Color Doppler Ultrasonography of digital arteries and digital ulcers development in systemic sclerosis.

BACKGROUND: The aim of this study was to evaluate the role of Color Doppler Ultrasonography (CDUS) of proper palmar digital arteries (PPDA) as predictive marker of new digital ulcers (DUs) in systemic sclerosis (SSc) patients during 5 years follow-up. METHODS: 36 SSc patients were examined using nailfold videocapillaroscopy (NVC) and CDUS of PPDA. RESULTS: Fourteen (38.9%) patients had chronic or acute occlusions (C and D pattern) on CDUS evaluation. Using a cut-off of 0.70, 21 (58.3%) patients had a Resistive Index (RI) ≥0.70. Nineteen (52.8%) patients developed new DUs during the follow-up. The median value of RI was higher in SSc patients with DUs than in SSc patients without DUs [0.73 (IQR 0.70-0.81) vs 0.67 (IQR 0.57-0.70), p < 0.0001]. The Kaplan-Meier analysis showed a free survival from new DUs higher (p < 0.01) in SSc patients with Pattern A and B than SSc patients with Pattern C and D. The Kaplan-Meier curves showed that free survival from new DUs is lower (p < 0.001) in SSc patients with increased RI (≥0.70) than in SSc patients with normal RI. In multivariate analysis with two co-variates, RI ≥ 0.70 [HR 5.197 (1.471-18.359), p < 0.01] and NVC late scleroderma pattern [HR 7.087 (1.989-25.246), p < 0.01] were predictive markers of new DUs. CONCLUSIONS: RI of PPDA in association with NVC could be used to evaluate SSc patients with increased risk of new DUs development.

Evidence of macro- and micro-angiopathy in scleroderma: An integrated approach combining 22-MHz power Doppler ultrasonography and video-capillaroscopy.

OBJECTIVE: We aimed to study in SSc patients macrovascular involvement by using power Doppler ultrasound (PDUS) and microvascular one by PDUS and nailfold video-capillaroscopy (NVC) and to examine the association between history of digital ulcers (HDU) and imaging (PDUS and NVC) parameters. METHODS: NVC and PDUS were systematically performed in 106 consecutive SSc patients at the 3rd and 4th finger of the dominant hand after exclusion of ulnar artery occlusion (UAO). 22 MHz PDUS measurements included nailbed and fingertip qualitatively graded, and resistivity index (RI) of ulnar and radial proper digital arteries. Capillary number/mm was calculated by NVC on the same digits examined by PDUS. RESULTS: Vascularization at fingertip and nailbed showed a good correlation between them and to capillary number. RI, representative of macrovascular involvement, did not correlate to microvascular involvement as assessed by PDUS and NVC. RI and capillary number at NVC showed significant correlation to HDU while fingertip and nailbed vascularization as assessed by PDUS did not. As such, PDUS and NVC provide different and potentially complementary information on SSc-related peripheral macro- and micro-vascular involvement. CONCLUSION: Macro- and micro-vascular involvement in SSc patients are different processes and are not present at the same time in every patient. Thus, both these aspects should be carefully evaluated in SSc patients.

Clinical and dermoscopic characterization of pediatric and adolescent melanomas: Multicenter study of 52 cases.

