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A 31-Year-Old Man With Bilaterally Limited Mobility of Joints

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

A 31-year-old man with insidious onset of limited mobility of joints visited our clinic for evaluation. Six months prior, he found his palms could not make full contact with each other (the “prayer sign”). He had not been able to fully clench his fists for 2 months or squat for the past 10 days. No erythema, edema, or sclerotic skin plaque had been found on his limbs or trunk. No Raynaud phenomenon had occurred. His medical and family histories were unremarkable. On examination, he showed bilateral limited mobility of interphalangeal (hands), metacarpophalangeal, wrist, interphalangeal (feet), metatarsophalangeal, and ankle joints (Video). No muscular atrophy or sclerodactyly was found. The prayer sign (Figure 1A) and the “groove sign” (Figure 1B) were observed. Blood test results indicated eosinophilia (absolute eosinophil count, 1300 μL; reference, <500μL [to convert to ×109/L, multiply by 0.001]), elevated erythrocyte sedimentation rate (ESR, 31 mm/h; reference, <15 mm/h) and C-reactive protein (CRP, 3.01 mg/dL; reference, <0.8 mg/dL [to convert to mg/L, multiply by 10]). Liver and kidney function tests, creatine kinase level, electrolyte level, thyroid function, globulin concentration, and antinuclear antibody (ANA) level were unremarkable. Electrocardiogram, cervical spine magnetic resonance imaging, and plain radiographs of hands, wrists, feet, and ankles were normal. Nerve conduction studies performed in all 4 limbs were normal. Electromyography (EMG) showed spontaneous activities in muscles of limbs, including fibrillation potentials and positive sharp waves (1+ or 2+). Myotonic discharges were seen in bilateral extensor digitorum.

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A 31-year-old man with insidious onset of limited mobility of joints visited our clinic for evaluation. Six months prior, he found his palms could not make full contact with each other (the “prayer sign”). He had not been able to fully clench his fists for 2 months or squat for the past 10 days. No erythema, edema, or sclerotic skin plaque had been found on his limbs or trunk. No Raynaud phenomenon had occurred. His medical and family histories were unremarkable. On examination, he showed bilateral limited mobility of interphalangeal (hands), metacarpophalangeal, wrist, interphalangeal (feet), metatarsophalangeal, and ankle joints (Video). No muscular atrophy or sclerodactyly was found. The prayer sign (Figure 1A) and the “groove sign” (Figure 1B) were observed. Blood test results indicated eosinophilia (absolute eosinophil count, 1300 μL; reference, <500μL [to convert to ×109/L, multiply by 0.001]), elevated erythrocyte sedimentation rate (ESR, 31 mm/h; reference, <15 mm/h) and C-reactive protein (CRP, 3.01 mg/dL; reference, <0.8 mg/dL [to convert to mg/L, multiply by 10]). Liver and kidney function tests, creatine kinase level, electrolyte level, thyroid function, globulin concentration, and antinuclear antibody (ANA) level were unremarkable. Electrocardiogram, cervical spine magnetic resonance imaging, and plain radiographs of hands, wrists, feet, and ankles were normal. Nerve conduction studies performed in all 4 limbs were normal. Electromyography (EMG) showed spontaneous activities in muscles of limbs, including fibrillation potentials and positive sharp waves (1+ or 2+). Myotonic discharges were seen in bilateral extensor digitorum.

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Article Information

Corresponding Author: Yafang Zhou, MD, PhD, Department of Geriatric Neurology, Xiangya Hospital, Central South University, 87 Xiangya Rd, Changsha 410008, China (zyf_1981@csu.edu.cn).

Published Online: August 22, 2022. doi:10.1001/jamaneurol.2022.2313

Conflict of Interest Disclosures: Drs Z. Wang and Zhou reported grants from Natural Science Foundation of Hunan Province, China, during the conduct of the study. No other disclosures were reported.

References
1.
Miller  TM .  Differential diagnosis of myotonic disorders.   Muscle Nerve. 2008;37(3):293-299. doi:10.1002/mus.20923PubMedGoogle ScholarCrossref
2.
Uslu  S , Gülle  S , Can  G , Sarı  İ , Önen  F , Birlik  M .  Evaluation of limited hand mobility in systemic sclerosis patients by using “prayer sign” and “tabletop sign”.   Clin Rheumatol. 2021;40(7):2771-2777. doi:10.1007/s10067-021-05623-9PubMedGoogle ScholarCrossref
3.
Fett  N , Arthur  M .  Eosinophilic fasciitis: current concepts.   Clin Dermatol. 2018;36(4):487-497. doi:10.1016/j.clindermatol.2018.04.006PubMedGoogle ScholarCrossref
4.
Laxer  RM , Zulian  F .  Localized scleroderma.   Curr Opin Rheumatol. 2006;18(6):606-613. doi:10.1097/01.bor.0000245727.40630.c3PubMedGoogle ScholarCrossref
5.
Berianu  F , Cohen  MD , Abril  A , Ginsburg  WW .  Eosinophilic fasciitis: clinical characteristics and response to methotrexate.   Int J Rheum Dis. 2015;18(1):91-98. doi:10.1111/1756-185X.12499PubMedGoogle ScholarCrossref
6.
Asaoka  K , Watanabe  Y , Itoh  K ,  et al.  A case of eosinophilic fasciitis without skin manifestations: a case report in a patient with lupus and literature review.   Clin Rheumatol. 2021;40(6):2477-2483. doi:10.1007/s10067-020-05416-6PubMedGoogle ScholarCrossref
7.
Huppke  P , Wilken  B , Brockmann  K , Sattler  B , Hanefeld  F .  Eosinophilic fasciitis leading to painless contractures.   Eur J Pediatr. 2002;161(10):528-530. doi:10.1007/s00431-002-1038-1PubMedGoogle ScholarCrossref
8.
Jinnin  M , Yamamoto  T , Asano  Y ,  et al.  Diagnostic criteria, severity classification and guidelines of eosinophilic fasciitis.   J Dermatol. 2018;45(8):881-890. doi:10.1111/1346-8138.14160PubMedGoogle ScholarCrossref
9.
Mango  RL , Bugdayli  K , Crowson  CS ,  et al.  Baseline characteristics and long-term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years.   Int J Rheum Dis. 2020;23(2):233-239. doi:10.1111/1756-185X.13770PubMedGoogle 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 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous 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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