS and joint highly characteristic. Treatment of SAPHO is variable de- pain in both hands after stopping adalimumab therapy 1 year pending on the case presentation. earlier. Examination revealed a temperature of 38.2 °C, HS lesions draining purulent material over the chest (Fig. 1), and erosions in bilateral proximal interphalangeal joints (Fig. 2). His white blood cell count was 21,000/μL and hand radio- graphs were consistent with inflammatory arthropathy. He received a diagnosis of hidradenitis suppurativa with superimposed cellulitis in the setting of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Treatment included antibiotics and wound care. He resumed adalimumab and started doxycycline for long-term suppres- sive therapy.1 He failed to improve, and his treatment was escalated from adalimumab to secukinumab. Figure 2 Hand radiograph. Left hand: erosions and periosteal Figure 1 Patient’s chest with nodules and sinus tracts draining reaction centered at the 4th proximal interphalangeal joint (arrow) purulent material. with soft tissue swelling. Received August 31, 2018 Revised January 11, 2019 Accepted May 22, 2019 , Published online February 19 2020 1307 1308 Falahati et al.: SAPHO Syndrome from Hidradenitis Suppurativa JGIM REFERENCES Corresponding Author: Veesta Falahati, MSc, MD; Department of Internal Medicine University of California, Davis Medical Center, 1. Jemec, GBE. MD Hidradenitis Suppurativa. N Engl J Med 2012;366:158– 4150 V Street Suite 1100, Sacramento, CA 95817, USA 64. (e-mail: [email protected]). 2. Hurtado-Nedelec M, Chollet-Marti S et al. Characterization of the immune response in the synovitis, acne, pustolosis, hyperostosis, osteitis (SAPHO) syndrome. Rheumatology 2008;47:1160–7. Compliance with Ethical Standards: 3. Firinu D, Garcial-Larsen V et al. SAPHO Syndrome: Current Develop- ments and Approaches to Clinical Treatment. Curr Rheum Rep Conflict of Interest: The authors declare that they do not have a 2016;18:35. conflict of interest. Publisher’sNoteSpringer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations..

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