Lupus (2015) 24, 341–342 http://lup.sagepub.com
LETTER TO THE EDITOR Diagnosis at your fingertips: splinters and microemboli – is it SLE? Sir, In winter 2011 a 24-year-old Caucasian woman presented with a 3-day history of painful lesions on the fingertips (Figure 1(a)) and subungual haemorrhages (Figure 1(b) and (c)). She negated joint pain, skin rash, sicca or constitutional symptoms. There was no history of thrombosis or abortion. Six weeks earlier she was vaccinated against HBV. Routine lab, ESR (2 mm/h) and urine sediment were normal. Pulse volume recording indicated normal peripheral perfusion and a cardiac or intravascular source of emboli was excluded. Capillary microscopy of the nail fold showed subungual bleeding (‘splinter’) (Figure 1(c)) but no other signs of collagen vascular disease (Figure 1(d)). Antinuclear antibodies (ANA) were slightly elevated with a titre of 1:80 (normal < 1:40) and anti-DNA antibodies were detectable at 59 IU/ml (normal < 10 IU/ml). Lupus anticoagulant was detectable using a DRVVT screening and confirmation test. Tests for complement consumption, anti-cardiolipin and anti-glycoprotein antibodies, rheumatic factor, HIV, HCV and cryoglobulinemia were all negative. Diagnosis of (secondary) antiphospholipid syndrome (APS), most likely on the basis of a systemic lupus erythematosus (SLE) predisposition, was made. Notably, the ACR SLE criteria were not fulfilled,1 but ANA, anti-DNA antibodies, and antiphospholipid antibodies can all occur years before definite diagnosis.2,3 Because of the absence of a severe thrombotic event, and since we expected the aetiology to be a minor arterial thrombotic occlusion, ASA 100 mg once daily was preferred over oral anticoagulation in this young patient. The pain and the subungual haemorrhages resolved within a few days, strongly supporting ischemic microinfarction rather than vasculitic haemorrhage as the pathogenic mechanism leading to the symptoms. The positive lupus anticoagulant was confirmed on two occasions 18 months apart. Correspondence to: Christoph T Berger, University Hospital Basel Department of Internal Medicine, Petersgraben 4, 403’1 Basel, Switzerland. Email: [email protected] The patient gave informed consent. Received 29 July 2014; accepted 3 October 2014 ! The Author(s), 2015. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav
Similarly, positive anti-DNA antibodies persisted on follow-up after 18 months at levels of 53–81 IU/ml. Furthermore, both splinter haemorrhages and the painful lesions on the fingertips reoccurred when ASA was transiently stopped after 12 months, suggesting a persistent underlying coagulopathy. However, during 2.5-year follow-up only mild intermittent arthralgia of the small finger joints, but no definite signs for SLE, occurred. Subungual splinter haemorrhages have been reported in APS but usually occur associated with severe thrombotic events and/or in clinically apparent SLE.4–6 In contrast, our case illustrates that this symptom may, however, represent the initial and isolated symptom of secondary APS. Previous studies reported that ASA was not effective in primary prevention of severe thrombotic events in patients with anti-phospholipid antibodies.7 Our case suggests, however, that it might be effective in treating and preventing reoccurrence of small arterial occlusion in secondary APS.
Funding CTB [PZ00P3-148000], CH [SNSF 310030_153059] and MR [PP00P3_144863] hold research grants from the Swiss National Science Foundation.
Conflict of interest statement The authors have no conflicts of interest to declare.
References 1 Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40: 1725. 2 McClain MT, Arbuckle MR, Heinlen LD, et al. The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum 2004; 50: 1226–1232. 3 Arbuckle MR, McClain MT, Rubertone MV, et al. Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 2003; 349: 1526–1533. 4 Frances C, Piette JC, Saada V, et al. Multiple subungual splinter hemorrhages in the antiphospholipid syndrome: A report of five cases and review of the literature. Lupus 1994; 3: 123–128. 10.1177/0961203314558862
Letter to the Editor CT Berger et al.
342 5 Frances C, Niang S, Laffitte E, Pelletier F, Costedoat N, Piette JC. Dermatologic manifestations of the antiphospholipid syndrome: Two hundred consecutive cases. Arthritis Rheum 2005; 52: 1785–1793. 6 Mujic F, Lloyd M, Cuadrado MJ, Khamashta MA, Hughes GR. Prevalence and clinical significance of subungual splinter haemorrhages in patients with the antiphospholipid syndrome. Clin Exp Rheumatol 1995; 13: 327–331. 7 Erkan D, Harrison MJ, Levy R, et al. Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: A randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. Arthritis Rheum 2007; 56: 2382–2391.
CT Berger1, T Baldi2, M Aschwanden2, K Scherer3, M Recher1, C Hess1 and T Daikeler1,4 1
Medical Outpatient Clinic, Department of Internal Medicine, University Hospital, Basel, Switzerland 2 Angiology Clinic, University Hospital, Basel, Switzerland 3 Allergy Unit, Department of Dermatology, University Hospital Basel, Switzerland 4 Rheumatology Clinic, University Hospital Basel, Switzerland
Figure 1 Patient presented with painful, erythematous lesions on the fingertips of the left hand (a) and subungual (‘splinter’) haemorrhages (b, c). Capillary microscopy of the nail fold showed subungual bleeding (‘splinter’) (c) but normal capillaries and no signs of collagen vascular disease (d).