BACKGROUND: Knowledge regarding the morphologic spectrum of pediatric melanoma (PM) is sparse, and this may in part contribute to delay in detection and thicker tumors. OBJECTIVE: To analyze the clinicodermoscopic characteristics of PM. METHODS: Retrospective study of 52 melanomas diagnosed in patients before the age of 20 years. RESULTS: On the basis of its clinical, dermoscopic, and histopathologic characteristics, PM can be classified as spitzoid or nonspitzoid. The nonspitzoid melanomas (n = 37 [72.3%]) presented in patients with a mean age of 16.3 years (range, 8-20) and were associated with a high-risk phenotype and a pre-existing nevus (62.2%). The spitzoid melanomas (n = 15 [27.7%]) were diagnosed in patients at a mean age of 12.5 years (range, 2-19) and were mostly de novo lesions (73.3%) located on the limbs (73.3%). Whereas less than 25% of PMs fulfilled the modified clinical ABCD criteria (amelanotic, bleeding bump, color uniformity, de novo at any diameter), 40% of spitzoid melanomas did. Dermoscopic melanoma criteria were found in all cases. Nonspitzoid melanomas tended to be multicomponent (58.3%) or have nevus-like (25%) dermoscopic patterns. Spitzoid melanomas revealed atypical vascular patterns with shiny white lines (46.2%) or an atypical pigmented spitzoid pattern (30.8%). There was good correlation between spitzoid subtype histopathologically and dermoscopically (κ = 0.66). LIMITATIONS: A retrospective study without re-review of pathologic findings. CONCLUSION: Dermoscopy in addition to conventional and modified clinical ABCD criteria helps in detecting PM. Dermoscopy assists in differentiating spitzoid from nonspitzoid melanomas.

Detection of severe digital vasculopathy in systemic sclerosis by colour Doppler sonography is associated with digital ulcers.

Objective: Colour Doppler ultrasonography (CDUS) is very important in general vascular diagnostic procedures. Its role in determining the extent of vasculopathy in Systemic Sclerosis (SSc) needs further investigation. The aim of this study was to compare the presence of altered arteries with nailfold capillaroscopy and clinical signs of ischaemia, that is, digital ulcers or pitting scars (DU/PS). A feasible CDUS protocol is provided. Methods: Two thousand five hundred and twenty-eight arteries of the fingers, palms and wrists from 79 SSc patients (32 arteries per patient) were examined using CDUS. Furthermore, nailfold capillaroscopy, clinical and laboratory data were evaluated. Results: Narrowed or occluded lumens were seen in 39.8% of all assessable arteries (n = 2489) and 48.9% of all proper palmar digital arteries (n = 1564) but only 15.6% (P < 0.0001) of proximal arteries (n = 924). Fingerwise analyses presented significant coincidence of pathological CDUS findings and DU/PS (P = 0.0009). Pathological CDUS findings were also associated with elevated CRP concentrations, current or past smoking with ⩾20 pack-years, male gender and present or past DU/PS. Receiver operating characteristic curve analysis (area under the curve = 0.727) suggested a cut-off value of ⩾20% pathological vessels (sensitivity: 90.7%; specificity: 47.8%) for the presence of DU/PS. An examination protocol focusing on the right-hand digits II-V (proper palmar digital arteries) revealed similar results (area under the curve = 0.751; sensitivity: 93.0%; specificity: 43.5%). Conclusion: CDUS of hand and finger arteries allows measurement of the extent of SSc vasculopathy, which is associated with clinical signs of chronic malperfusion. A shortened examination protocol of CDUS (right-hand digits II-V; 15 min instead of 45 min examination time) could complement vascular diagnostics in SSc.

Dermoscopy of Subungual Exostosis: A Retrospective Study of 10 Patients.

INTRODUCTION: Subungual exostosis (SE), the most common nail tumor of young adults, is a benign bony proliferation of the distal phalanx occurring beneath the nail. Misdiagnosis or late diagnosis frequently occurs and no dermoscopy features of this tumor were previously outlined. MATERIAL AND METHODS: To describe the dermoscopic appearance of SE, 10 patients with radiologically and histologically confirmed SE were retrospectively retrieved from our tertiary referral centers. Data regarding age, gender, time to diagnosis, clinical presentation, dermoscopic features, involved nail and history of trauma were recorded for each patient. RESULTS: In our patients, clinical findings were similar to previous reports. Among the dermoscopic features, vascular ectasia was the most common finding (70%), followed by hyperkeratosis (60%), onycholysis (40%), and ulceration (30%). CONCLUSION: Dermoscopy could be a useful technique aimed at creating diagnostic suspicion of this benign nail condition, although X-ray examination and histopathology are mandatory for the diagnosis.

