Pneumothorax in the Ehlers-Danlos syndrome: consequence of coincidence?

A 25 year-old man, known to suffer from Ehlers-Danlos syndrome, developed a left-sided pneumothorax. At thoracotomy, pulmonary blebs and bullae were found at the apex of the lung. Bullectomy with removal of adjacent lung tissue was performed. The histological and ultrastructural findings in the lung tissue must be regarded as non-specific. These findings do not reflect a direct cause-and-effect relationship, even in the presence of the inheritable connective tissue disorder.