The pathogenesis, diagnostic utility and clinical relevance of cutaneous telangiectasia in systemic sclerosis

Abstract

Cutaneous telangiectasia (Tel) are visible permanently dilated postcapillary dermal venules and are one of the most common disease-specific manifestations of systemic sclerosis (SSc). SSc-Tel have long been recognised for their utility in the diagnosis and classification of SSc, but the clinical and prognostic relevance of these aberrant cutaneous vascular manifestations has been somewhat neglected by clinicians. Similarly, the impact of SSc-Tel on body image dissatisfaction and social discomfort has been under-appreciated. The paucity of evidence-based approaches to management has limited access to potential effective treatments for SSc-Tel. The present review examines the pathogenesis, diagnostic value, impact and clinical relevance of telangiectasia in SSc. We highlight the potentially overlooked prognostic value and clinical utility of SSc-Tel, as part of a broader appraisal of areas of unmet research need.

Introduction

Systemic sclerosis (SSc) is a rare, multisystem, autoimmune connective tissue disease (CTD) characterised by immune dysfunction, aberrant fibrotic processes, and vascular injury (1). Immune-mediated endothelial injury is considered an important initiating event in SSc, and tissue hypoxia is thought to contribute to aberrant tissue remodelling and disorganised neoangiogenesis. The excessive deposition of collagen within the dermis of many patients resulted in the historical appellation “scleroderma” and the extent of skin involvement is central to the traditional classification into limited (lcSSc) and diffuse cutaneous (dcSSc) subsets (2). Vascular manifestations such as Raynaud’s phenomenon (RP) are omnipresent and an important requisite to diagnosis and classification of early disease.

A comparatively neglected cutaneous vascular manifestation common across the SSc disease spectrum are telangiectasia. Telangiectasia (Tel) are visible permanently dilated dermal postcapillary venules that blanch under pressure (3). The term is a portmanteau of the Greek words ‘telos’ meaning end, ‘angeion’ meaning vessel and ‘ektasis’ meaning dilatation. Telangiectasia are seen in a variety of diseases and can vary in morphology accordingly. ‘Matted, non-stellate’ telangiectasia are the typical lesions found in SSc (4) . Spider naevi, described as a red dot with fine vessels radiating from the centre are seen in liver disease, pregnancy and associated with oral contraceptive use (5). In hereditary haemorrhagic telangiectasia, they are described as pink to red ‘pinpoint to pinhead’ sized lesions (6). The clinical relevance of telangiectasia has been largely overlooked by clinicians historically, whose focus has centred on potentially life-threatening internal organ manifestations. The importance to patients and potential value to clinicians of SSc-Tel as a prognostic and potential surrogate marker of organ involvement has potentially been under-estimated. The purpose of the present review is to consider the pathogenesis, diagnostic value, impact and clinical utility of SSc-Tel. Where relevant, we shall highlight the potentially overlooked prognostic value of SSc-Tel and other areas of unmet research need.

Search Strategy and Selection Criteria

We set out to undertake a comprehensive scoping review of the literature underpinned by a robust search strategy. The references were identified through a search in PubMed (1^st March 2024) using the following search terms: ((systemic sclerosis) OR (CREST) OR (scleroderma)) AND (Telangiect*). No language or date restrictions were placed on the search. Of the 780 studies identified, the titles and abstracts were reviewed by two reviewers for inclusion in the review based on their relevance and originality. In general, case reports and non-original research studies (such as reviews, editorials, letters etc.) were excluded. Additional cited manuscripts were identified through searches of the author’s own files. A visual representation of the search strategy is presented in Figure 1.

Figure 1.

Flow chart depicting search strategy for review (86).

Pathogenesis of telangiectasia in SSc

Cutaneous telangiectasia in SSc are dilated post-capillary venules (occurring in the absence of neoangiogenesis) within the papillary or superficial reticular dermis (7–9). The pathogenesis of telangiectasia development in SSc has yet to be fully elucidated, but is thought to involve endothelial injury and accelerated endothelial proliferation; features commonly identified within the microvasculature of the dermis in both fibrosed and clinically normal skin in SSc (10). Histological analysis has identified endothelial thickening, but no vasculitis or perivascular infiltrate (8). Outside of the context of SSc, it is accepted that sun damage to cutaneous and subcutaneous tissue is a significant aetiological factor in the appearance of telangiectasia (11), and may contribute in SSc-Tel as evidenced by their distribution in exposed areas of skin of the hands and face. The use of vasodilator therapies in SSc has been noted to be associated with more florid facial telangiectasia, but such observations are subject to bias by indication, rather than causative effects of the treatments themselves (12). A microvascular imaging study of 20 telangiectasia in SSc found that the visible/clinically apparent size of the telangiectasia was not indicative of blood flow at deeper levels of the local surrounding tissue (13).

