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Vol. 12. Issue 4.
Pages 471-480 (July - August 2006)
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Vol. 12. Issue 4.
Pages 471-480 (July - August 2006)
Caso Clínico\Clinical Case
Open Access
Síndroma de Ehlers-Danlos – Uma causa rara de pneumotórax espontâneo
Ehlers-Danlos syndrome – A rare cause of spontaneous pneumothorax
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6356
Carlos Lopes1,
Corresponding author
carlos.lopes@hsm.min-saude.pt

Correspondência: Carlos Lopes, Serviço de Pneumologia, Hospital de Santa Maria, Lisboa, Portugal
, Alda Manique2, Renato Sotto-Mayor3, Jorge Cruz4, Margarida Mendes de Almeida5, João Cravino6, A Bugalho de Almeida7
1 Interno do Internato Complementar de Pneumologia, Serviço de Pneumologia
2 Assistente Hospitalar Graduada de Pneumologia
3 Chefe de Serviço de Pneumologia. Assistente Convidado da Faculdade de Medicina de Lisboa
4 Assistente Hospitalar Graduado de Cirurgia Cardiotorácica
5 Assistente Hospitalar Graduada de Anatomia Patológica
6 Director do Serviço de Cirurgia Cardiotorácica, Serviço de Cirurgia Cardiotorácica
7 Director do Serviço de Pneumologia. Professor Associado da Faculdade de Medicina de Lisboa
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Resumo

A síndroma de Ehlers-Danlos (cutis hyperelastica), constitui uma patologia do tecido conjuntivo caracterizada por alterações da pele, ligamentos e órgãos internos.

Apresenta transmissão hereditária, em geral autossómica dominante. Afecta primordialmente a síntese do colagéneo, pelo que a pele e os vasos sanguíneos se tornam extremamente elásticos e frágeis. A pele é macia, com consistência de borracha, e as equimoses surgem facilmente. As articulações são hiperextensíveis e têm mobilidade aumentada.

Os autores apresentam o caso clínico de um doente de dezasseis anos, com história de hipermobilidade articular desde a infância e fractura esplénica, a quem foi feito o diagnóstico de síndroma de Ehlers-Danlos na sequência de pneumotórax espontâneo recidivante.

Apresentam-se as complicações pulmonares mais frequentes desta síndroma e discute-se a importância de não esquecer as etiologias menos comuns, perante casos de pneumotórax espontâneo.

Rev Port Pneumol 2006; XII (4): 471-480

Palavras-chave:
Síndroma de Ehlers-Danlos
pneumotórax
hipermobilidade articular
Abstract

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.

Rev Port Pneumol 2006; XII (4): 471-480

Key-words:
Ehlers-Danlos Syndrome
pneumothorax
joint hypermobility
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Copyright © 2006. Sociedade Portuguesa de Pneumologia/SPP
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