A case of systemic pseudo-pseudoxanthoma elasticum with diverse symptomatology caused by long term penicillamine use

摘要

A 47 year old man presented with a two year history of increasing cervical dys- phagia, dyspnoea, and cutaneous signs. He had been diagnosed 27 years previ- Oulsy with Wilson's disease and was Treted with penicillamine (1.5 g daily). Systemci abnormality of elastic fibres was Confirmed by light and electron micros- Copy following biopsy of skin, lung, Oesophageal muscle, gum, pharyngeal tis- Sue, and cervical connective tissue. Dys- Phagia was relieved by cricopharyngeal Myotomy. Substitution of trientene dihy- Drochloride for penicillamine relieved cu- Taneous and systemic manifestations.