Pemphigus-like mucosal lesions: A side effect of penicillamine therapy

A patient with Wilson's disease on long-term penicillamine therapy was seen for evaluation and management of chronic persistent debilitating stomatitis, which was subsequently determined to be cytologically and histologically consistent with pemphigus vulgaris. A brief review of the pertinent literature disclosed that the dermatologic lesions of pemphigus secondary to penicillamine are alleviated by discontinuation of penicillamine and institution of prednisone therapy. Our patient's primarily oral lesions did not resolve with this recommended alteration in therapy, since penicillamine could be discontinued only briefly in the interest of maintaining acceptable serum copper levels and because of the paucity of available alternative copper-chelating agents. Eventually the patient was placed on tetraethylene tetramine (TETA), an experimental copper-chelating agent, and betamethasone (Celestone). This has provided a satisfactory reduction in serum copper levels and resolution of the oral lesions.