Lupus
Copyright of Lupus is the property of Sage Publications, Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
LETTER TO THE EDITOR Diagnosis at your fingertips: splinters and microemboli – is it SLE? Sir, In winter 2011 a 24-year-old Caucasian woman presented with a 3-day history of painful lesions on the fingertips (Figure 1(a)) and subungual haemorrhages (Figure 1(b) and (c)). She negated joint pain, skin rash, sicca or constitutional symptoms. There was no history of thrombosis or abortion. Six weeks earlier she was vaccinated against HBV. Routine lab, ESR (2 mm/h) and urine sediment were normal. Pulse volume recording indicated normal peripheral perfusion and a cardiac or intravascular source of emboli was excluded. Capillary microscopy of the nail fold showed subungual bleeding (‘splinter’) (Figure 1(c)) but no other signs of collagen vascular disease (Figure 1(d)). Antinuclear antibodies (ANA) were slightly elevated with a titre of 1:80 (normal < 1:40) and anti-DNA antibodies were detectable at 59 IU/ml (normal < 10 IU/ml). Lupus anticoagulant was detectable using a DRVVT screening and confirmation test. Tests for complement consumption, anti-cardiolipin and anti-glycoprotein antibodies, rheumatic factor, HIV, HCV and cryoglobulinemia were all negative. Diagnosis of (secondary) antiphospholipid syndrome (APS), most likely on the basis of a systemic lupus erythematosus (SLE) predisposition, was made. Notably, the ACR SLE criteria were not fulfilled,1 but ANA, anti-DNA antibodies, and antiphospholipid antibodies can all occur years before definite diagnosis.2,3 Because of the absence of a severe thrombotic event, and since we expected the aetiology to be a minor arterial thrombotic occlusion, ASA 100 mg once daily was preferred over oral anticoagulation in this young patient. The pain and the subungual haemorrhages resolved within a few days, strongly supporting ischemic microinfarction rather than vasculitic haemorrhage as the pathogenic mechanism leading to the symptoms. The positive lupus anticoagulant was confirmed on two occasions 18 months apart. Correspondence to: Christoph T Berger, University Hospital Basel Department of Internal Medicine, Petersgraben 4, 403’1 Basel, Switzerland. Email: [email protected] The patient gave informed consent. Received 29 July 2014; accepted 3 October 2014 ! The Author(s), 2015. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav
Similarly, positive anti-DNA antibodies persisted on follow-up after 18 months at levels of 53–81 IU/ml. Furthermore, both splinter haemorrhages and the painful lesions on the fingertips reoccurred when ASA was transiently stopped after 12 months, suggesting a persistent underlying coagulopathy. However, during 2.5-year follow-up only mild intermittent arthralgia of the small finger joints, but no definite signs for SLE, occurred. Subungual splinter haemorrhages have been reported in APS but usually occur associated with severe thrombotic events and/or in clinically apparent SLE.4–6 In contrast, our case illustrates that this symptom may, however, represent the initial and isolated symptom of secondary APS. Previous studies reported that ASA was not effective in primary prevention of severe thrombotic events in patients with anti-phospholipid antibodies.7 Our case suggests, however, that it might be effective in treating and preventing reoccurrence of small arterial occlusion in secondary APS.
Funding CTB [PZ00P3-148000], CH [SNSF 310030_153059] and MR [PP00P3_144863] hold research grants from the Swiss National Science Foundation.
Conflict of interest statement The authors have no conflicts of interest to declare.
References 1 Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40: 1725. 2 McClain MT, Arbuckle MR, Heinlen LD, et al. The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum 2004; 50: 1226–1232. 3 Arbuckle MR, McClain MT, Rubertone MV, et al. Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 2003; 349: 1526–1533. 4 Frances C, Piette JC, Saada V, et al. Multiple subungual splinter hemorrhages in the antiphospholipid syndrome: A report of five cases and review of the literature. Lupus 1994; 3: 123–128. 10.1177/0961203314558862
Letter to the Editor CT Berger et al.
342 5 Frances C, Niang S, Laffitte E, Pelletier F, Costedoat N, Piette JC. Dermatologic manifestations of the antiphospholipid syndrome: Two hundred consecutive cases. Arthritis Rheum 2005; 52: 1785–1793. 6 Mujic F, Lloyd M, Cuadrado MJ, Khamashta MA, Hughes GR. Prevalence and clinical significance of subungual splinter haemorrhages in patients with the antiphospholipid syndrome. Clin Exp Rheumatol 1995; 13: 327–331. 7 Erkan D, Harrison MJ, Levy R, et al. Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: A randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. Arthritis Rheum 2007; 56: 2382–2391.
CT Berger1, T Baldi2, M Aschwanden2, K Scherer3, M Recher1, C Hess1 and T Daikeler1,4 1
Medical Outpatient Clinic, Department of Internal Medicine, University Hospital, Basel, Switzerland 2 Angiology Clinic, University Hospital, Basel, Switzerland 3 Allergy Unit, Department of Dermatology, University Hospital Basel, Switzerland 4 Rheumatology Clinic, University Hospital Basel, Switzerland
Figure 1 Patient presented with painful, erythematous lesions on the fingertips of the left hand (a) and subungual (‘splinter’) haemorrhages (b, c). Capillary microscopy of the nail fold showed subungual bleeding (‘splinter’) (c) but normal capillaries and no signs of collagen vascular disease (d).
Lupus
Copyright of Lupus is the property of Sage Publications, Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
