joint hypermobility (BJH), consistent of erythromelalgia and/or acrocyanosis with concomitant over-sweating (hyperidrosis-HI) of palms. Vounotrypidis P. 1.
Peripheral Vasomotor Symptoms in Patients with Joint Hypermobility 1,
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Vounotrypidis P Pappas PE Toulis Sakellariou GT Berberidis C 1 2 Rheumatology and Vascular Surgery Departments, 424 General Army Hospital, Thessaloniki, Macedonia, Greece
Background Benign articular hypermobility has been associated by some authors with an increased incidence of Raynaud’s phenomenon (RP)1,2 while this is not supported by others.3 We observed a particular pattern of vasomotor symptoms in patients with benign joint hypermobility (BJH), consistent of erythromelalgia and/or acrocyanosis with concomitant over-sweating (hyperidrosis-HI) of palms.
Objectives To demonstrate the high frequency of peripheral vasomotor symptoms (PVS), not typical of Raynaud’s phenomenon, in patients with BJH.
Materials & Methods Beighton’s scoring method was used to identify the BJH in a series of 38 patients who attended the rheumatology outpatient clinic between June and December 2007. The reason of consultation, the presence of joint complaints and vasomotor symptoms of the extremities were recorded. A full medical history as well as clinical and laboratory investigation among with cold exposure test were performed. Echocardiogram, vascular imaging and immunology investigation performed in selected patients (Table 1). Patients with signs suggesting a hereditary collagen disorder were excluded. We used SPSS v.15.0, Chicago, USA for the statistical analysis.
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Abstract No 1574
Results
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38 patients attended the rheumatology outpatient clinic were identified to have joint hypermobility. One patient was excluded due to Marfanoid habitus. Finally, there were 30 males and 7 females with mean age 26.4, SD: 5.3) and 33.2, SD: 4.89 respectively. Females had statistically higher Beighton scores than males (Table 2) 3
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Fig. 1. Acrocyanosis, 2. Erythromelalgia after cold exposure test, 3-4 and 4-5-6: Late blood restoration after mild pressure
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Fig.8: Comparing normal and abnormal Fig.9: Livedo reticularis in one patient Tests performed
Findings/Tested
Chest & Cervical x-rays
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Doppler vascular ultrasound Echocardiogram Mitral valve prolapse
0/15
ANA, Anti-ENA, Anti-Scl70, ACA
PVS were present in 40% of males and 42.8% of females (Fisher’s Exact test p=0.67>0.05). None of the patients had symptoms suggesting a typical Raynaud’s phenomenon HI of palms (HI) was present in 33.3% of males and 14.3% of females (Fisher’s Exact test p=0.36>0.05). Both PVS and HI had 23.3% of males and 14.3% of females. Symptoms may be exaggerated by the same reasons with RP. They were biphasic, persistent and long lasting with predominant features of acrocyanosis or erythromelalgia. Late blood restoration after mild presure is special characteristic (Figures 3-4 & 5-6). One male patient had livedo reticularis of the extremeties (Fig 9).
0/6 M & 1/3 F 1 / 5 M (ANA: 1/80)
Table 1: Performed tests in selected patients
Beighton score PVS HI PVS + HI
Males Ν=30 (%) 5.77 (SD:0.96) 12 (40) 10 (33.3) 7 (23.3)
Females Ν=7 (%) 7.14 (SD:0.77) 3 (42.8) 1 (14.3) 1 (14.3)
Statistics Student t test p=0.05 Fisher’s exact test p=0.67 Fisher’s exact test p=0.36
Table 2: Prevalence of PVS, HI and PVS + HI in our series of Hypermobile joint patients
Conclusions Vasomotor symptoms of the extremities are common complains in BJH. They are presented either as acrocyanosis or erythromelalgia as well as hyperidrosia of the extremities. Their incidence is sex independent. Individuals with high Beighton scores are more likely to have both peripheral vasomotor symptoms plus hyperidrosia of palms. The late blood restoration sign is suggesting an autonomous peripheral nerve dysfunction.
References 1. http://www.helix-health.co.uk/medical/JHS.htm 2. El-Garf AK, Mahmoud GA, Mahgoub EH. Hypermobility among Egyptian children: prevalence and features. J Rheumatol 1998;25:1003-5 3. Bravo JF, Wolff C. Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers-Danlos syndrome. Arthritis Rheum 2006;54:515-23