Diagnostic value of telangiectasia in SSc

The high prevalence and visible nature of SSc-Tel have resulted in them being identified as diagnostic markers of SSc. Telangiectasia were one of the clinical hallmarks of early attempts to define the CREST (Calcinosis, Raynaud’s, oEsophageal involvement, Sclerodactyly, Telangiectasia) syndrome and also featured prominently in the early Barnett classification proposals for SSc (14,15). The identification of autoantibodies targeting kinetochore (anti-centromere) antigens in the sera of SSc patients with prominent vascular features such as SSc-Tel strengthened the adoption of the CREST acronym (16). This acronym is not used in the classification of SSc, and subsequent recognition of the importance of internal organ manifestations across the spectrum of the condition has resulted in its re-classification. Telangiectasia have however retained their place as valuable markers of SSc and are included (achieving 2 of the 9 points required for disease classification) in the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification of SSc (17). They have also been identified as a clinical feature to support a diagnosis of SSc in patients with interstitial pneumonia with autoimmune features (18).

Frequency, distribution and morphology of telangiectasia in SSc

Telangiectasia occur in approximately 80% of patients with SSc, making them the second most common disease-specific manifestation of SSc (19). Reported prevalence estimates have varied between 47% and 92.5% in SSc (20–25). While the majority of studies have identified a similar overall prevalence of any SSc-Tel occurrence in both diffuse (dcSSc) and limited (lcSSc) cutaneous subsets of SSc (3,4,21,26), some have reported higher frequencies in lcSSc patients than diffuse (27). Absolute numbers of SSc-Tel also appear to be higher in lcSSc (24). Consistent with this, one study reported the presence of telangiectasia early in the disease course appears to be associated with patients exhibiting a continuous slow progression of skin fibrosis in SSc (28).

Telangiectasia are typically distributed over the upper extremities, as well as on the face, lips and oral mucosa. Indeed, the presence of buccal mucosal telangiectasia is a very common feature of SSc (56.3%), although their presence was not found to be associated with salivary secretion or capillary abnormalities in labial biopsy specimens (29). The upper chest is often affected but the abdomen is typically spared. SSc-Tel appear to be evenly distributed on the face and upper limbs, with a significant correlation between the number of SSc-Tel on the hands compared with the face (3,24). Others have noted a strong correlation between SSc-Tel count on the hands and face and total SSc-Tel count (23).

SSc-Tel vary in size and structure, ranging from small circular lesions of <1mm in diameter to larger matted SSc-Tel of >5mm in diameter, with patients typically exhibiting a range of different sized SSc-Tel lesions (Figure 2). Attempts to sub-classify SSc-Tel according to their clinical or dermatoscopic appearance have led to the proposal of terms such as “spot” (30,31), “reticular” (30,31), pseudo-tumoral (>5mm in diameter) (21) and “matt” telangiectasia (32).

Figure 2. Telangiectasia in systemic sclerosis.

Examples of different morphological subsets of telangiectasia in systemic sclerosis. A, Widespread matted facial telangiectasia consisting of networks of dilated and intertwined dermal vessels in a patient with lcSSc (anti-U3-RNP antibodies); B, Striking cyanosis of the fingers in the same patient as image A but telangiectasia less prevalent (arrows); C, A different morphological variant of telangiectasia in a patient with dcSSc (anti-RNA polymerase III antibodies) with matted and reticular telangiectasia across the face under dermatoscope D, Matted telangiectasia under dermatoscope, consisting of a network of dilated vessels, on the fifth digit of the same patient as image C.

In a study of 87 patients, 86% had SSc-Tel, 25% were reported to have pseudo-tumoral telangiectasia, and a third of patients had >10 SSc-Tel on their hands and face(21). Another study of 106 patients reported at least 1 pseudo-tumoral telangiectasia in 51.9% with the median number of telangiectasia and pseudo-tumoral telangiectasia 30 (IQR, 82.7) and 1 (IQR, 5) respectively(23).

