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Postgraduate Medical Journal 2002;78:692; doi:10.1136/pmj.78.925.692
Copyright © 2002 The Fellowship of Postgraduate Medicine.
Postgraduate Medical Journal 2002;78:692
© 2002 The Fellowship of Postgraduate Medicine

Bullous lungs: diverse aetiology

The first 150 words of the full text of this article appear below.

Q1: There was extensive cystic bullous involvement of lungs in both the cases, what could be the possible diagnosis?

In the first case a diagnosis of cutis laxa—that is, Ehlers-Danlos syndrome—was made in view of elastic skin, past history of being operated for bilateral inguinal hernia, aphakic eyes, and presence of mitral valve prolapse. In the second case the diagnosis of Marfan’s syndrome was made in view of cardiac and skeletal abnormalities.

Discussion

The differential diagnoses of cystic bullous disease of the lungs to be considered are1:

  • {alpha}1-Antitrypsin deficiency.
  • {alpha}1-Antichymotrypsin deficiency.
  • Cutis laxa syndromes.
  • Sallas disease.

Other acquired causes are:

  • Inorganic dust exposure and cadmium exposure.
  • Injected dissolved methyl phenidate tablets.
  • Idiopathic non-arteriosclerosis cerebral calcification syndrome.

Ehlers-Danlos syndrome is a hereditary disease of connective tissue and sufferers were classically known as "India rubber men". In this syndrome there are nine subtypes, type-1–9 varying in severity from the severe form with bilateral joint hypermobility and velvety hyperextensible skin to milder forms. Other associated abnormalities are inguinal and diaphragmatic hernias, ectasia . . . [Full text of this article]


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Bullous lungs: diverse aetiology
S Kolekar, P Sundaram, and J M Joshi
Postgrad. Med. J. 2002 78: 689. [Extract] [Full Text] [PDF]

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