|IMAGES IN ACADEMIC MEDICINE
|Year : 2017 | Volume
| Issue : 1 | Page : 197-201
Finger nail changes: A red flag for connective tissue disease
Mark W Fegley1, Rodrigo Duarte-Chavez2, Whitney Fegley3, Lauren E Stone4, Amitoj Singh1, Sudip Nanda2
1 Department of Family Medicine, Surgery, St. Luke's University Health Network, Bethlehem, USA
2 Department of Internal Medicine, Surgery, St. Luke's University Health Network, Bethlehem, USA
3 Department of Medicine Surgery, Surgery, St. Luke's University Health Network, Bethlehem, USA
4 Lewis Katz School of Medicine at Temple University, 3500 N Broad St, Philadelphia, Pennsylvania, USA
|Date of Web Publication||7-Jul-2017|
Department of Internal Medicine, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, Pennsylvania - 18015
Source of Support: None, Conflict of Interest: None
We report a 68-year-old female who presented to the cardiology clinic with ventricular tachycardia and specific finger nail abnormalities including proximal capillary loops and proximal and periungual erythema. The patient had multiple underlying connective tissue disorders and pulmonary fibrosis. Finger nail changes are highly specific and are an indication for all healthcare providers that connective tissue diseases (CTDs) are likely underlying. We review the clinical signs and symptoms, review diagnostic criteria, and further testing to evaluate for CTDs.
The following core competencies are addressed in this article: Patient care, Medical knowledge.
Keywords: Connective tissue disease, dermatomyositis, finger nail changes, periungual changes, Sjögren's syndrome, systemic lupus erythematous
|How to cite this article:|
Fegley MW, Duarte-Chavez R, Fegley W, Stone LE, Singh A, Nanda S. Finger nail changes: A red flag for connective tissue disease. Int J Acad Med 2017;3:197-201
|How to cite this URL:|
Fegley MW, Duarte-Chavez R, Fegley W, Stone LE, Singh A, Nanda S. Finger nail changes: A red flag for connective tissue disease. Int J Acad Med [serial online] 2017 [cited 2019 Mar 22];3:197-201. Available from: http://www.ijam-web.org/text.asp?2017/3/1/197/209837
| Introduction|| |
We report a 68-year-old female who presented to the cardiology clinic with ventricular tachycardia with specific finger nail abnormalities. The patient had multiple underlying connective tissue disorders and pulmonary fibrosis. There are multiple finger nail changes that are indicative of connective tissue disorders. We review specific finger nail changes and provide a review of diagnostic criteria including clinical signs and symptoms and further tests needed to rule out or confirm underlying connective tissue disease (CTD).
| Case Report|| |
A 68-year-old woman presented to the cardiology clinic for episodic ventricular tachycardia. The patient had a 30–plus-year history of systemic lupus erythematous (SLE), Sjögren's syndrome (SS), and dermatomyositis with interstitial lung disease. Her underlying medical therapy included the following: Prednisone 10 mg, hydroxychloroquine 200 mg, and mycophenolate mofetil 2500 mg daily. Her past medical history included (1) well controlled hypertension-therapy hydrochlorothiazide 12.5 mg, (2) chronic gastroesophageal reflux disease-therapy omeprazole 20 mg, (3) sicca syndrome-therapy 5 mg pilocarpine and finally, (4) long standing hypothyroid-therapy levothyroxine 100 mcg.
Her physical examination was remarkable for finger nail changes including the following: (1) Multiple dilated capillaries around fingernails, (2) erythema of proximal and periungual fingernail [Figure 1], and (3) rash on dorsal surface of hands, knuckles, and wrist. Cardiopulmonary examination was unremarkable. An in-office electrocardiogram was normal sinus rhythm. Prior antibody work up was positive for antinuclear antibodies, anti-SS antibody A, and cyclic citrullinated peptide. More recently, she had a weakly positive antisignal recognition particle and weakly positive p140. The most recent pulmonary function tests revealed forced expiratory volume 1 second (FEV1) 84% predicted, forced expiratory capacity (FEC) 75%, and FEV1/FEC ratio of 86% corresponding to mild restrictive disease. Diffusion limit of carbon dioxide was 43% predicted corresponding to moderate to severe diffusion capacity dysfunction. She was on verapamil 120 mg extended release daily and was stable as far as ventricular tachycardia was concerned.
|Figure 1: Finger nail changes in our patient: Finger nail capillary loops can be clearly seen (black arrows). The patient also has periungual and proximal nail-fold erythema|
Click here to view
| Discussion|| |
CTDs are common, often overlap, require lifelong care and are associated with numerous organ involvement. CTD commonly affects many organs, including skin and fingernails. In a case–control study by Tunc et al., 190 patients with known CTD were enrolled and compared against two control groups. For those with SLE, a control group of young healthy patients was used, whereas patients with other CTD were compared against healthy elderly controls. The study found fingernail changes are highly specific for CTDs (98–100%) but have poor sensitivity (13–31%). The results are summarized in [Table 1]. The study concluded that finger nail changes do not correlate with disease activity with the following two exceptions: Splinter hemorrhage-SLE and increase in transverse nail curvature-systemic sclerosis (SSc). Two additional studies have attempted to determine the frequency of nail-fold changes in patients with CTD. Both studies corroborate with the study by Tunc in that nail changes have poor sensitivity for CTD., Fingernail changes are not exclusive to CTD, and an alternative diagnosis should be sought if fingernail changes are seen outside of those listed in [Table 1].
