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Recently, dermoscopy has begun to be used for the observation of nailfold capillaries in connective tissue disease (CTD). However, dermoscopic features of other skin lesions and the utility of such information remains unclear. In this review, we summarize the typical dermoscopic findings of nailfold capillaries in CTD and discuss their significance. We compared the findings between dermoscopy and video capillaroscopy, and propose that dermoscopy could serve as a substitute to some extent for video capillaroscopy. The utility of dermoscopy for othe skin lesions of CTD remains unknown. However, dermoscopy findings may help to differentiate discoid lupus erythematosus from other skin disorders in patients with systemic lupus erythematosus. Interestingly, telangiectasia found in the skin other than nailfold resembles the nailfold capillary changes in patients with systemic sclerosis. Gottron’s sign accompanied with punctate hemorrhage may reflect the existence of rapidly progressive interstitial pneumonia. We propose that daily use of dermoscopy could improve the clinical care of CTD patients, since it enables the recognition of vascular structures and other subtle features that are less visible to the naked eye. We hope that this review will promote increased use of dermosopy for clinical practice in patients with CTD, and believe that further investigation will yield additional valuable information in the near future.
Connective tissue disease (CTD) causes various skin lesions that are composed of vascular tissue and inflammation. Dermoscopy has been widely used in differentiating malignant skin disorders. However, dermoscopy may be useful for evaluating nonpigmented skin disorders, since it provides an improved view of vascular structures and other subtle features that are usually not visible to the naked eye (1-4).
Much work has been done to show that nailfold video capillaroscopy(NVC) can distinguish the Raynaud’s phenomenon associated with scleroderma spectrum disorder (SSD; systemic sclerosis (SSc) and its related diseases) from primary Raynaud’s phenomenon (Raynaud’s disease) (5-8). Distinct NVC patterns are also be useful in evaluating the severity and stage of SSD microvascular damage. Furthermore, NVC changes are as prevalent and as prominent in dermatomyositis (DM) as in SSD (9, 10). However, such findings are not found in patients with other connective tissue diseases. Routine use of NVC at the bedside has yet to become fully integrated into standard clinical practice, since the equipment is relatively expensive and not easily transported. Recently, it has been suggested that dermoscopy can replace NVC, to some extent, for detection of representative nailfold capillary abnormalities (11-15). However, dermoscopic finding and its significance of nailfold capillaries have not been summarized yet.
Furthermore, there are few studies regarding the use of dermoscopy for other skin lesions of CTD and an overview of them is missing. In this review, we show some representative dermoscopic findings and pictures of skin lesions in CTD.,
Usage of dermoscopy for nailfold capillaries
Given a patient with Raynaud’s phenomenon and no other symptoms, it is first important to determine whether the patient has “Raynaud’s disease” or “Secondary Raynaud’s phenomenon associated with CTD”. Raynaud’s phenomenon is most frequently found in patients with SSD, and is usually the first symptom of the disease. Therefore, the existence of SSD or other CTD must be determined. Blood examination for antinuclear antibodies, including CTD-specific autoantibodies, enable the most accurate diagnosis. However, NVC findings are also useful for early diagnosis of SSD, especially in patients negative for CTD-specific autoantibodies. When we use dermoscopy for evaluating CTDs the established findings of capillaroscopy are definitely useful. Therefore, we would like to review capillaroscopic findings of CTD before discussing our findings using dermoscopy.
Scleroderma NVC pattern
We used a video capillaroscopy system (Kekkan bijin, Kenkou Kagaku Kenkyu-kai, Co., Ltd, Kyoto, Japan). Diagnostic capillaroscopy patterns are grouped as follows: normal pattern, scleroderma pattern, and nonspecific pattern(12). The normal pattern in Figure 1A. shows homogeneous capillary distribution in the nailfold plexus without capillary loss (normal linear density: 30 capillaries per 5 mm) and no morphological alterations. The scleroderma pattern in Figures 1B-D is defined according to Maricq et al. (16, 17), with modifications according to Bergman et al. (13).Two or more of the following abnormalities are observed:①enlarged capillaries (Figures 1B and C);②hemorrhages (more than two punctuate hemorrhages per finger or confluent hemorrhage areas)(Figure 1C);③disorganization of the normal capillary distribution (Figure 1B-D);④moderate or extensive capillary loss (avascular areas)(Figure 1 D), and ⑤tortuous, crossed, and/or ramified capillaries (Figure 1D). The nonspecific pattern lacks the complete scleroderma pattern criteria.
Subclassification of scleroderma NVC pattern
The NVC scleroderma pattern is subdivided further as previously reported (18). The subclasses are as follows: (1) Early NVC pattern (Figure 1B): few enlarged/giant capillaries, few capillary hemorrhages, relatively well-preserved capillary distribution, and no evident loss of capillaries; (2) Active NVC pattern (Figure 1C): frequent giant capillaries, frequent capillary hemorrhages, moderate loss of capillaries with some avascular areas, mild disorganization of the capillary architecture, and absence of ramified capillaries; and (3) Late NVC pattern (Figure 1D): irregular capillary enlargement, few or absent giant capillaries, absence of hemorrhages, severe loss of capillaries with large avascular areas, severe disorganization of the normal capillary array, and frequent ramified/bushy capillaries. We have summarized the typical findings from each scleroderma pattern in Figure 1E (19). This subclassification has been useful for evaluation of activity and severity of vascular injury. In severe SSc cases with anti-topoisomerase I Ab present, the NVC pattern progresses quickly to the characteristic Late pattern, within two years of disease onset. On the other hand, mild SSc patients who have anticentromere Ab, gradually progress to the late pattern, more than 20 years after disease onset (20). NVC findings can be improved after treatment of SSc (21)(22).
NVC pattern in other CTD
NVC patterns are also found at high frequency in patients with DM and are generally similar to the patterns found in SSD patients , although the frequency of Raynaud’s phenomenon is much lower. NVC patterns are referred to as a “scleroderma-like pattern” if thepattern is found in DM or disorders other than SSD. Although subtle capillary abnormality such as mild disorganization can be detected in patients with systemic lupus erythematosus (SLE), a typical scleroderma-like pattern is rarely found.
Nailfold capillary findings using dermoscopy
Nailfold capillaries may also be observed using dermoscopy. Although we use several types of dermoscopy, the picutres in this review were taken using dermoscopic camera lens with adaptor (Heine Optotechnik, Herrsching, Germany). We usually choose the third or fourth finger for examination. In a healthy person, we can see homogeneously-lined capillary distribution around the nailfold without hemorrhage (Figure 2A). Most specific NVC findings can also be detected by dermoscopy [11-15]. Our dermoscopic observations of nailfold capillariesare as follows:
1) Disorganization of the capillary architecture
Disorganization of capillary loops can be found in patients with CTD including SSD (Figure 2B~2D), DM (Figure 2E~G), and SLE (Figure 2H).
2) Enlarged/giant capillaries
Enlarged capillaries can be found in patients with CTD-associated Raynaud’s phenomenon. Extremely enlarged capillaries (giant capillaries) are specific for SSD (Figure 2C) and DM (Figure 2F). The enlarged/giant capillaries are considered to be an abnormal angiogenic response, secondary to peripheral ischemia.
3) Capillary hemorrhages