Images in Clinical Medicine
Pemphigus Foliaceus
N Engl J Med 2005; 353:2589December 15, 2005
- Article
A 63-year-old man presented with a nine-year history of relapsing pemphigus foliaceus that had become refractory to therapy. After treatment with plasma exchange and oral corticosteroids, his disease flared in association with a rise in antibody titer to 1:1280. Rituximab was administered intravenously at a dose of 800 mg, and the patient was admitted with widespread erythroderma with erosions, scaling, and crusting (Panel A). Despite the administration of intravenous corticosteroids, oral cyclophosphamide, and three additional infusions of rituximab, new lesions continued to develop. Remission was achieved after monthly treatments with high-dose intravenous immune globulin and pulsed intravenous cyclophosphamide over a six-month period, with ongoing administration of oral corticosteroids (Panel B). Treatment was then changed to oral mycophenolate mofetil, which continues, and corticosteroids were slowly withdrawn. Despite the presence of detectable antibody (with a titer of 1:5 to 1:80), the patient remains well.
Sarah Johnston, M.R.C.P., M.R.C.Path.
Cameron Kennedy, F.R.C.P.
Southmead Hospital, Bristol BS10 5NB, United Kingdom- Citing Articles (1)
Citing Articles
1
R. J. Lock, M. M. Gompels. (2007) C1-inhibitor deficiencies (hereditary angioedema): Where are we with therapies?. Current Allergy and Asthma Reports 7:4, 264-269
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