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Revista Portuguesa de Pneumologia

versão impressa ISSN 0873-2159


LOPES, Carlos et al. Ehlers-Danlos syndrome : A rare cause of spontaneous pneumothorax. Rev Port Pneumol [online]. 2006, vol.12, n.4, pp.471-480. ISSN 0873-2159.

Ehlers-Danlos syndrome (cutis hyperelastica), is a group of connective tissue disorders characterized by abnormalities of the skin, ligaments and internal organs. It is a hereditary syndrome, usually with autossomal dominant inheritance; that primarily affects the collagen synthesis. The skin and blood vessels are extremely fragile and elastic. The skin is soft with rubber consistency and easily bruising. There are hypermobile joints with increased extensibility. We summarize the case of a sixteen year old boy with a history of joint hypermobility since childhood and splenic fracture that was diagnosed with Ehlers-Danlos syndrome after the occurrence of recidivant spontaneous pneumothorax. We present the most common pulmonary complications of Ehlers-Danlos syndrome and discuss the importance of not forgetting the least commons etiologies of pneumothorax, in cases of spontaneous pneumothorax.

Palavras-chave : Ehlers-Danlos Syndrome; pneumothorax; joint hypermobility.

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