Association between dermoscopic and reflectance confocal microscopy features of cutaneous melanoma with BRAF mutational status.

BACKGROUND: Melanomas harbouring common genetic mutations might share certain morphological features detectable with dermoscopy and reflectance confocal microscopy. BRAF mutational status is crucial for the management of metastatic melanoma. OBJECTIVES: To correlate the dermoscopic characteristics of primary cutaneous melanomas with BRAF mutational status. Furthermore, a subset of tumours has also been analysed for the presence of possible confocal features that might be linked with BRAF status. METHODS: Retrospectively acquired dermoscopic and confocal images of patients with melanoma in tertiary referral academic centres: Skin Cancer Unit in Reggio Emilia and at the Melanoma Unit in Barcelona. Kruskal-Wallis test, logistic regressions, univariate and multivariate analyses have been performed to find dermoscopic and confocal features significantly correlated with BRAF mutational status. RESULTS: Dermoscopically, the presence of irregular peripheral streaks and ulceration were positive predictors of BRAF-mutated melanomas with a statistically significance value, while dotted vessels were more represented in wild-type melanomas. None of the evaluated reflectance confocal microscopy features were correlated with genetic profiling. CONCLUSIONS: Ulceration and irregular peripheral streaks represent dermoscopic feature indicative for BRAF-mutated melanoma, while dotted vessels are suggestive for wild-type melanoma.

Assessment of treatment effects on digital ulcer and blood perfusion by laser speckle contrast analysis in a patient affected by systemic sclerosis.

Laser speckle contrast analysis (LASCA) is a good tool to evaluate the variation in peripheral blood perfusion during long-term follow-up and is able to safely monitor digital ulcer evolution in scleroderma patients. It evaluates blood perfusion in different areas within the skin lesions and surrounding them during standard treatment.

Pilot study assessing pathophysiology and healing of digital ulcers in patients with systemic sclerosis using laser Doppler imaging and thermography.

OBJECTIVES: Systemic sclerosis (SSc)-related digital ulcers (DU) cause significant pain and disability and are often a primary endpoint in clinical trials. However, their pathophysiology has been little studied. The objectives of this prospective study were to determine whether laser Doppler imaging (LDI) and thermography can identify ischaemic components in both fingertip and extensor surface DU and assess ulcer healing. METHODS: Patients prospectively reported new DU over a year. Patients' DU underwent imaging until the ulcer had healed. Ischaemia was defined as lower blood flow or skin temperature (and inflammation as higher) within the ulcer, compared to a non-affected site. RESULTS: 53 ulcers (19 fingertip, 18 extensor, 16 'other' sites) in 17 patients were imaged (53 with LDI, 52 with thermography). For LDI data 32 (60%) ulcers were ischaemic; median perfusion ulcer/unaffected area; 0.79 (range 0.11-2.9). For thermography data 35 (66%) were ischaemic; 0.98 (0.89 to 1.1). Inflammation in the surrounding area was identified for all ulcers by LDI but not thermography. In the 36 ulcers with repeat imaging, LDI showed trends (with healing) towards increased ulcer perfusion (p=0.23) and decreased hyperaemia in adjacent areas (p=0.59). Skin temperature at the ulcer site showed no significant change (p=0.13) but adjacent area showed decreased temperature (p=0.04 signifying decreased blood flow). CONCLUSIONS: LDI and thermography are sufficiently sensitive to measure ischaemia in both fingertip and extensor ulcers. LDI was better suited to monitoring change in perfusion with healing (due to higher imaging resolution, or vascular changes occurring in more superficial skin layers).

[CHANGES IN THE AREA OF THE WOUND SURFACE ON EXPOSURE LOW­DOSE VACUUM].

Results of treatment of were analysed 30 patients over a long non­healing wounds and trophic ulcers, in which as a topical treatment, the method of vacuum therapy was applied. To study the activity of reparative regeneration in the wound using a dynamic method of determining the area of wounds using lqSquare computer program and immunohistochemical method for determining the Ki 67 positive cells in tissues of near wound area. It was found that low­dose exposure to vacuum at the wound contributed to increase the proliferative activity of tissues of near wound area and reduce the area of wound defect. Assessment of changes in the area of the wound surface is an important predictor of treatment efficacy, prevention of complications.

