Penicillamine-induced changes in elastic tissue of the upper respiratory tract

Abstract

We describe a patient who developed upper respiratory tract symptoms following long-term treatment of Wilson's disease with penicillamine. These symptoms were attributed to areas of pharyngeal thickening and were treated with a laser. Histological examination of the lesions showed proliferations of abnormal elastic fibres similar to those previously described at other sites, especially the skin, in patients receiving penicillamine. This drug impairs the maturation and reduces the stability of elastic fibres and although elastic tissue throughout the body is affected, we are aware of no previous reports of penicillamine- induced changes presenting with upper respiratory tract symptoms.

Intravascular synovial sarcoma.

“…At concentrations above 1000 U/ml, the specificity of the CA19-9 assay is reportedly more than 99% for malignant gastrointestinal disease. 7 The present patient had evidence of neither CA19-9 producing tumours nor obstructive jaundice. Liver biopsy specimen showed severe necrosis in the hepatic lobes and large numbers of proliferated bile ductules.…”

“…Over three years, excision biopsies of skin, a gum dermatofibroma, and thickened pharyngeal tissue indicated changes of PPXE. 7 Clinical examination consistently revealed a yellow "plucked chicken" appearance of the folded skin of the neck and axillae but no angioid streaks in the retina. Barium studies showed hold-up to flow through the oesophagus at the cricopharyngeus.…”

Penicillamine-induced elastosis perforans serpiginosa with abnormal "lumpy-bumpy" elastic fibers in lesional and non-lesional skin

“…[4] Long-term d-penicillamine therapy causes multiple degenerative changes in elastic fibers of skin. Similar elastic fiber changes have been reported in upper respiratory tract, [5] pulmonary tissue, [4] aortic wall, visceral adventitia and alveolar septa. [4] Abnormal elastic fibers are not only confined to lesional skin, but also present in non-lesional skin.…”

Penicillamine-induced elastosis perforans serpiginosa and cutis laxa in Wilson’s disease

“…3 , 4 These conditions can sometimes occur at the lower dosages used in rheumatoid arthritis. 5 Elastolytic changes may also be seen in other tissues such as the upper respiratory tract, 6 the joint capsules, 7 the lungs, aorta and adventitia of the viscera. 5 The histological feature seen consistently in patients with elastolysis due to penicillamine is ‘lumpy‐bumpy’ 8 or ‘bramble‐bush’ 9 ‐like elastic fibres, which appears to be quite specific.…”