[Penicillamine-induced elastosis perforans serpiginosa and pulmonary cyst in Wilson's disease (author's transl)]

A large air cyst was removed from the right lung of a 29-year-old female patient with Wilson's disease and penicillamine-induced perforating elastosis, the cyst first appearing after 9 years of treatment with penicillamine. Since, on the one hand, the microscopic and ultrastructural changes in the elastic tissue of the lungs were identical to those observed in the skin, both in areas of clinically-demonstrable elastosis perforans serpiginosa and clinically unaffected skin and, on the other hand, no other pulmonary disease was demonstrable to account for the development of the cystic lesion, it is concluded that penicillamine is the causative factor by means of extensive alteration of the elastic tissue. The morphological changes in the elastic fibres are so characteristic that it is easy to distinguish penicillamine-induced elastosis perforans serpiginosa from the idiopathic variant.