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Rhinology and Facial Plastics
Published in Adnan Darr, Karan Jolly, Jameel Muzaffar, ENT Vivas, 2023
Adnan Darr, Karan Jolly, Shahzada Ahmed, Claire Hopkins
Examination: Telangiectasia is usually through direct examination: Anterior rhinoscopyNasendoscopyOropharyngeal examinationSkin (fingers, face and ears)
Skin
Published in A. Sahib El-Radhi, Paediatric Symptom and Sign Sorter, 2019
Telangiectasia indicates permanently dilated superficial blood vessels in the skin or mucous membranes. One or two telangiectases on the face (occasionally on the dorsa of the hands) are common and trivial in children, and highly characteristic in the vascular centre, from which radiates fine vessels that give the appearance of a spider web. Unlike petechiae, which have a similar appearance, they blanch with pressure and refill immediately after the pressure is released. Other lesions may suggest serious systemic disease such as ataxia telangiectasia (progressive cerebellar ataxia, sinopulmonary infection and immunodeficiency) and hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease), which is autosomal dominant, characterised by arteriovenous malformation on the skin, mucous membranes and internal organs such as brain, liver and lungs.
Pathogenesis of normal tissue side effects
Published in Michael C. Joiner, Albert J. van der Kogel, Basic Clinical Radiobiology, 2018
Telangiectasia, i.e. pathologically dilated capillaries, is observed in virtually all irradiated tissues and organs. The pathogenesis is unclear, but it is assumed that endothelial cell damage is involved. The loss of smooth muscle cells surrounding larger capillaries and veins may also contribute to the development of telangiectasia. In the intestine, the urinary system or also the central nervous system, telangiectasia can be clinically relevant because of the tendency for capillary haemorrhage. In the skin, telangiectasia are mainly a cosmetic problem; they also served, however, as a well-defined quantitative endpoint for radiobiological studies (see Section 14.5). Over longer time periods, the dilated, telangiectatic capillaries may collapse, thus resulting in a reversibility of their consequences, which has been demonstrated, e.g. after radiotherapy for uterine cervix or prostate cancer (15,16).
Clinical efficacy analysis of 110 cases of childhood vitiligo with non-surgical combined therapy
Published in Journal of Dermatological Treatment, 2022
Yuan-Yuan Liu, Jun-Feng Zhou, Yu Zhen, Yan Cui, Yang Song, Lei Yao, Shan-Shan Li
Thirty-two patients experienced adverse effects during the therapy. Eleven patients developed marked erythema, and seven patients developed a blister at the application site. These problems led to a temporary interruption of phototherapy, but the patients were all safely restarted on NB-UVB therapy at a lower dose. Among the children treated with a TCI, six experienced pruritus with tacrolimus 0.03% ointment, which was gradually tolerated by reducing the dosage, and none had discomfort with pimecrolimus 1% cream. Five patients who were administered a TCS experienced telangiectasia. Three adolescents treated with an SC developed weight gain, which returned to normal following the withdrawal of the SC. None of the patients who were administered a TCS or SC experienced Cushing’s syndrome or adrenal suppression.
Coats like response in healed choroiditis
Published in Ocular Immunology and Inflammation, 2022
Saurabh Verma, Indrish Bhatia, Mousumi Banerjee, Vinod Kumar
Abnormal telangiectatic retinal vessels accompanied with subretinal exudation with or without retinal detachment in association with other ocular or systemic disorder are included in Coats’-like response.1 The etiology of retinal telangiectasia, either congenital or secondary remains unclear till now. Tarkkanen et al.7 studied the histopathological specimen of retina in Coats’ disease and inferred functional or structural loss of the blood-retinal barrier with disorganization of mural architecture. Reese8 postulated that endothelial cell dysfunction causes localized microvascular atresia and occlusion, which leads to vascular ectasia and formation of collateral vessels. Thus, the possible etiology could be any vaso-occlusive event, autoimmune vasculopathy or chronic inflammation causing structural damage to retinal vasculature which may precede the development of retinal telangiectasias.6 The clinical significance of this entity arises from the fact that such an exudative response further deteriorates the visual acuity due to its involvement of the posterior pole. In our case, the damage to the retinal vasculature secondary to inflammatory response might have lead to the development of Coats’-like response. Early diagnosis of this response can halt the ongoing neovascular drive and stabilize the blood retinal barrier by treating with intravitreal anti-VEGFs/steroids and/or laser photocoagulation. It also reduces the need for vitrectomy in the future.
Intractable bleeding from the renal pelvis in a patient with hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease)
Published in Scandinavian Journal of Urology, 2020
L. F. Qvigstad, O. J. Grøtta, C. Hammarström, E. Baco
Hereditary haemorrhagic telangiectasia is an autosomal dominant disease that affects 1 in 5–8000 and leads to the development of abnormal vascular structures such as telangiectasias and arteriovenous malformations, in which the latter most commonly affects pulmonary, hepatic and cerebral circulations [1]. Affection of the renal pelvis is an exceptionally rare manifestation and has only been described in two previous case reports requiring embolization [2] and surgery [3], respectively. One cohort study including 11 patients with HHT, where renal structure and function were assessed, found no evidence supporting renal involvement in this disease [4]. Renal arteriovenous malformations are rarely seen unless it is after trauma or iatrogenic injury, in which case it would be defined as an arteriovenous fistula. Further research would be needed to define the role of renal arteriovenous malformations in the context of HHT. This case reports a female patient with an advanced stage of HHT, including systemic comorbidities, where the intractable bleeding from the renal pelvis demanded a sequential approach, gradually increasing the invasiveness, before ultimately being successfully treated with laparoscopic surgery.