Quantification of telangiectasia in SSc

Whilst many observational studies have simply documented the presence or absence of telangiectasia (33), a growing number of investigators have attempted to quantify the size and number of SSc-Tel. This has ranged from basic counting of telangiectasia to the development of formal semi-quantitative scoring systems across different body sites (4,21). The semi-quantitative ‘Telangiectasia Score’ (TS) examines the burden of SSc-Tel (0, absent; 1–9, for 1 point and >10 for 2 points) across 11 body sites (maximum telangiectasia score of 22), although some of the sites used are seldom associated with high numbers of telangiectasia (4). A modified version has been devised that focuses on the hands and face alone; with classifications of absent, mild-to-moderate (1–10 on hands or face) or diffuse (>10 on hands or face) (21). Studies utilising the TS to quantify SSc-Tel have reported mean TS between 2.3 and 6 per patient (4,20,23). These scoring systems offer clinicians an additional tool to objectively monitor telangiectasia. As more evidence emerges linking SSc-Tel with other manifestations of SSc, these scores may also offer valuable prognostic insights.

Clinical significance of telangiectasia in SSc

A strong association between disease duration and both the presence and number of SSc-Tel provides evidence of the relationship with the advancing microangiopathy of SSc (3,4,22–24,26). Others have suggested the correlation with disease duration may be limited to SSc patients without a history of digital ulcers (20). Analyses of the Very Early Diagnosis of Systemic Sclerosis EUSTAR (VEDOSS) cohort demonstrated SSc-Tel are more prevalent (19.4%) in those meeting the VEDOSS criteria of SSc-specific nailfold capillary changes and/or the presence of SSc-related autoantibodies than in other ANA positive Raynaud’s patients (8.6%) (34). Higher rates of SSc-Tel have been reported in Caucasian patients when compared to Black and Hispanic patients with SSc (35), although this may be due to difficulty in assessing SSc-Tel in darker skin tones and requires further study. An analysis of a Chinese cohort of SSc patients suggested a higher cumulative burden of SSc-Tel in males over a mean follow-up period of 4 years (36), although sex differences are not consistently reported across the literature (37). Whilst SSc-Tel are non-fatal (notwithstanding the friable telangiectasia-like lesions present in the gastrointestinal tract that can be susceptible to major bleeding), their presence in SSc have been shown to be an independent risk factor for poorer outcomes in SSc (38) and they strongly associate with a number of important disease-specific manifestations of SSc.

Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is the most frequent cause of disease-related mortality in lcSSc. SSc-PAH is typically a late disease manifestation of SSc providing clinicians with the opportunity to screen for and treat PAH promptly, which can improve patient outcomes (39). SSc-PAH occurs in ~11% of patients with lcSSc, with the interval from first symptoms to the diagnosis of isolated pulmonary hypertension being 15–20 years (40). A number of studies have identified a strong association between the presence of SSc-Tel and PAH (4,21,26,41,42). Some studies have suggested a correlation between the presence of SSc-Tel number and PAH confirmed on RHC, as well as increased right ventricular systolic pressure (RVSP) on doppler echocardiography (4,21,23). In contrast, others have indicated that SSc-Tel morphology might also be important. One study of 87 patients found that patients with SSc-Tel >5mm and/or SSc-Tel count of >10 are more likely to have increased systolic pulmonary artery pressure (PAP) on echocardiography and raised plasma troponin levels, however a significantly higher risk of pre-capillary PAH at right heart catheterisation (RHC) was only seen in those with SSc-Tel >5mm(21). Others have observed that the presence in the fingers and the face of smaller telangiectasia of >1 mm in size, is sufficient to confer an increased risk of isolated SSc-PAH (40). The presence of SSc-Tel in the data-driven DETECT algorithm to suggest when to order right-heart catheterisation in suspected SSc-PAH (6) further substantiates this. Taken together, these findings strongly indicate the clinical significance of SSc-Tel in predicting the occurrence of SSc-PAH.

