[D-penicillamine-induced pemphigus, polymyositis and myasthenia].
BACKGROUND
D-penicillamine can induce autoimmune disease, particularly in patients with associated immune disorders.


METHODS
A 67-year old woman who had been taking D-penicillamine for 15 months for rheumatoid arthritis was hospitalized due to the development of a bullous eruption and proximal muscle deficiency. Search for intercellular antisubstance antibodies in serum was negative. The skin biopsy histology revealed intra-epidermal cleavage in the mucosal body and direct immunofluorescence revealed epidermal frame-marking with anti-IgG and anti-C3 antibodies. Other tests revealed muscular cytolysis, and anti-acetylcholine receptor antibodies. The electromyogram showed neuromuscular block without muscle deficiency and muscle biopsy showed moderate myositis. D-penicillamine was interrupted and was followed by cure of the pemphigus and aggravation of the myositis, requiring high-dose systemic corticosteroid therapy.


CONCLUSIONS
This patient developed D-penicillamine induced pemphigus, a rather frequent observation. The desmoglein immunolabelling favored drug-induced pemphigus and the course was rapidly favorable after withdrawal. Pemphigus had developed simultaneously with signs of myasthenia and polymyositis. Polymyositis and myasthenia are also known complications of D-penicillamine therapy. The association of these three complications suggests that D-penicillamine can unmask certain antigens or have an immunomodulator effect.
Jan
V
V
Service de Dermatologie, CHU Trousseau, Tours.
Callens
A
A
Machet
L
L
Machet
M C
MC
Lorette
G
G
Vaillant
L
L
D000368:Aged; D018501:Antirheumatic Agents / Q000009:adverse effects* / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000188:drug therapy; D001706:Biopsy; D005260:Female; D006801:Humans; D009132:Muscles / Q000473:pathology; D009157:Myasthenia Gravis / Q000139:chemically induced* / Q000175:diagnosis; D010392:Pemphigus / Q000139:chemically induced* / Q000175:diagnosis / Q000473:pathology; D010396:Penicillamine / Q000009:adverse effects* / Q000627:therapeutic use; D017285:Polymyositis / Q000139:chemically induced* / Q000175:diagnosis / Q000473:pathology; D012867:Skin / Q000473:pathology
D018501:Antirheumatic Agents; D010396:Penicillamine