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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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D. Eosinophilic fasciitis

Insidious onset of bilaterally limited joint mobility is infrequently seen by neurologists. EMG showed electrical myotonia, reminiscent of myotonic dystrophy (choice A).1 However, myotonic dystrophy is characterized by abnormally slow or delayed muscle relaxation following a normal muscle contraction,1 which was absent in this patient. Limited joint mobility and the prayer sign can be seen in systemic sclerosis (choice B).2 However, the negative ANA test results and the absence of Raynaud phenomenon, sclerodactyly, and internal organ involvement helped exclude systemic sclerosis.3 Limited mobility of joints, peripheral eosinophilia, and increased ESR and CRP can be found in deep morphea (choice C), a rare subtype of morphea that primarily involves the deep dermis and subcutaneous tissue and may extend to underlying fascia and muscle.4 Nevertheless, the symmetrical distribution of joint involvement, histology displaying preferential fascial inflammation (Figure 2), and the absence of sclerotic skin plaque in this patient point to the diagnosis of eosinophilic fasciitis (EF) over deep morphea.

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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.

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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
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
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
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
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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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