The correlation between dermoscopy and clinical and pathological tests in the evaluation of skin photoaging.

BACKGROUND: There are no standards for evaluating skin photoaging. Dermoscopy is a non-invasive detection method that might be useful for evaluating photoaging. OBJECTIVE: To assess the correlation between the dermoscopic evaluation of photoaging and clinical and pathological evaluations. METHODS: The age, clinical evaluation (Fitzpatrick classification, Glogau Photoaging Classification, and Chung's standardized image ruler), histopathology (Masson staining and MMP-1 immunohistochemistry), and dermoscopy (Hu's and Isik's) of 40 donor skin samples were analyzed statistically, and Spearman rank correlation analysis was performed. RESULTS: There was a robust correlation between the total Hu scores and Isik dermoscopy. The correlation of dermoscopy with histopathology was higher than that of clinical evaluation methods. There is a strong correlation between telangiectases and lentigo. Xerosis, superficial wrinkle, diffuse erythema, telangiectases, and reticular pigmentation were significantly correlated with the three clinical evaluation methods. Superficial wrinkles were correlated with Masson, MMP-1, various clinical indicators, and other dermoscopic items. CONCLUSION: There is a good correlation between dermoscopy and clinical and histopathological examination. Dermoscopy might help evaluate skin photoaging.

How efficient is laser therapy for telangiectasias, spider veins, and cherry angiomas?-A study using dynamic optical coherence tomography.

OBJECTIVES: A clinical study to investigate the effectiveness of pulsed dye laser (PDL) versus Nd:YAG laser in the treatment of telangiectasias, spider veins and cherry angiomas. Dynamic optical coherence tomography (D-OCT) was introduced as an innovative follow-up tool for evaluation of blood flow within superficial vessels and to allow visualization of morphological changes of the vasculature in vivo. The final aim of this study was to demonstrate a possible treatment benefit comparing both laser types. MATERIALS AND METHODS: Vessel structures of 102 skin lesions were documented photographically and dermoscopically. Subsequently, lesions were imaged using optical coherence tomography before laser therapy (a), directly after the treatment (p) and after a follow-up 4-6 weeks after laser treatment. All lesions were treated using either a 595 nm PDL or a 1064 nm Nd:YAG laser. Two main vessel parameters, namely density and diameter, and their possible changes during follow-up were observed in 150/300/500 µm penetration depth using D-OCT and were subsequently compared between both treatment groups. Other analyzed vessel parameters were depth of the plexus, mean diameter, mean density, top edge of the vessel, columns, and spikes. RESULTS: Both laser types are suitable options for the treatment of vascular skin lesions, with the most significant effect on cherry angiomas. PDL shows better results treating smaller vessels in upper skin regions, in comparison to Nd:YAG laser, achieving better results on deeper vessels, like spider veins. Using the applied laser settings, there was no statistically significant effect on telangiectasias. CONCLUSION: D-OCT represents a new, noninvasive imaging method to evaluate blood flow and vessel morphology in the follow-up of telangiectasias, spider veins, and cherry angiomas, which underwent laser therapy.

Dorsal arachnoid web: A rare cause of syringomyelia and myelopathy / Telaraña aracnoidea dorsal: una causa rara de siringomielia y mielopatía

Dorsal arachnoidal webs are condensations of arachnoidal tissue densely adherent to the pial surface of the posterior aspect of the dorsal spinal cord. Infrequently described in literature they disrupt the flow of CSF in the spinal subarachnoid space leading to syringomyelia and myelopathy. While there are several theories on their origin, the “scalpel sign” on magnetic resonance imaging is considered to be pathognomonic of this condition. An illustrative case of a 58 year old man with syringomyelia and dorsal cord indentation who presented with spastic paraparesis, gait instability, parasthesias and bilateral non radicular upper limb pain that resolved following excision of the web is described to highlight the importance of considering this diagnosis when cases of so called “idiopathic” syringomyelia are encountered (AU)

Las membranas aracnoideas dorsales son condensaciones de tejido aracnoideo densamente adherido a la superficie pial de la cara posterior de la médula espinal dorsal. Descritas con poca frecuencia en la literatura, interrumpen el flujo del líquido cefalorraquídeo (LCR) en el espacio subaracnoideo espinal y provocan siringomielia y mielopatía. Si bien existen varias teorías sobre su origen, el «signo del bisturí» en la resonancia magnética se considera patognomónico de esta afección. Se describe un caso ilustrativo de un varón de 58 años con siringomielia e indentación medular dorsal que presentó paraparesia espástica, inestabilidad de la marcha, parestesias y dolor bilateral no radicular en miembro superior que se resolvió tras la exéresis de la telaraña para resaltar la importancia de considerar este diagnóstico cuando se encuentran casos de la llamada siringomielia «idiopática» (AU)

The impact of dermatoscopy on the pretreatment assessment of lower limbs telangiectasias: a prospective study.

