Comparison between intravenous immunoglobulin and conventional immunosuppressive therapy regimens in patients with severe oral pemphigoid: effects on disease progression in patients nonresponsive to dapsone therapy.
BACKGROUND
Mucous membrane pemphigoid has a wide clinical spectrum. The clinical context was to determine whether pemphigoid disease that initiates in the oral cavity progresses to involve other mucosae and to determine the influence of systemic therapy on such progression.


OBJECTIVE
To determine the clinical outcomes and disease progression in patients with oral pemphigoid for whom dapsone therapy was impossible.


METHODS
Retrospective analysis of a cohort of 20 patients with immunopathologic-proven oral pemphigoid studied between September 1, 1994, and October 31, 2000. Twelve patients received conventional therapy that consisted of a combination of oral prednisone with an immunosuppressive agent. Eight patients in whom such therapy was contraindicated received intravenous immunoglobulin therapy. Patients were followed up for 33 to 62 months (mean follow-up, 47.5 months).


METHODS
Patients were treated in an ambulatory tertiary medical care facility of a university-affiliated hospital.


METHODS
The 20 patients had pemphigoid disease limited to the oral cavity only at the initial clinical presentation and when enrolled in the study.


METHODS
The following variables were compared between the 2 groups of patients: (1) duration of treatment, (2) frequency of relapses, (3) induction of remission, (4) adverse effects of therapy, (5) extra oral involvement, and (6) quality of life.


RESULTS
Using the aforementioned factors, the group treated with intravenous immunoglobulin had statistically significant shorter treatment duration, fewer relapses, higher remission rate, fewer adverse effects, no extraoral involvement, and a better quality of life compared with the group who received conventional therapy.


CONCLUSIONS
Intravenous immunoglobulin is a safe and effective modality to treat mucous membrane pemphigoid. It seems to be a good option for patients who cannot be treated with dapsone and in whom conventional therapy is contraindicated or results in the development of serious adverse effects. In patients with progressive mucous membrane pemphigoid, intravenous immunoglobulin therapy may arrest disease progression.
Ahmed
A R
AR
Department of Medicine, New England Baptist Hospital, Boston, MA, USA. razzaque_ahmed@hms.harvard.edu
Colón
J E
JE
D000328:Adult; D000368:Aged; D003622:Dapsone / Q000009:adverse effects / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007116:Immunization, Passive / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000188:drug therapy*; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy*; D011241:Prednisone / Q000627:therapeutic use; D011788:Quality of Life; D012008:Recurrence; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D003622:Dapsone; D011241:Prednisone