Digital ulcers and pitting scars

Digital ulcers (DU) occur in up to 63% of patients with SSc at some stage in the disease course and are associated with internal organ involvement, overall disease severity and mortality (33,44). The strength of the association between telangiectasia and DU remains less clear (20,21,33,45,46). Like PAH, multiple studies have identified a higher prevalence of SSc-Tel amongst patients with DU (26,33,44,47), with two studies reporting an independent relationship between DU and SSc-Tel on multivariate analysis after correcting for relevant confounders such as disease duration and serology (33,44). The relationship between DU and SSc-Tel appears to be particularly strong in patients with dcSSc (48). A retrospective analysis of 282 patients over a 10 year period identified a significant association between SSc-Tel and both fingertip DU and ulceration overlying bony prominences of the upper limbs (47). As with PAH, the total number of SSc-Tel appears to increase the likelihood of DU occurrence, with patients with “profuse” SSc-Tel (>10 in total) having a greater risk of past or current active DU (21). Consistent with these findings, SSc-Tel are also associated with the presence of digital pitting scars, particularly amongst patients with the dcSSc subset (49,50). The relationship between SSc-Tel and DU appears to be weaker than for PAH (4,20), suggesting possible differences in mechanistic aspects driving central and peripheral vascular manifestations of SSc.

Cardiac involvement in SSc

Pericarditis and arrhythmia requiring treatment are more prevalent amongst patients with SSc-Tel (50). Another study identified an independent association between left ventricular diastolic dysfunction and the presence of SSc-Tel, although this was not associated with increased mortality (51).

GI/hepatic associations

Primary biliary cirrhosis (PBC) can occur alongside lcSSc (typically in association with anti-centromere antibodies) (52). Clinical features associated with lcSSc-PBC overlap include the presence of SSc-Tel and calcinosis, with oesophageal dysmotility showing a trend but not reaching significance (52). A 2023 study demonstrated an association between slow colonic transit and the presence of SSc-Tel (53). Other possible gastrointestinal associations include shortening of the sublingual frenulum amongst patients with SSc-Tel (50) and Gastric Antral Vascular Ectasia (GAVE) (54).

Other associations

Recent work demonstrates poor quality of sleep and sleep apnoea to be higher in those with SSc-Tel in univariate analysis (55).

Psychosocial Impact

Whilst not associated with physical symptoms or directly resulting in organ damage, the emotional impact of SSc-Tel can be significant and is often overlooked by clinicians. Telangiectasia can be disfiguring and be a major cause of body image dissatisfaction, particularly when they occur in prominent areas such as the face (Figure 3). Telangiectasia can reduce confidence and difficulty in maintaining a healthy body image and in turn lead to distress and anxiety (56). Studies have found that the extent of skin involvement and the presence of upper body SSc-Tel was associated with self-image dissatisfaction and social discomfort, with feelings of ‘being uncomfortable with strangers’, ‘being unattractive to others’ and ‘that others wouldn’t want to touch me’ (56). A study of 141 SSc patients found that ‘dissatisfaction with appearance’ scores were significantly higher in those with SSc-Tel (45). Further exploration of the specific impact of SSc-Tel identified changes in patient behaviour, a major impact on public and private self-image, a negative emotional impact and an impact on the appreciation of life (45).

Figure 3. Impact of telangiectasia on body image dissatisfaction in systemic sclerosis.

A, Widespread facial telangiectasia in a Caucasian male with diffuse cutaneous systemic sclerosis (anti-U3-RNP antibodies). B, Response to the brief SWAP score questionnaire. The patient expressed the impact of the widespread telangiectases recalling “… meeting people for the first time I am very self-conscious of how I must appear to them and feel uncomfortable by the way they try not to stare at my face…”.

Relationship between telangiectasia and biomarkers in SSc

Autoantibodies

The strongest SSc-specific antibody association with SSc-Tel is with anti-centromere antibodies (57–59), even following adjustment for disease duration (57). The association between SSc-Tel and poorer outcomes in SSc is of particular concern given anti-centromere antibodies generally confer a lower mortality risk in SSc (38). Studies have also identified a strong association between SSc-Tel and the presence of antibodies targeting TRIM-21/Ro 52 (60). Given the strong association between PAH and the presence of antibodies targeting U3 ribonucleoprotein-associated fibrillarin, it is unsurprising that such patients are also often found to have SSc-Tel (61).