BACKGROUND: Telangiectasias treatment can lead to skin hyperpigmentation, and pretreatment evaluation with dermoscopy was never performed. This study aimed to evaluate the applicability of dermatoscopy before telangiectasias treatment. METHODS: A prospective study evaluating patients of both sexes (18 to 60 years old), with telangiectasias (venous disease C2-C3 CEAP) of the lower limbs treated at outpatient clinics. Subjects who had never undergone previous interventional treatment for CVI and Fitzpatrick Classification up to phototype III, were included. Patients were submitted to both naked and dermoscopy evaluations of their skin and blindly evaluated by three vascular surgeons and an experienced dermatologist. Agreement by naked eye versus dermoscopy and among examiners was performed using Kappa correlation. Agreement by naked eye among patients and the examiners consensus were performed. RESULTS: There was a more significant agreement between the most experienced examiners in the naked eye assessment. With the dermatoscopic device, the highest agreement was maintained among the more experienced examiners, with a predominance of choice of the purple pigment in 29 of the 38 limbs, which represents a simple agreement of 76.3% (95% CI: 62.8-89.8%) with a Kappa concordance Index of 0.178. There was an agreement between the patient and the consensus of the naked eye examiners in 41.2% (95% CI: 24.7-57.7%). CONCLUSIONS: The dermatoscopy was not decisive for diagnosing skin pigmentation in areas of telangiectasia that had never been treated. The diagnostic accuracy was directly related to the clinical experience of the examiner. Dermatoscopy did not help in aligning expectations with treatment between physicians and patients.

Macular Dystrophy with Bilateral Macular Telangiectasia Related to the CYP2U1 Pathogenic Variant Assessed with Multimodal Imaging Including OCT-Angiography.

PURPOSE: We report the case of a neurologically asymptomatic young boy presenting with an unusual phenotype of CYP2U1 related macular dystrophy associating bilateral macular telangiectasia (MacTel) and fibrotic choroidal neovascularization (CNV), assessed with complete multimodal imaging including optical coherence tomography angiography (OCT-A). CASE PRESENTATION: A twelve-year-old boy from a non-consanguineous family complained of bilateral progressive visual loss and photophobia. The best-corrected visual acuity was 2/10 on the right eye and 3/10 on the left eye. Fundus examination showed central pigmented fibrotic macular scar and yellowish punctuate deposits in both eyes. En face OCT-A detected typical macular telangiectasia (MacTel) in both eyes with dilated telangiectatic capillaries in the deep capillary plexus associated with vascular anomalies in the superficial and deep capillary plexus. Typical hypo-reflective cavities were observed within the inner foveal layers on structural OCT. En face OCT-A also confirmed the presence of bilateral inactive CNV within the fibrotic scars, showing high-flow vascular network at the level of the subretinal hyperreflective lesions. Whole exome sequencing identified a known homozygous pathogenic variant in CYP2U1 gene (c.1168C > T, p.Arg390*), which is a disease-causing mutation in autosomal recessive spastic paraplegia type 56 (SPG56). The neurological examination was normal, and electromyography and brain magnetic resonance imaging were unremarkable as well. CONCLUSION: Macular dystrophy can be the first manifestation in SPG56. A particular phenotype with MacTel was observed, and neovascular complications are possible. CYP2U1 should be included in the panels of genes tested for macular dystrophies, especially in the presence of MacTel and/or neurological manifestations.

Infrared reflectance image-guided laser photocoagulation of telangiectatic capillaries in persistent diabetic macular edema.

This study aimed to assess detection rate of telangiectatic capillaries (TelCaps) with infrared reflectance (IR) and optical coherence tomography (OCT) images and to evaluate the clinical efficacy of IR image-guided focal laser photocoagulation of TelCaps in persistent diabetic macular edema (DME). This retrospective case series included 28 eyes of 28 patients with TelCap and persistent DME refractory to intravitreal anti-vascular endothelial growth factor or corticosteroids. The presence of TelCaps was confirmed using IR and OCT images. All patients were followed up for more than 12 months after direct focal laser photocoagulation of the TelCaps. The TelCap closure rate, changes in best-corrected visual acuity, and central subfield thickness were analyzed. On IR imaging, TelCap appeared as a characteristic hyperreflectivity within a hyporeflective spherical lesion in 85.7% of the eyes. After focal laser photocoagulation, the TelCap closure rate was 57.1% at 3 months and 71.4% at 12 months. A significant improvement in visual acuity and reduction in central subfield thickness were observed at three and 12 months after focal laser photocoagulation (all p < 0.05). The characteristic hyperreflectivity within hyporeflective lesions on the IR image in conjunction with OCT helps identify the TelCap. Our results suggest that IR imaging and OCT-guided focal laser photocoagulation of TelCaps can improve functional and anatomical outcomes in persistent DME.

