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Severe Cervicodynia in a Patient With Pustules on the Palms

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 51-year-old previously healthy man was admitted with severe neck pain for 6 days. In the past month, he felt neck stiffness, accompanied by swelling and persistent dull pain of the upper anterior chest wall, which could be transiently relieved by taking oral ibuprofen. Six days before admission, the patient’s neck stiffness transited to an endurable dull ache. When turning his head, there was severe burning pain in the posterior neck and occiput, followed immediately by an electric shocklike numbness on ipsilateral side tongue, which led to a forced head position. Magnetic resonance imaging of the cervical spine with gadolinium on admission revealed signal abnormality in C1/C2 vertebrae (Figure 1A). In the past 3 days, with the intensification of neck pain, the patient found pustules on both hands, which gradually increased. He had no history of tobacco, alcohol, or drug misuse. On examination, he was afebrile. There was tenderness in the right sternoclavicular articulation, upper neck, post aurem, and occiput. Multiple pustules were seen in the palm and back of both hands (Figure 1B). Complete blood cell count and liver and kidney function yielded normal results, except for elevated erythrocyte sedimentation rate (ESR) of 93 mm/h and C-reactive protein (CRP) level of 5.5 mg/dL (to convert to milligrams per liter, multiply by 10). Results of workup for rheumatic and infectious disease, including antinuclear, antineutrophil cytoplasmic and anticardiolipin antibodies, human leukocyte antigen B27, repeated blood cultures, rapid plasma reagin test, Aspergillus galactomannan antigen, brucella, Mycobacterium tuberculosis, and rickettsial antibodies, were all negative. Cell count, protein, and glucose in cerebrospinal fluid were normal. Cultures of cerebrospinal fluid revealed no organisms. Whole-body fluorodeoxyglucose–positron emission tomography/computed tomography revealed no malignancy.

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D. Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome

Sudden rotation of the neck inducing a sharp pain on the side of the upper neck or occiput, followed immediately by transient numbness of the tongue on the same side is the character of neck-tongue syndrome, a uncommon headache syndrome.1 The pain is related to an irritation of the C2 and/or C3 roots, with tongue involvement due to afferent impulses traveling from the lingual nerve via the hypoglossal nerve to the C1/C2 roots.2 Abnormalities showed by magnetic resonance imaging in C1/C2 vertebrae were consistent with spondylitis and spondylodiscitis.

Neurosyphilis (choice A), metastatic tumor (choice B), and rickettsial infection (choice C) were all excluded after the tests of rapid plasma reagin, rickettsial antibodies, and whole-body fluorodeoxyglucose–positron emission tomography/computed tomography, respectively. Multiple pustules on the hand accompanying C1/C2 spondylitis and spondylodiscitis and tenderness in the right sternoclavicular articulation reminded us of suspicion of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. A whole-body bone scintigraphy was ordered, revealing the classic bull head sign with increased tracer uptake in bilateral sternoclavicular joints and the sternal angle (Figure 2), which is highly specific for SAPHO syndrome.3

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Article Information

Corresponding Author: Yan Lv, MD, Department of Neurology, General Hospital of Northern Theater Command, No. 83 Wenhua St, Shenyang 110016, China (lana323@foxmail.com).

Published Online: December 27, 2022. doi:10.1001/jamaneurol.2022.4739

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

Queiroz  LP .  Unusual headache syndromes.   Headache. 2013;53(1):12-22. doi:10.1111/head.12002PubMedGoogle ScholarCrossref
Lance  JW , Anthony  M .  Neck-tongue syndrome on sudden turning of the head.   J Neurol Neurosurg Psychiatry. 1980;43(2):97-101. doi:10.1136/jnnp.43.2.97PubMedGoogle ScholarCrossref
Schaub  S , Sirkis  HM , Kay  J .  Imaging for synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.   Rheum Dis Clin North Am. 2016;42(4):695-710. doi:10.1016/j.rdc.2016.07.011PubMedGoogle ScholarCrossref
Chamot  AM , Benhamou  CL , Kahn  MF , Beraneck  L , Kaplan  G , Prost  A .  Le syndrome acné pustulose hyperostose ostéite (SAPHO): résultats d’une enquête nationale: 85 observations.   Rev Rhum Mal Osteoartic. 1987;54(3):187-196.PubMedGoogle Scholar
Guo  C , Tian  X , Han  F , Liu  L , Gao  J , Ma  X .  Copy number variation of multiple genes in SAPHO syndrome.   J Rheumatol. 2020;47(9):1323-1329. doi:10.3899/jrheum.181393PubMedGoogle ScholarCrossref
Przepiera-Będzak  H , Brzosko  M .  SAPHO syndrome: pathogenesis, clinical presentation, imaging, comorbidities and treatment: a review.   Postepy Dermatol Alergol. 2021;38(6):937-942. doi:10.5114/ada.2020.97394PubMedGoogle ScholarCrossref
Hayem  G , Bouchaud-Chabot  A , Benali  K ,  et al.  SAPHO syndrome: a long-term follow-up study of 120 cases.   Semin Arthritis Rheum. 1999;29(3):159-171. doi:10.1016/S0049-0172(99)80027-4PubMedGoogle ScholarCrossref
Kahn  MF , Khan  MA .  The SAPHO syndrome.   Baillieres Clin Rheumatol. 1994;8(2):333-362. doi:10.1016/S0950-3579(94)80022-7PubMedGoogle ScholarCrossref
Schilling  F , KesslerS.  Das SAPHO-syndrom: klinisch-rheumatologische und radiologische differenzierung und klassifizierung eines krankengutes von 86 fällen.   Z Rheumatol. 2000;59(1):1-28. doi:10.1007/s003930050001PubMedGoogle ScholarCrossref
Liu  S , Tang  M , Cao  Y ,  et al.  Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update.   Ther Adv Musculoskelet Dis. 2020;12:1759720X20912865. doi:10.1177/1759720X20912865Google ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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