Resumo

PILA PEREZ, Rafael et al. Pseudoxanthoma elasticum associated with severe hypertension. AMC [online]. 2011, vol.15, n.4, pp. 724-732. ISSN 1025-0255.

Background:pseudoxanthoma elasticum is a rare disorder characterized by degeneration of the elastic fibers with calcification areas in the skin, retina and cardiovascular system. Objective: to present the case of a patient with this disease, the second reported in our country. Case report: a female of 37 years old patient presented micropapulae in body regions of skinfolds, associated with angioid bands in the eyeground, edema in lower limbs, severe hypertension and very weak peripheral pulse. The histopathologic lesions study is conclusive of a pseudoxanthoma elasticum; that is classified as type I according to the inheritance pattern as is clearly shown. This syndrome is a disease without specific treatment so it is recommend a multidisciplinary assessment for a better study. The most common cause of morbidity is the decrease in visual acuity, hence the importance of the systematic fulfillment of the eyeground.

Palavras-chave : PSEUDOXATHOMA ELASTICUA; HYPERTENSION; ADULT; CASE STUDIES; SKIN [injuries].

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