ABSTRACT
Resumo O pseudoxantoma elástico é uma doença generalizada do tecido conjuntivo envolvendo a pele, olhos e sistema cardiovascular desencadeando a fragmentação e calcificação das fibras elásticas. Geralmente ocorre após a puberdade, as manifestações características são manchas pequenas, circunscritas, amareladas, localizadas no pescoço, axila e pregas inguinais. Estrias angioides na retina, tendência à hemorragia e insuficiência arterial são as complicações mais comuns. Esta doença pode ser herdada como autossômica dominante ou recessiva. O tratamento das manifestações oculares convencional é através da fototerapia a laser impedindo a ocorrência de hemorragias locais. Entretanto, novas abordagens terapêuticas estão sendo desenvolvidas como a utilização em longo prazo de drogas antiangiogênicas, as quais atuam inibindo a neovascularização ocular. Apesar de não ter ainda efetivamente substituído o tratamento original, pesquisas recentes já evidenciam benefícios da nova técnica. O objetivo deste estudo é relatar sobre o caso de uma paciente de 37 anos, portadora do pseudoxantoma elástico, com estrias angioides e hemorragia ocular, e o tratamento eficaz com a terapia antiangiogênica no ambulatório de oftalmologia em Nova Iguaçu, Rio de Janeiro.
Abstract The pseudoxanthoma elasticum is a generalized disease of the connective tissue involving the skin, eyes and cardiovascular system triggering the fragmentation and calcification of elastic fibers. Usually occurs after puberty, the manifestations characteristics are small spots, circumscribed, yellowish, located on the neck, axilla and inguinal folds. Angioid streaks in the retina, tendency to hemorrhage and arterial insufficiency are the most common complications. This disease can be inherited as autosomal dominant or recessive. The treatment of ocular manifestations is through the conventional phototherapy laser preventing the occurrence of local hemorrhages. However, new therapeutic approaches are being developed as the long-term use of drugs antiangiogenic, which act by inhibiting the ocular neovascularization. Despite not having yet effectively replaced the original treatment, recent research already show benefits of new technique. The objective of this study is to report on a case of a patient of 37 years, the carrier of the Pseudoxanthoma Elasticum, with angioid streaks and ocular hemorrhage, and the effective treatment with antiangiogenic therapy at the clinic of Ophthalmology in Nova Iguaçu, Rio de Janeiro.
Subject(s)
Humans , Female , Adult , Pseudoxanthoma Elasticum/complications , Eye Hemorrhage/etiology , Angioid Streaks/etiology , Ophthalmoscopy , Tonometry, Ocular , Eye Hemorrhage/diagnosis , Eye Hemorrhage/drug therapy , Fluorescein Angiography , Visual Acuity , Angiogenesis Inhibitors/therapeutic use , Intravitreal Injections , Bevacizumab/therapeutic use , Ranibizumab/therapeutic use , Slit Lamp Microscopy , Angioid Streaks/diagnosis , Angioid Streaks/drug therapyABSTRACT
Angioid streaks also called Knapp striae are small breaks in the Bruch's membrane and have been reported with a host of systemic diseases. Rupture of streaks or development of secondary choroidal neovascular membrane (CNVM) carries a dismal visual prognosis. We report the successful treatment of CNVM secondary to Paget's disease using low fluence photodynamic therapy (PDT) and intravitreal ranibizumab.
Subject(s)
Angiogenesis Inhibitors/administration & dosage , Angioid Streaks/complications , Angioid Streaks/diagnosis , Angioid Streaks/etiology , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal, Humanized , Choroidal Neovascularization/diagnosis , Choroidal Neovascularization/drug therapy , Choroidal Neovascularization/etiology , Drug Therapy, Combination , Fluorescein Angiography , Fundus Oculi , Humans , Intravitreal Injections , Male , Middle Aged , Osteitis Deformans/complications , Osteitis Deformans/pathology , Photochemotherapy/methods , Treatment OutcomeABSTRACT
Pseudoxanthoma elasticum [PXE] is an inherited disorder of elastic tissue with many systemic manifestations. We performed a retrospective study from all the patients diagnosed with PXE at the department of dermatology of La Rabat hospital of Tunis, between 1986 and 2003. During the observation period, we identified 11 patients with PXE, 5 males and 6 females with a mean age of 28 years [10-47 years]. Family history was found in 5 patients. Exhibit yellowish, pigskin, and popular lesions on the sides of the neck were observed in all cases. Systematic ophthalmologic examination revealed angioid streaks in 4 patients. No abnormalities were found in cardiovascular and metabolic explorations. Diagnosis of PXE is based on clinical, histological and genetic criteria. Ocular and cardiovascular damage make all the gravity of the disease, from where interest of an ophthalmologic and cardiovascular examination systematic
Subject(s)
Humans , Male , Female , Pseudoxanthoma Elasticum/diagnosis , Retrospective Studies , Angioid Streaks/diagnosis , Elastic Tissue/pathologyABSTRACT
Foram analisados os prontuários de 22 pacientes (43 olhos) com diagnóstico de estrias angióides no período de março de 1977 a novembro de 1994. A idade média foi de 40,95 anos, variando de 17 a 77 anos. Vinte e um pacientes eram da cor branca, 1 da cor amarela, näo tivemos pacientes da cor preta. A acuidade visual inicial era de 20/40 ou melhor em 55,8 por cento pacientes, entre 20/50 a 20/70 em 19,3 por cento, 20/80 a 20/200 em 23,2 por cento e o pior que 20/200 em 11,7 por cento. Treze pacientes (60 por cento) tinham pseudo xantoma elástico, 1 paciente 4 por cento com anemia falciforme, 9 (40 por cento) de causa idiopática. Näo tivemos paciente com doença de Paget. Membrana neovascular sub retiniana estava presente em 6 pacientes e 2 pacientes desenvolveram a membrana em 4 anos de seguimento.