Microvascular imaging

Morphological assessment of the nailfold capillaries allows direct visualisation of the evolving microangiopathy of SSc. Advanced disease is associated with progressive capillary drop-out and disorganised neoangiogenesis, which can be qualitatively and (semi)-quantitatively graded (21,30,62). An analysis of 87 SSc patients found an association between the ‘late’ NVC pattern (characterised by significant reduction in capillary density) and both the number of SSc-Tel (those with >10, labelled ‘profuse Cutaneous Telangiectasia (CT)’) and their size (>5mm, termed pseudo-tumoral telangiectasia), after adjustment for potential confounders (21). Disorganised neoangiogenesis was also more frequent in patients with profuse or pseudotumoral CT (21). Others have not only identified a relationship between the number of SSc-Tel and the ‘late’ NVC pattern, but have furthermore reported a specific association between this ‘late’ NVC pattern and the presence of SSc-Tel of particular morphology including reticular (reflecting angiogenesis) and spot (reflecting giant capillaries and single dilations) telangiectasia (30). The absence of SSc-pattern nailfold capillary changes meanwhile, are associated with a lower frequency of SSc-Tel (63). Taken together, these findings strongly suggest that the development of SSc-Tel are related to progression of the obliterative microangiopathy of SSc.

Other circulating biomarkers

The relationship between a large number of putative circulating vascular biomarkers and the presence of SSc-Tel has been explored across a number of studies; the findings of which are summarised in Table 1. The majority of these studies have been comparatively small (14/18 involving fewer than 100 subjects). Nonetheless, these biomarkers, involved in angiogenesis, inflammation, coagulation and wound repair may be relevant to the formation of SSc-Tel and provide novel therapeutic targets for telangiectasia prevention in SSc (Table 1).

Table 1.

Summary of findings exploring relationship between putative biomarkers and telangiectasia formation in systemic sclerosis

Author & Year	Subjects	Biomarker	Main function(s)	Summary of key findings
Bassyouni 2011(70)	47 SSc 38 HC	CD36	Anti-angiogenic protein	CD36 levels significantly higher in SSc patients with telangiectasia vs those without
Fujimoto 2006(71)	70 SSc 20 SLE 20 HC	Endoglin	Angiogenesis	Patients with elevated serum Endoglin levels had telangiectasia more frequently. Positive correlation between with estimated pulmonary arterial pressure and endoglin levels in patients with lcSSc
Shah 2010(4)	11 SSc	Endoglin	Angiogenesis	The positive correlation between endoglin levels and telangiectasia score achieved borderline statistical significance in this small sample
Jinnin 2010(72)	42 SSc 10 RP 22 HC	KDR (aka VEGF R2)	Angiogenesis	Increased KDR levels were associated with higher SSc-Tel in females
Jouvray 2018(23)	106 SSc	Soluble endoglin & VEGF	Angiogenesis	Total telangiectasia number associated with soluble endoglin levels but not VEGF
Niazy 2020(73)	42 SSc 27 HC	α -Klotho	Angiogenesis	Serum α-Klotho levels significantly lower in SSc patients when compared to HC, but patients with SSc-Tel had significantly higher serum α-Klotho levels than those without SSc-Tel in the SSc cohort
Hamaguchi 2008(74)	92 SSc 14 SLE 20 HC	IGF-1 + IGFBP-3	Immune cell activation	SSc-Tel less frequent in SSc patients with elevated IGF-1 and IGFBP-3 levels
Wastowski 2009(75)	Skin: 21 SSc 28 HC	HLA G	Immune cell activation	HLA-G expression was correlated with a lower frequency telangiectasia
Taniguchi 2017 (76)	Skin: 5 SSc Serum:57 SSc	CXCL6 Fli1	Immune cell activation & angiogenesis	Serum CXCL6 levels higher in patients with SSc-Tel
Taniguchi 2018(77)	56 SSc	CXCL13	Immune cell activation & angiogenesis	Serum CXCL13 elevated in SSc patients and associated with presence of SSc-Tel
Sundblad 2021 (78)	83 SSc	(Gal)-1 and Gal-3	Immune cell activation & fibrosis	Higher Gal-1 and Gal-3 values associated with SSc-Tel
Louthrenoo 2011(79)	50 SSc 99 HC	CTGF*−945C/G	Pro-fibrotic	No association of the CTGF*−945C/G alleles with any SSc clinical manifestations including SSc-Tel
Wu CY 2016(80)	Serum: 65 SSc 20 HC	Syndecan-1	Wound healing, inflammation & vascular biology	SSc-Tel more frequently seen in patients with elevated syndecan-1 levels. Elevated syndecan-1 level also associated with raised RVSP and decreased DLCO
Hirabayashi 2018(81)	Skin: 7 SSC 5 HC; Serum: 51 SSc,20 HC	HB-EGF	Wound healing	Serum HB-EGF significantly higher in those with telangiectasia
Takahashi 2018(82)	Skin:10 SSc, 5 HC Serum:51 SSc,19 HC	Psoriasin	Epidermal remodelling	Presence of telangiectasia associated with higher psoriasin levels
Smolenska 2020 (83)	42 SSc 27 HC	Amino acids	Protein synthesis	Patients with SSc-Tel have a higher concentration of glutamate, lysine, and L-NAME (a NOS inhibitor)
Norimatsu 2021 (84)	67 SSc 20 HC	TFPI	Coagulation	Serum TFPI levels were significantly lower in patients with SSc-Tel
Balanescu 2015 (85)	35 SSc 20 HC	IL-17	Cytokine	When controlled for age and gender, those with SSc-Tel have higher IL-17 levels

