|Year : 2019 | Volume
| Issue : 2 | Page : 115-120
Nailfold capillaries in connective tissue diseases in skin of color: A dermoscopic view
Balachandra S Ankad, Priyanka S Jaju
Department of Dermatology, S. Nijalingappa Medical College, Bagalkot, Karnataka, India
|Date of Web Publication||15-Jul-2019|
Balachandra S Ankad
Department of Dermatology, S. Nijalingappa Medical College, Navanagar, Bagalkot - 587 102, Karnataka
Source of Support: None, Conflict of Interest: None
Introduction: Dermoscopy has gained tremendous importance in the recent past. It helps in the visualization of subsurface structures, whereby details of skin lesion are studied in depth. Nailfold capillaries are involved early in the course of disease process in connective tissue diseases. Videocapillaroscopy is used to examine the patterns in the nailfold capillaries. However, training, cost, and skilled technique limits the use of it. Dermoscopy, being handheld and easy to perform, is best alternative to videocapillaroscopy. Authors evaluated the utility of dermoscopy in the study of nailfold capillaries in connective tissue diseases in patient with the skin of color. To the best knowledge of authors, this is the first study from the Indian subcontinent. Materials and Methods: The study was conducted in a tertiary care hospital from January 2017 to June 2017. It was a cross-sectional pilot study. Sixteen consecutive patients with connective tissue diseases were included in the study. Connective tissue diseases comprised of systemic sclerosis, lupus erythematosus, mixed connective tissue disease, dermatomyositis, and rheumatoid arthritis. DermLite 3 dermoscope with Sony camera was employed. Polarized mode and ultrasound gel were used. Results: Of 16 patients, three, two, and three had systemic sclerosis, systemic lupus erythematosus, and discoid lupus erythematosus, respectively. Mixed connective tissue disease, dermatomyositis, and rheumatoid arthritis were seen in one, one, and five patients, respectively. One patient had Rowell syndrome. Scleroderma and nonspecific scleroderma patterns were observed in 75% and 12.5% of patients, respectively. In two patients, the nailfold capillaries appeared normal. Conclusion: Dermoscopy is an in vivo cost-effective method for studying nailfold capillaries in connective tissue diseases. Results obtained using handheld dermoscope were comparable to that of a videocapillaroscope. Authors recommend further studies involving large sample size of the population with skin of color to affirm the nailfold capillaries pattern observed in this study.
Keywords: Connective tissue disease, dermoscopy, nailfold capillaries, scleroderma pattern
|How to cite this article:|
Ankad BS, Jaju PS. Nailfold capillaries in connective tissue diseases in skin of color: A dermoscopic view. Clin Dermatol Rev 2019;3:115-20
| Introduction|| |
Dermoscopy is a noninvasive diagnostic technique using incident light to illuminate subsurface structures of the skin making it accessible for visual examination. Dermoscopy helps the clinician to look into the superficial skin layers, and hence allows a more detailed study of the underlying vasculature.
Nailfold capillaries were first described in the 17th century using a primitive magnifying lens, and correlation between inflammation and capillary changes were made in the early 19th century. In the 21st century, after the introduction of modern digital equipment and evidence-based medicine, capillaroscopic technique gained significant popularity.
In systemic diseases with microvasculature damage as a prominent feature, abnormalities of nailfold capillaries can be appreciated before the onset of clinical disease. In patients with such clinically manifest diseases, changes in the nailfold capillaries may reflect internal organ involvement and helps the clinician to determine the stage of the disease. Thenceforth, nailfold capillaroscopy become an important diagnostic tool for diagnosing progressive connective tissue diseases such as systemic sclerosis, mixed connective tissue disease, lupus erythematosus, dermatomyositis, and polymyositis, and also in patients with Raynaud's phenomenon.
However, nailfold capillaroscopy is a nonportable device, and it demands considerable technical skills and training of personals. These factors limit its use in clinical practice. Recently, dermoscopy has shown to be an effective tool in assessing the capillary network of nail folds. In this article, authors have studied the precision of a dermoscope as a capillaroscopy in evaluating nailfold capillaries in various connective tissue diseases in the skin of color.
| Materials and Methods|| |
This study was conducted in a tertiary care hospital in South India from January 2017 to June 2017. Institutes ethical clearance was obtained and informed written consent were taken from the patients. It was a cross-sectional pilot study. Sixteen consecutive patients with connective tissue diseases were included in the study. Connective tissue diseases comprised of systemic sclerosis, lupus erythematosus, mixed connective tissue disease, dermatomyositis, and rheumatoid arthritis. The study included both the newly diagnosed as well as known cases on or off treatment. Patients with overlap syndrome, history of diabetes mellitus, hypertension, malignancy, atopic dermatitis, psoriasis, and patients who were smokers were excluded from the study. Patients with any type of skin lesions on the nail unit are excluded from the study. Systemic examination was done and relevant blood investigations and including skin biopsy were carried out to confirm the diagnosis. Statistical analysis of collected data was done and presented in the percentages.
