Oral complications associated with D-penicillamine treatment for Wilson disease: a clinicopathologic report.
BACKGROUND
Wilson disease (WD) is a hereditary disease inhibiting copper release from the liver. Multi-organ manifestations involve the liver, nervous system, kidneys, eyes, heart, and skin. Elastic fiber damage is a complication of the most frequently used medication in the treatment of WD D-penicillamine (D-PCA). These changes have very rarely been described in the oral cavity. The article describes oral complications associated with WD and its treatment by D-PCA.


METHODS
Clinical, radiographic, and microscopic evaluation was done on two WD female patients (aged 28 and 53), treated by D-PCA, with clinical and pathological evidence for oral drug-related complications.


RESULTS
The lesions included multiple small red papules of the lips, gingival enlargement, early onset periodontitis, and repeated oral candidiasis. Biopsies of oral mucosa (gingiva, buccal) exhibited in one case granulomatous inflammation, and in both cases, thick irregular clumps of tortuous, red-staining abnormal elastic fibers. The red lip papules resemble elastosis perforans serpiginosa (EPS). Similar lesions have been described in the skin, but never before in association with oral or perioral tissue. In addition to the oral lesions, one of the patients developed general intolerance to the drug and was switched to trientine hydrochloride.


CONCLUSIONS
WD patients and others treated by D-PCA may develop oral and perioral complications, in some cases exhibiting features of damaged elastic fibers in the mucosa and periodontal apparatus. It is possible that this damage may be one of the factors responsible for poor periodontal health in WD patients. Recognition of the lesions can lead to replacement of the affecting therapeutic agent.
Tovaru
Serban
S
Department of Oral Medicine, Oral Pathology, Faculty of Dental Medicine, Carol Davila University, Bucharest, Romania.
Parlatescu
Ioannina
I
Dumitriu
Anca-Silvia
AS
Bucur
Alexandru
A
Kaplan
Ilana
I
D000328:Adult; D002180:Candidiasis, Oral / Q000209:etiology; D002613:Cheilitis / Q000139:chemically induced; D002614:Chelating Agents / Q000009:adverse effects*; D004547:Elastic Tissue / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D005884:Gingival Hemorrhage / Q000139:chemically induced; D005885:Gingival Hyperplasia / Q000139:chemically induced; D005889:Gingival Recession / Q000139:chemically induced; D006099:Granuloma / Q000139:chemically induced; D006527:Hepatolenticular Degeneration / Q000188:drug therapy*; D006801:Humans; D008047:Lip Diseases / Q000209:etiology; D008875:Middle Aged; D009059:Mouth Diseases / Q000139:chemically induced*; D010396:Penicillamine / Q000009:adverse effects*; D010514:Periodontal Pocket / Q000139:chemically induced; D010518:Periodontitis / Q000139:chemically induced; D014060:Tongue Diseases / Q000139:chemically induced / Q000382:microbiology; D014266:Trientine / Q000627:therapeutic use
D002614:Chelating Agents; D010396:Penicillamine; D014266:Trientine