sapho: a case of skin and bones - ohsu acp.pdf · (hands and feet), synovitis (knees), hyperostosis...

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Bone scintigraphy showed multiple signs of costochondritis. With this constellation of pustolosis (hands and feet), synovitis (knees), hyperostosis (syndesmophytes), and osteitis (discitis), Rheumatology postulated a unifying diagnosis of SAPHO. General consensus among the consulting services was that the discitis was likely inflammatory, and thus, CT-guided biopsy was deferred. The patient was started on certolizumab with complete disease remission after 6 months of therapy and remains in remission after 12 months of follow up. CONCLUSION The prevalence of SAPHO in the general population is estimated to be 1 in 10,000. It is a disease primarily affecting young women and thought to be underdiagnosed due to lack of awareness in the primary care setting. Delay in treatment can lead to chronic pain and disfigurement of skin and joints. Given its chronicity and relapsing course, continuity of patient care and medical records can aid in recognizing the pattern of disease. Remember: SAPHO: A Case of Skin and Bones Teena Huan Xu MD, Cong-Qiu Chu MD Portland VA Medical Center, 2016 INTRODUCTION SAPHO is a relapsing inflammatory disease involving the skin, bones, and joints. It is a rare syndrome involving a constellation of common symptoms that requires high clinical suspicion to unify the diagnosis. CASE SUMMARY This is a case of a 23-year-old female soldier with chronic pain and pustular skin rash over a 10 year period. Her pain remained refractory to an arsenal of analgesics and physical therapy. She was referred to Dermatology for hand and foot dermatitis refractory to topical steroids. Initially, she received a diagnosis of severe Palmoplantar Pustular Psoriasis; however, courses of methotrexate, adalimumab, and etanercept were all ineffective. Two weeks after stopping treatment with etanercept, she presented with severe neck pain without associated infectious or neurologic symptoms. Plain films showed a possible C5 fracture and subsequent CT and MRI revealed C5-C6 discitis with anterior bridging cervical syndesmophytes. This prompted admission for a CT-guided biopsy with concern for an infectious, inflammatory, or malignant etiology. On exam, she had scaling palmoplantar plaques, tenderness at multiple costochondral junctions, and cervical midline tenderness with restricted movement in all directions. Labs and cultures were unremarkable other than a mildly elevated CRP. S ynovitis A cne P ustulosis H yperostosis O steitis Figure 7. Bone scintigraphy: multiple signs of costochondritis (2013) Figure 6. MRI cervical: C5-C6 discitis, anterior bridging syndesmophytes (2013) Figure 5. CT cervical: C5-C6 discitis (2013) Figure 4. Body map: summation of chronic pain syndrome in this SAPHO patient (2005-2015) Figure 3. Photo: plantar pustulosis (2012) Figure 2. Photo: palmar plaques and pustulosis (2012) Figure 1. Photo: palmar plaques (2009) REFERENCES 1. Zimmerman et al. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: A challenging diagnosis not to be missed. Journal of Infection (2016) 72, S106-S114. 2. Kundu et al. Diagnosing the SAPHO syndrome: a report of three cases and review of literature. Clin Rheumatol (2013) 32:1237–1243.

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Page 1: SAPHO: A Case of Skin and Bones - OHSU ACP.pdf · (hands and feet), synovitis (knees), hyperostosis (syndesmophytes), and osteitis (discitis), Rheumatology postulated a unifying diagnosis

Bone scintigraphy showed multiple signs ofcostochondritis. With this constellation of pustolosis(hands and feet), synovitis (knees), hyperostosis(syndesmophytes), and osteitis (discitis),Rheumatology postulated a unifying diagnosis ofSAPHO. General consensus among the consultingservices was that the discitis was likelyinflammatory, and thus, CT-guided biopsy wasdeferred. The patient was started on certolizumabwith complete disease remission after 6 months oftherapy and remains in remission after 12 monthsof follow up.

CONCLUSIONThe prevalence of SAPHO in the general populationis estimated to be 1 in 10,000. It is a diseaseprimarily affecting young women and thought to beunderdiagnosed due to lack of awareness in theprimary care setting. Delay in treatment can lead tochronic pain and disfigurement of skin and joints.Given its chronicity and relapsing course, continuityof patient care and medical records can aid inrecognizing the pattern of disease. Remember:

SAPHO: A Case of Skin and BonesTeena Huan Xu MD, Cong-Qiu Chu MD

Portland VA Medical Center, 2016

INTRODUCTIONSAPHO is a relapsing inflammatory diseaseinvolving the skin, bones, and joints. It is a raresyndrome involving a constellation of commonsymptoms that requires high clinical suspicionto unify the diagnosis.

CASE SUMMARYThis is a case of a 23-year-old female soldierwith chronic pain and pustular skin rash over a10 year period. Her pain remained refractory toan arsenal of analgesics and physical therapy.She was referred to Dermatology for hand andfoot dermatitis refractory to topical steroids.Initially, she received a diagnosis of severePalmoplantar Pustular Psoriasis; however,courses of methotrexate, adalimumab, andetanercept were all ineffective.

Two weeks after stopping treatment withetanercept, she presented with severe neckpain without associated infectious orneurologic symptoms. Plain films showed apossible C5 fracture and subsequent CT andMRI revealed C5-C6 discitis with anteriorbridging cervical syndesmophytes. Thisprompted admission for a CT-guided biopsywith concern for an infectious, inflammatory, ormalignant etiology.

On exam, she had scaling palmoplantarplaques, tenderness at multiple costochondraljunctions, and cervical midline tenderness withrestricted movement in all directions. Labs andcultures were unremarkable other than a mildlyelevated CRP.

S ynovitisA cneP ustulosisH yperostosisO steitis

Figure 7. Bone scintigraphy: multiplesigns of costochondritis (2013)

Figure 6. MRI cervical: C5-C6 discitis,anterior bridging syndesmophytes (2013)

Figure 5. CT cervical: C5-C6 discitis(2013)

Figure 4. Body map: summation of chronic pain syndrome inthis SAPHO patient (2005-2015)

Figure 3. Photo: plantar pustulosis(2012)

Figure 2. Photo: palmar plaques andpustulosis (2012)

Figure 1. Photo: palmar plaques (2009)

REFERENCES1. Zimmerman et al. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: A challenging diagnosis not to be missed. Journal of Infection (2016) 72, S106-S114.

2. Kundu et al. Diagnosing the SAPHO syndrome: a report of three cases and review of literature. Clin Rheumatol (2013) 32:1237–1243.