Desquamative gingivitis associated with IgG/IgA pemphigoid presents a challenging diagnosis and treatment: a case report.
BACKGROUND
Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune blistering disorders characterized by subepithelial separation and the deposition of immunoglobulins and complement along the basement membrane zone (BMZ). This disease is diagnosed with direct immunofluorescence testing showing a linear deposition of immunoglobulins and/or complement along the BMZ and indirect immunofluorescence testing showing circulating IgG (and sometimes IgA) autoantibodies along the BMZ. In this case report we describe desquamative gingivitis secondary to IgG/IgA pemphigoid and the management of this challenging variant of MMP.


METHODS
Routine histology, direct immunofluorescence testing, and indirect immunofluorescence testing were utilized and correlated to the clinical findings to diagnose this unusual immunobullous disease.


RESULTS
Direct and indirect immunofluorescence testing confirmed the clinical diagnosis of IgG/IgA pemphigoid as the cause of desquamative gingivitis and the other mucosal findings in this patient. A treatment program including dapsone and other drugs completely resolved the oral lesions after 14 months of therapy.


CONCLUSIONS
Desquamative gingivitis associated with IgG/IgA pemphigoid can be challenging to diagnose and treat. After 14 months of treatment, a combination therapy consisting of dapsone with cimetidine and vitamin E to enhance drug efficacy and frequent intramuscular administrations of triamcinolone achieved control of both the oral and genital elements of IgG/IgA pemphigoid in this patient.
Sánchez
Andrés R
AR
Division of Periodontics, Department of Dental Specialties, Mayo Clinic, Rochester, MN 55905, USA. sanchez.andres@mayo.edu
Rogers
Roy S
RS
Kupp
Leo I
LI
Sheridan
Phillip J
PJ
D000368:Aged; D003622:Dapsone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D019084:Fluorescent Antibody Technique, Indirect; D005891:Gingivitis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D003622:Dapsone