Presentación bilateral atípica en telangiectasia macular idiopática tipo 1 / Atypical bilateral presentation in idiopathic macular telangiectasia type 1

La telangiectasia macular idiopática tipo 1 (MacTel 1) se presenta casi siempre de forma unilateral. En este artículo publicamos el caso de un varón de 73 años, sin antecedentes de importancia, diagnosticado de MacTel 1, pero de presentación atípica bilateral, algo muy infrecuente en esta enfermedad. Se resalta la utilidad de estudio con imagen multimodal, incluyendo la angiografía por tomografía de coherencia óptica (OCTA) para diferenciarla de la telangiectasia macular tipo 2 (MacTel 2). El cuadro del paciente se caracterizó por edema macular cistoideo (EMC), con discreta respuesta al tratamiento con implante de dexametasona. Sin embargo, la terapia con aflibercept mostró resultados favorables, pero con recurrencias al extender las dosis (AU)

Idiopathic macular telangiectasia type 1 (MacTel 1) almost always occurs unilaterally. In this article, we report the case of a 73-year-old man with no significant history diagnosed with MacTel 1 but with atypical bilateral presentation, something very rare in this disease. The usefulness of multimodal imaging studies, including optical coherence tomography angiography (OCTA), is highlighted to differentiate it from macular telangectasia type 2 (MacTel 2). The patient's condition was characterized by cystoid macular edema (CME) with discreet results with treatment with a dexamethasone implant. However, aflibercept therapy showed favorable results, but with recurrences when extending the doses (AU)

Idiopathic and hereditary haemorrhagic telangiectasia associated pulmonary arteriovenous malformations: comparison of clinical and radiographic characteristics.

AIM: To determine whether there are differences between idiopathic and hereditary haemorrhagic telangiectasia (HHT) associated pulmonary arteriovenous malformations (PAVMs) (HHT-PAVM) regarding clinical and radiographic characteristics, and the results of embolotherapy. MATERIALS AND METHODS: A retrospective analysis was undertaken of all adult and adolescent patients who were diagnosed with a PAVM on chest computed tomography (CT) from January 2006 until August 2019. RESULTS: In total, 41 patients with idiopathic PAVMs and 194 patients with genetically confirmed HHT and PAVMs were included. Idiopathic PAVMs were more frequently observed in female patients, were more solitary, and predominantly located in the lower lobes. The diameter of the feeding artery and type of PAVM (simple versus complex) were similar. Embolotherapy results were comparable between both groups with similar re-embolisation rates. CONCLUSIONS: PAVMs of idiopathic origin are predominantly found in women, more frequently located in the lower lobes, and solitary compared to HHT-PAVMs; however, the outcome of treatment is the same, suggesting that treatment and follow-up should be similar in both groups.

Clinical and neuroimaging findings in 33 patients with MCAP syndrome: A survey to evaluate relevant endpoints for future clinical trials.

Megalencephaly-CApillary malformation-Polymicrogyria (MCAP) syndrome results from somatic mosaic gain-of-function variants in PIK3CA. Main features are macrocephaly, somatic overgrowth, cutaneous vascular malformations, connective tissue dysplasia, neurodevelopmental delay, and brain anomalies. The objectives of this study were to describe the clinical and radiological features of MCAP, to suggest relevant clinical endpoints applicable in future trials of targeted drug therapy. Based on a French collaboration, we collected clinical features of 33 patients (21 females, 12 males, median age of 9.9 years) with MCAP carrying mosaic PIK3CA pathogenic variants. MRI images were reviewed for 21 patients. The main clinical features reported were macrocephaly at birth (20/31), postnatal macrocephaly (31/32), body/facial asymmetry (21/33), cutaneous capillary malformations (naevus flammeus 28/33, cutis marmorata 17/33). Intellectual disability was present in 15 patients. Among the MRI images reviewed, the neuroimaging findings were megalencephaly (20/21), thickening of corpus callosum (16/21), Chiari malformation (12/21), ventriculomegaly/hydrocephaly (10/21), cerebral asymmetry (6/21) and polymicrogyria (2/21). This study confirms the main known clinical features that defines MCAP syndrome. Taking into account the phenotypic heterogeneity in MCAP patients, in the context of emerging clinical trials, we suggest that patients should be evaluated based on the main neurocognitive expression on each patient.