Ulnar artery occlusion is predictive of digital ulcers in SSc: a duplex sonography study.

OBJECTIVES: To assess the prevalence and risk factors of ulnar artery occlusion (UAO) in an unselected SSc patient cohort and to determine whether UAO is associated with digital ulcers (DUs). METHODS: A total of 79 SSc patients and 40 'healthy' controls underwent colour duplex sonography of the radial and ulnar artery to compare blood flow velocity, resistive indices (RIs) and presence of occlusion and were followed for a mean of 53 months. RESULTS: In both, radial and ulnar arteries, peak systolic velocity (PSV) and end-diastolic velocity (EDV) were significantly lower and RI higher in SSc patients compared with controls (PSVrad: 40.1 vs 48.6 cm/s; PSVuln 38.2 vs 56.6 cm/s; EDVrad 3.8 vs 10.4 cm/s; EDVuln 3.0 vs 13.0 cm/s; RIrad 0.91 vs 0.82; RIuln 0.92 vs 0.80; all P < 0.01). Seventeen (21.5%) SSc patients had UAO (11 patients bilateral) compared with none in the control subjects. Patients with UAO had a significantly longer disease duration (170 vs 66 months, P < 0.001). At baseline, the prevalence of DU was not different in upper extremities with UAO [8/28 (28.6%)] compared with upper extremities without UAO [36/129 (27.9%)]. However, during follow-up new or recurrent DU occurred more often in upper extremities with UAO than in those without UAO [14/28 (50%) vs 24/113 (21.2%); relative risk (RR) = 2.4; 95% CI 1.4, 3.7; P = 0.002]. CONCLUSION: Blood flow is significantly decreased in radial and ulnar arteries in SSc. UAO is frequent and an important risk factor for the development of DUs in patients with SSc.

Role of 18F-FDG PET/CT in the diagnosis of clinically suspected Marjolin ulcer.

OBJECTIVE: The purpose of our study is to retrospectively determine the diagnostic role of (18)F-FDG PET/CT at the primary lesion site in burned patients with chronic nonhealing ulcers who are suspected of having Marjolin ulcers. MATERIALS AND METHODS: Thirty-three burn scar contractures with nonhealing chronic ulcer in 28 patients were included in this study. The lesions were sorted into two groups: 22 squamous cell carcinomas and one basal cell carcinoma were assigned to group 1 (Marjolin ulcer), and 10 lesions of chronic ulcer with inflammation and fibrosis were assigned to group 2. The maximum standardized uptake value (SUV(max)) and the lesion thickness and size for the two groups were evaluated. To determine the utility of PET/CT in the evaluation of invasion depth, we compared the imaging findings of PET/CT with surgical or pathologic results and the findings of additional imaging modalities, such as CT or MRI. RESULTS: The SUV(max) and the lesion thickness for group 1 were significantly higher than those for group 2 (p < 0.01 and p = 0.03, respectively). The sensitivity, specificity, and area under the receiver operating characteristic curve were 82.6%, 90%, and 0.900, respectively, for SUV(max) and 65.2%, 80%, and 0.741, respectively, for lesion thickness. PET/CT was helpful and showed quite good correlation with surgical or pathologic results in determining invasion depth. CONCLUSION: PET/CT is useful in differentiating Marjolin ulcer from benign inflammatory conditions of chronic nonhealing ulcer in burn scars. It is also useful in the evaluation of the depth of invasion in Marjolin ulcer cases.

Ischemic skin ulceration complicating glue embolization of type II endoleak after endovascular aneurysm repair.