A manual DermLite 3 (3Gen, San Juan Capistrano, CA, USA) dermoscope attached to a Sony (Cyber-Shot DSC-W800, Sony Electronics Inc., San Diego, California, USA, digital, 14 megapixels) camera was employed. All the participants were asked to avoid caffeine consumption for at least 5 h before the test. Before capillaroscopic examination, the patients were asked to clean their fingernails with a soft brush to remove any dust particles. The patient was then asked to rest in an environment of temperature 23°C–25°C for at least 15–20 min before commencing the procedure. Polarized version was used for examination on nailfold capillaries. Ultrasound gel was put on the proximal nail fold, and the face plate was held gently so that blood vessels were not blanched. Ring and middle finger were selected for the examination. Alternatively, other fingers were also examined. Fingers were positioned at the level of heart while examining through the dermoscopy. The patterns were documented with the help of digital camera.
The following patterns were taken as references to evaluate the nailfold capillaries patterns under dermoscopy, (i) normal pattern (homogeneous aligned capillary plexus, with a mean linear density of 30 capillaries per 5 mm, with no morphological alterations); (ii) nonspecific scleroderma pattern; and (iii) scleroderma pattern which includes two or more of the following: enlarged capillaries, hemorrhages (more than two punctuate hemorrhages per finger or confluent hemorrhage areas), disorganization of the normal capillary distribution, moderate or extensive capillary loss (i.e., avascular areas), and tortuous, crossed, and/or ramified capillaries.
| Results|| |
Out of 16 (14 females and 2 males), three (18%) had systemic sclerosis, 2 (12.5%) had systemic lupus erythematosus, and 3 (18%) patients were having discoid lupus erythematosus. Mixed connective tissue disease, dermatomyositis, and rheumatoid arthritis were observed in 1 (6%), 1 (6%), and 5 (31%) patients, respectively. One patient was diagnosed with Rowell syndrome. Age of study population ranged from 19 to 55 years. Among the 16 patients, the scleroderma pattern was observed in 12 (75%) of patients, 2 (12.5%) patients had nonspecific scleroderma pattern, and 2 (12.5%) patients had normal nailfold capillary changes.
The most common pattern observed was giant capillaries which was seen in 13 (81.25%), disorganization of the capillary distribution was seen in 12 (75%), tortuous capillaries were seen in 11 (68.75%); followed by microhemorrhages which was seen in 7 (43.75%) and reduction in number of capillaries seen in 6 (37.5%) patients.
In systemic sclerosis, avascular areas and dilated loops were observed. Moreover, in early stages, there was no much change except for the dilatation of loops. In lupus erythematosus, number of capillaries was normal, but for the loops, there was hugely enlarged. Paucity of capillaries and enlarged and tortuous capillaries were noted in mixed connective tissue disease. In dermatomyositis, capillaries were enlarged and tortuous with thrombosed loops and reduced visible capillaries. In Rowell syndrome, scarcity of capillaries with microhemorrhages was noted. In rheumatoid arthritis, capillaries were normal in number but were irregular and slightly enlarged. Dermoscopic patterns of nailfold capillaries in various connective tissue diseases are depicted in [Table 1].
| Discussion|| |
Capillaroscopy is a noninvasive, bedside, diagnostic technique designed to evaluate the small vessel morphology. Over the years, magnifying glasses to microscopes of varying powers have been used to observe capillary changes. Videocapillaroscope which is considered as the gold standard instrument has 100–200 magnification with a monitor attachment allowing the physician to store the image with specific algorithmic processing giving detailed observations of individual capillaries., Dermoscope had been devised to study skin tumors but recently had gained popularity in all the conditions in the dermatologic spectra. Moreover, the dermoscopy is quicker to perform, and the light instrument makes it a boon for the physician.