Naevus vascularis mixtus and mixed vascular naevus syndrome.

Naevus vascularis mixtus (NVM), or mixed vascular naevus (MVN), is a binary phenotype resulting from allelic twin spotting, consisting of a naevus anaemicus paired with a telangiectatic naevus reminiscent of naevus roseus, and caused by a mosaic GNA11 mutation. MVN syndrome is characterized by an NVM associated with soft tissue hypotrophy or central nervous system abnormalities, mainly involving the cerebral vasculature. The differential diagnoses of NVM and its syndrome include vascular twin naevi, syndromes featuring naevus flammeus and other port-wine naevi, and the various types of phacomatosis pigmentovascularis. NVM and MVN syndrome are rare but probably underdiagnosed and under-reported.

Clinical outcome of short-term compression after sclerotherapy for telangiectatic varicose veins.

BACKGROUND: Sclerotherapy is considered to be the method of choice for the treatment of telangiectatic varicose veins (C1 veins). Whereas the use of compression stockings after sclerotherapy is recommended, little is known about the impact of compression on the outcome of sclerotherapy. The aim of this study was to assess the influence of compression on the outcome of injection sclerotherapy of C1 varicose veins. METHODS: There were 100 legs of 50 consecutive patients with chronic venous insufficiency (C1) included. After randomization per patient, both legs were treated with sclerotherapy in a predefined area of the thigh (measuring 100 cm2), followed by eccentric compression for 24 hours. Group A received no further compression, whereas group B was additionally equipped with compression stockings of 18 to 20 mm Hg above the ankle and continued wearing these for 1 week. Photodocumentation was performed before, 1 week after, and 4 weeks after sclerotherapy, and the clinical outcome was assessed at these postprocedure follow-up dates. The photographs were reviewed by an internal unblinded rater and an independent blinded external rater. RESULTS: There was no discernible difference between the groups in terms of clinical outcome or side effects after 4 weeks. Whereas inter-rater reliability was high, there was no correlation between the raters and patients in terms of outcome. In 55% of the treated legs, the patients deemed the result of the treatment to be good; in 27% of the treated legs, fair; and in 18%, poor. Postprocedure hyperpigmentation occurred in 13% of patients and was comparable in both groups. Compression therapy was found to be comfortable by the majority (58%) of patients. CONCLUSIONS: One week of postinterventional compression therapy had no clinical benefit compared with no compression.

Dynamic Optical Coherence Tomography Imaging of Telangiectasia Prior to Intense Pulsed Light Treatment-An Opportunity to Target Treatment?

BACKGROUND AND OBJECTIVES: To investigate whether optical coherence tomography (OCT) could be utilized to characterize blood flow and vessel dimensions of facial telangiectasias before and during consecutive intense pulsed light (IPL) treatment. STUDY DESIGN/MATERIALS AND METHODS: Dynamic OCT (D-OCT) was used to image telangiectasia immediately before and after, 1-3 days after, and 1 month after IPL treatment. Measurements included vessel width and depth, blood flow, and attenuation. Vessel dimensions at baseline were verified by a blinded observer. Clinical improvement was detected as good, moderate, or none, and adverse effects were registered at 1-month follow-up. RESULTS: In total, 14 patients with facial telangiectasia were included. At baseline, vessel width was median 0.25 mm (interquartile range [IQR]: 0.19-0.34 mm) with an intra-class coefficient (ICC) of 0.89 (95% confidence interval [CI]: 0.70; 0.97). Vessel depth was 0.30 mm (IQR: 0.25-0.33 mm; ICC: 0.40 [CI: -0.07; 0.75]). Vessel depth increased significantly from baseline to 1-month follow-up (P = 0.008), whereas no significant changes in vessel width, blood flow, or attenuation were detected. Clinical efficacy seemed related to the relation between vessel dimensions and applied energy settings. CONCLUSIONS: The D-OCT imaging technique demonstrated that facial telangiectasias were found deeper within the skin after one IPL treatment. By characterizing the vessel dimensions and blood flow of telangiectasia, D-OCT may improve efficacy and safety of IPL. Lasers Surg. Med. © 2020 Wiley Periodicals LLC.