Fli-1, Friend leukemia virus integration 1; HC, healthy controls; HB-EGF, Heparin-binding epidermal growth factor; KDR, Kinase insert domain receptor; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; TFPI, Tissue factor pathway inhibitor; VEGF, Vascular endothelial growth factor; IL-17, Interleukin-17.

Management of telangiectasia in SSc

For some patients, the use of concealment approaches, such as make-up camouflage, may provide an acceptable long-term approach to management of SSc-Tel. For others, attempts at eradication using ablative techniques such as injected sclerosing agents or thermocoagulation methods such as pulsed dye laser (PDL) therapy would be more desirable (64,65). The limited evidence-base for such treatments, alongside a misguided perception that such treatments are purely cosmetic, have limited access to and reimbursement for these therapies. In a study of 19 SSc patients who received both PDL and intense pulsed light (IPL) treatment, with treatment randomly assigned to left and right-sided lesions, both were found to be effective for SSc-Tel (65). PDL appeared to have better outcomes in terms of appearance, although IPL had fewer side effects (65). Another study of 21 patients found that PDL to facial and neckline SSc-Tel significantly reduced their number with a mean decrease of 5 (32%), and that this corresponded with an improvement in their quality of life assessment score; although there was no long term follow up data (66). Recently, blue-light laser has been used in telangiectasia treatment, although not in SSc patients. One small study found 6/6 patients with facial telangiectasia saw in improvement with blue-light laser, but no improvement in lower limb telangiectasia(67). This requires further study within SSc-Tel.

Patients should be counselled on the high rate of recurrence of SSc-Tel following ablative approaches. Treatment efficacy following interventions for telangiectasia generally compare before and after photographs, rating improvements using ordinal scales ranging from ‘much worse’ to ‘much improved’ (64,65). Other studies have reported lesion size and extent of lesion clearance (8). These clinician assessment tools have been used alongside non-invasive imaging approaches such as laser Doppler and dermatoscopy (64,65). Surprisingly, the patient perspective has been less often captured, although patient-reported outcome instruments such as an Adapted (Brief) Satisfaction With Appearance Scale (SWAP)(68) and SKINDEX score(69) (see supplementary material) have been used to measure treatment efficacy and impact on quality of life following laser therapy (65,66). Additional work to explore the patient experience of SSc-Tel could identify domains that patients find important in terms of telangiectasia burden and treatment success (reduction in size, eradication in specific body areas etc.).

Systemic vasodilator therapy has not been shown to prevent the development of SSc-Tel. One observational study of 27 SSc patients treated with either with Bosentan or Iloprost reported a higher frequency of centrofacial SSc-Tel at 10 months amongst patients treated with Bosentan (12).

Conclusions

While SSc-Tel do not cause physical symptoms or directly contribute to mortality, their association with organ-specific manifestations of SSc and direct contribution to disease morbidity is significant. The clinical utility of SSc-Tel as prognostic markers for disease progression and clinical phenotype has been largely overlooked and requires further evaluation. These visible and disfiguring vascular malformations have traditionally been afforded greater significance by patients than by their healthcare professionals. In addition to their role in diagnosis and classification, SSc-Tel are attracting increasing recognition for their prognostic value and deserve recognition as evidence of ongoing vascular injury and damage. Patients require better access to effective treatments for SSc-Tel, and it should be hoped that future treatment approaches in SSc may effectively halt the development of these harbingers of vascular injury and disease progression in SSc.

Key Messages:

• Telangiectasia are common manifestations of systemic sclerosis and useful for diagnosis and classification

• Telangiectasia are associated with disease-specific manifestations of SSc including pulmonary arterial hypertension and digital ulcers

• Telangiectasia in SSc contribute to body image dissatisfaction and impaired quality of life