The optimal treatment for type II endoleaks remains unclear. The present report describes a case of ischemic skin ulceration after glue embolization of a type II endoleak with challenging access in a multiply comorbid 82-year-old woman with an expanding aneurysm sac 3 years after endovascular aneurysm repair. Embolization was performed from a proximal position with an n-butyl cyanoacrylate/Ethiodol mixture to allow flow into the endoleak because direct sac puncture was hazardous. One week after intervention, an eschar, which progressed to superficial necrosis as a result of partial nontarget delivery of sclerosant, developed over the left iliac crest. The eschar was self-limiting, with complete resolution by 6 months.

[Neurotrophic ulceration following thermocoagulation of the Gasserian ganglion]. / Ulcération neurotrophique après thermocoagulation du ganglion de Gasser.

INTRODUCTION: Neurotrophic ulceration (NTU) in the trigeminal nerve sensitive area is rare. It may be caused by a lesion anywhere on the trigeminal nerve's trajectory. The diagnosis is usually clinical, but other diagnoses, particularly neoplastic, must be ruled out first. The physiopathology and treatment of NTU remain controversial. We report a severe case of NTU and describe the main features of this poorly documented disease. OBSERVATION: A 67-year-old female patient consulted in the dermatology department for a progressive ulceration of the nose ala and the right upper lip, having developed over the two previous years. She had undergone two thermocoagulations of the right Gasserian ganglion for facial neuralgia 3 years before. The diagnosis of NTU, initially ruled out because of biopsies suggesting verrucous carcinoma, was finally retained because of the clinical presentation and anamnesis. Treatment consisted in surgical excision and primary reconstruction using a forehead flap. The diagnosis was confirmed after histopathological examination of the surgical specimen. A recurrence was noted 2 years postoperatively, then the patient was lost to follow-up. DISCUSSION: The physiopathology of NTUs is badly documented. The cutaneous ulcerations look like facial neoplasms but the clinical findings (unilateral and paranasal location; lesion of the trigeminal nerve; local trauma; psychological instability) suggest neurotrophic ulceration. The histopathological examination, sometimes difficult, reveals non-specific chronic inflammatory ulceration. There is no consensus on treatment. The psychological profile may be a risk factor for recurrences and must be taken into account.

Ultrasonographic imaging of systemic sclerosis digital ulcers: A systematic literature review and validation steps.

INTRODUCTION/BACKGROUND: Skin ulcers are a complex array of clinical manifestations of systemic sclerosis (SSc) and are often difficult to treat. However, the definition of SSc-skin ulcers has to date not been promising, demonstrating poor reliability and accuracy. There are emerging data that ultrasound has significant potential to evaluate SSc-skin ulcers. OBJECTIVE: To perform a systematic literature review (SLR) to understand the degree to which ultrasound of SSc skin ulcers has been validated according to OMERACT criteria. METHODS: In a SLR, we investigated the Cochrane Library, Web of Science and Pubmed databases for manuscripts from inception to 1st April 2020. Inclusion and exclusion criteria included manuscripts on SSc patients aged over 16 years, performing SSc-related skin ulcer evaluation with ultrasound machines. Papers on animal model, diseases other than SSc, venous ulcers were excluded. Data extraction used a uniform case report form which collected data on patient demographics, disease activity, description of the ultrasound machine and procedures and the degree to which domains of validity were fulfilled. Manuscript evaluation and extraction was performed by two independent assessors, with a third author being consulted in case of disagreement. RESULTS: amongst 308 manuscripts that were identified, 6 published manuscripts/ posters fulfilled the inclusion/exclusion criteria and were extracted. Face validity was found. Three studies developed an US definition of SSc-ulcers across patients (content validity); one study evaluated the concordance between US image and clinical assessment. Criterion validity was shown by one study and ultrasound detected improvement (sensitivity to change) of SSc-skin ulcer in response to therapy. Feasibility was demonstrated by US use for skin ulcers in multiple settings (the 6 manuuscripts/posters). CONCLUSION: This systematic literature review shows that ultrasound for skin ulcers in SSc has been partially validated. It has face, content, criterion validity, responsiveness and reasonable feasibility. Further validation for construct validity, reliability and discrimination is required.