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Rev. bras. oftalmol ; 74(6): 390-392, nov.-dez. 2015. graf
Article in English | LILACS | ID: lil-767073

ABSTRACT

RESUMO A síndrome de Rothmund (RTS) é uma rara genodermatose, de herança autossômica recessiva. Sua incidência é desconhecida, com aproximadamente 300 casos descritos na literatura. A síndrome é determinada por eritema facial (poiquilodermia), seu marco diagnóstico, além de alterações esqueléticas, alopecia, catarata juvenil e predisposição a osteossarcoma. Neste relato, descrevemos uma paciente com esta síndrome, que foi referida ao serviço de oftalmologia por baixa visão e hiperemia ocular.


ABSTRACT Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis. While its incidence is unknown, approximately 300 cases have been reported in the literature. The syndrome typically presents with a characteristic facial rash (poikiloderma), its diagnostic hallmark, and heterogeneous clinical features including congenital skeletal abnormalities, sparse hair distribution, juvenile cataracts, and a predisposition to osteosarcoma. This is a report describing a patient diagnosed with RTS referred to us because of low vision and red eyes.


Subject(s)
Humans , Female , Rothmund-Thomson Syndrome/complications , Rothmund-Thomson Syndrome/diagnosis , Rothmund-Thomson Syndrome/pathology , Visual Acuity , Entropion/surgery , Entropion/etiology , Rothmund-Thomson Syndrome/genetics , Corneal Transplantation , Limbus Corneae , Corneal Opacity/diagnosis , Corneal Opacity/etiology , Corneal Opacity/pathology , Genetic Predisposition to Disease , Hyperemia
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