In normal individuals, nailfold capillaries exhibit a regular, hairpin or U-shaped arrangement with homogeneous shape, size, and distribution [Figure 1].
|Figure 1: Nailfold capillaroscopy in healthy individual: hair-pin shaped or U-shaped capillaries are seen running from the proximal nail fold. Note the regularity in shape, size, and distribution of capillaries (×10, DermLite 3, polarized mode)|
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Systemic sclerosis is a rare connective tissue disease presenting with diffuse fibrosis and dysfunction of internal organs due to microangiopathy. Furthermore, there is the destruction of nailfold capillary pattern because of vasculopathy.
The patterns described in systemic sclerosis are:
- In early scleroderma, irregularly enlarged capillaries with a few giant capillaries and hemorrhages are seen, and regular capillary architecture is maintained
- In active scleroderma, frequent giant capillaries and hemorrhages with mild loss of capillaries. Disorganization of capillary architecture is also noted
- In late or evolved scleroderma, there is severe loss of capillaries with few giant capillaries, avascular areas with capillary architecture disorganization, and ramified capillaries are seen.
In three patients of systemic sclerosis, the first patient had very few enlarged capillaries and well-preserved capillary distribution which is suggestive of early scleroderma pattern [Figure 2]. The second had frequent giant capillaries, frequent hemorrhages, with moderate loss of capillaries, and mild disorganization of capillary architecture which indicated active scleroderma pattern of the disease [Figure 3]. Moreover, the third one showed few or absent giant capillaries and hemorrhages, severe loss of capillaries with extensive avascular areas, and disorganized capillary architecture which indicated late scleroderma pattern of systemic sclerosis [Figure 4] and [Figure 5]. These patterns were consistent with early, active, and late pattern as described by Cutolo et al., and in this study, stage of disease was proven by serological profiles. This pattern is 76.9% sensitive and 90.9% specific. Specificity increases when combined with serology such as antinuclear antibodies.
|Figure 2: Dermoscopy of nailfold capillaries in systemic sclerosis: (a) Enlarged capillaries (black circles), preserved capillary plexus are noted; (b) Note disorganization of nail capillary plexus and avascular areas (black stars); (c) Giant capillaries (yellow circles), few hemorrhagic spot (blue arrows) are noted; (d) Many avascular areas (black stars), few hemorrhagic spots (yellow arrows), bushy capillaries (black arrows), and disorganization of architecture and loss of capillary plexus are well appreciated (×10, DermLite 3, polarized mode)|
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|Figure 3: Dermoscopy of nailfold capillaries in systemic lupus erythematosus: prominent subpapillary with tortuous capillaries (black arrows) is well appreciated. Note the meandering of capillaries (yellow arrows) (×10, DermLite 3, polarized mode)|
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|Figure 4: Dermoscopy of nailfold capillaries in mixed connective tissue disease: It demonstrates scleroderma pattern consisting of dilated and enlarged capillary loops (circles) with avascular areas (stars) (×10, DermLite 3, polarized mode)|
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|Figure 5: Dermoscopy of nailfold capillaries in dermatomyositis: Panels (“a” and “b”) reveal avascular areas (black star), and bushy (black circles). Note the elongated capillaries and hemorrhagic spots (yellow arrows) (×10, DermLite 3, polarized mode)|
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It is shown that sclerodermatous changes can be seen as early as 6 months before onset of clinical symptoms. Hence, nailfold capillaries is a helpful method to prognostication and helps in early diagnosis of scleroderma. As the authors did not find any difference in the near field communication patterns in the skin of color compared to Fitzpatrick skin types I–III, they are of the opinion that dermoscopic nailfold capillaries patterns could be considered as additional diagnostic criteria for systemic sclerosis.
Out of the five patients (two patients systemic lupus erythematosus and three discoid lupus erythematosus), three patients showed normal nailfold capillaries pattern while in the rest two patients, tortuous capillaries with a prominent subpapillary plexus which is referred to as “systemic lupus erythematosus capillaroscopic pattern” was observed. Meandering of capillaries was typically seen in one patient [Figure 6]. However, authors could not find the classical scleroderma pattern in any of the patients in this study. Thus, in systemic lupus erythematosus, the nailfold capillaries pattern is often normal. Bergman et al. observed scleroderma pattern in 1 out of 22 systemic lupus erythematosus patients. Hence, scleroderma pattern is unusual in lupus erythematosus, and nondiagnostic patterns with meandering of capillaries are quite characteristic of lupus erythematosus. It should be noted that patterns of nailfold capillaries were indiscernible in skin of color form skin types 1–3.