|Table 1: Summary of reported nail findings in connective tissue disease in Tunc study|
Click here to view
Reynaud's phenomena can also be associated with various CTD, including SSc. Nailfold videocapillaroscopy (NVC) and analysis of red blood cell velocity offers a noninvasive indicator that is both sensitive, specific, and prognostic for underlying SSc. Initially, capillary morphology and analysis with microscopy appeared to offer little relation between clinical features. A specific classification of NVC has been developed by Carpentier and Maricq further modified by Bergman et al. to classify a pattern consistent with SSc., Cutolo et al. further sub classified the NVC into early, active, and late stage prognostic indicators that may be predictive of organ involvement in SSc based on capillary arrangement, quantity and level of organization., Red blood cell velocity offers an additional diagnostic tool separate from NVC. In a study by Mugii et al. analyzing patients who were healthy versus known SSc, even patients with normal NSV with SSc were found to have a 51.7% reduction in red blood cell velocity.
The pathogenesis of fingernail changes from CTD involves antibodies which alter the underlying structure and vessel supply of the proximal nail and nailbed. When detecting the previously mentioned nail changes in [Table 1], the physician should attempt to elicit other signs and symptoms of CTD and perform further evaluation detailed in [Table 2].,,,,, If clinical signs and symptoms are detected, further laboratory testing can be useful as outlined in [Table 3]. If underlying Reynaud's phenomena is detected, sending the patient for NVC and red blood cell velocity analysis is warranted for early diagnosis of underlying SSc. These test also have the additional benefit of prognostic indications of SSc.
|Table 2: An Algorithm of further diagnostic studies when fingernail changes are detected|
Click here to view
|Table 3: An Algorithm for laboratory studies if finger nail changes and the clinical criteria is met in diagnostic criteria dermatology|
Click here to view
|Figure 2: Images of a normal finger nail in a 30-year-old female with no significant medical history. Arrows in each display where each abnormality would be located in fingernail. (a) Capillary loops in proximal nail-folds. (b) Erythema of proximal nail-fold. (c) Longitudinal curvature increase in nails. (d) Periungual erythema. (e) Red lunulae. (f) Splinter hemorrhages. (g) Thin nail plate. (h) Transverse curvature increase in nails. (i) White dull color in fingernails|
Click here to view
| Conclusion|| |
CTD can be difficult to diagnose, and often times patients can present with devastating signs and symptoms. However, the finger nail changes listed in [Table 2] are an indicator for further investigation and workup for underlying CTD. If the healthcare provider suspects underlying CTD in the absence of finger nail changes, a workup is still indicated due to poor sensitivity of such changes.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Gaubitz M. Epidemiology of connective tissue disorders. Rheumatology (Oxford) 2006;45 Suppl 3:iii3-4.
Tunc SE, Ertam I, Pirildar T, Turk T, Ozturk M, Doganavsargil E. Nail changes in connective tissue diseases: Do nail changes provide clues for the diagnosis? J Eur Acad Dermatol Venereol 2007;21:497-503.
Elmansour I, Chiheb S, Benchikhi H. Nail changes in connective tissue diseases: A study of 39 cases. Pan Afr Med J 2014;18:150.
Nabil P, Rao R, Shenoi S, Balachandran C. Nail unit in collagen vascular diseases: A clinical, histopathological and direct immunofluorescence study. Indian Journal of Dermatology 2006;41:265-8.
Fawcett RS, Linford S, Stulberg DL. Nail abnormalities: Clues to systemic disease. Am Fam Physician 2004;69:1417-24.
Lefford F, Edwards JC. Nailfold capillary microscopy in connective tissue disease: A quantitative morphological analysis. Ann Rheum Dis 1986;45:741-9.
Carpentier PH, Maricq HR. Microvasculature in systemic sclerosis. Rheum Dis Clin North Am 1990;16:75-91.
Bergman R, Sharony L, Schapira D, Nahir MA, Balbir-Gurman A. The handheld dermatoscope as a nail-fold capillaroscopic instrument. Arch Dermatol 2003;139:1027-30.
Smith V, Riccieri V, Pizzorni C, Decuman S, Deschepper E, Bonroy C, et al.
Nailfold capillaroscopy for prediction of novel future severe organ involvement in systemic sclerosis. J Rheumatol 2013;40:2023-8.
Cutolo M, Sulli A, Secchi ME, Paolino S, Pizzorni C. Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement? Rheumatology (Oxford, England) 2006;45 Suppl 4:iv43-6.
Mugii N, Hasegawa M, Hamaguchi Y, Tanaka C, Kaji K, Komura K, et al.
Reduced red blood cell velocity in nail-fold capillaries as a sensitive and specific indicator of microcirculation injury in systemic sclerosis. Rheumatology (Oxford) 2009;48:696-703.
Arnett FC, Edworthy SM, Bloch DA, McShane DJ, Fries JF, Cooper NS, et al.
The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988;31:315-24.
Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al.
The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7.
Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al.
American College of Rheumatology classification criteria for Sjögren's syndrome: A data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) 2012;64:475-87.
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al.
2013 classification criteria for systemic sclerosis: An American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 2013;65:2737-47.
Hunder GG. Atlas of Rheumatology. 3rd
ed. Philadelphia: Lippincott Williams and Wilkins; 2002. p. vii, 227.
[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]