|Figure 6: Dermoscopy of nailfold capillaries in Rowell syndrome: multiple hemorrhagic spots (arrows) and microhemorrhages (circles) are seen (×10, DermLite 3, polarized mode)|
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Mixed connective tissue disease
Mixed connective tissue disease is a clinical diagnosis comprising of systemic sclerosis, dermatomyositis, rheumatoid arthritis, and systemic lupus erythematosus. Dermoscopy demonstrated the pattern similar to scleroderma with paucity in number of capillaries with dilated and enlarged capillary loops [Figure 7]. Scleroderma pattern was most commonly observed up to 50% of patients in a study. In this study, single patient showed scleroderma pattern. Nevertheless, the skin of color had no influence and authors could not notice difference between different skin types in the patterns of nailfold capillaries in mixed connective tissue disease.
|Figure 7: Dermoscopy of nailfold capillaries in rheumatoid arthritis: It demonstrates increased capillary tortuosity (yellow arrows) and enlargement (black arrows) of capillaries (×10, DermLite 3, polarized mode)|
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In dermatomyositis, nailfold capillaries depicted grossly enlarged and tortuous capillary loops with multiple hemorrhagic spots. This pattern is not specific for dermatomyositis as it can be seen in systemic sclerosis as well. However, it has a great prognostic value in dermatomyositis. Increased microhemorrhages and elongated capillaries are well correlated to myalgia and arthralgia, respectively. Avascular areas and budding or bushy capillaries which suggest ischemia and revascularization were also seen. These are observed most frequently in dermatomyositis. Nevertheless, they are also seen in systemic sclerosis. It is affirmed that homogeneously enlarged capillaries is typical and characteristic pattern of mixed connective tissue disease, scleroderma, and dermatomyositis.
It should be noted that dermatomyositis and polymyositis are distinguishable based on nailfold capillaries findings. In polymyositis, capillary changes are minimal and comparable to the healthy population. This is in accordance with pathogenic mechanism of an autoimmune response of cytotoxic T cells and due to mere absence of microangiopathy which is characteristic of dermatomyositis.
Due to small sample size, avascular areas were not found in this study. Hence, disease activity and nailfold capillaries changes could not be correlated. There was no difference in nailfold capillaries patterns between the skin of color and other skin types.
Rowell syndrome is a rare autoimmune disease with systemic lupus erythematosus, erythema multiforme-like lesions and immunologic serum abnormalities such as speckled antinuclear antibody pattern.
The patient with Rowell syndrome in this study showed dilated capillary loops with significant multiple hemorrhagic spots on nail capillaroscopy.
To the best of our knowledge, it is a first attempt to describe nail capillary changes in Rowell syndrome using a dermoscope.
Rheumatoid arthritis is a chronic inflammatory autoimmune disease with extensive extra-articular involvement. Rheumatoid arthritis, especially, the seronegative type, and psoriatic arthritis present similarly and pose a diagnostic challenge. In this study, a high frequency of increased capillary tortuosity with dilated loops was observed. However, the number of capillaries remained normal without hemorrhagic spots. The subpapillary venous plexus was significantly and prominently visible in all the cases of rheumatoid arthritis.
Finding in this study was similar to that of a study by Altomonte et al., wherein increased tortuosity and elongated capillary loops were the main changes in rheumatoid arthritis on nail capillaroscopy. Angiogenesis was noted in addition to increased tortuosity of capillaries in a study by Rajaei et al. This particular pattern was not observed in this study. This is probably due to small sample size. Capillaroscopy is also a useful tool for predicting the development of visceral complication and digital ulceration in systemic diseases. There was no distinction in nailfold capillaries patterns in rheumatoid arthritis between different skin types.
| Conclusion|| |
Dermoscopy is a noninvasive, rapid, easy, cost-effective method for studying nailfold capillaries in various connective tissue disorders. In this study, the results obtained using hand-held manual dermoscopic nailfold capillaroscopy were qualitatively satisfactory for clear delineation of the nailfold capillaries features. Hence, the authors believe nailfold dermoscopy is a good cost-effective alternative to videocapillaroscopy. It aids in recognition of alternations in nailfold capillaries making early diagnosis of connective tissue diseases and thus preventing morbidities and sequelae of connective tissue diseases. Importantly, the skin of color was not influential in nailfold capillaries pattern in this study. Therefore, dermoscopic patterns of nailfold capillaries in various connective tissue diseases can be considered in the present diagnostic criteria. Authors recommend further studies involving large sample size of the population with skin of color to affirm the nailfold capillaries pattern observed in this study. The limitations in this study were small sample size, subjective interpretation of the observer, and no follow-up during disease.
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Conflicts of interest
There are no conflicts of interest.
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