Author Affiliations: Dermatology Branch, Division of Clinical Sciences, National Cancer Institute, National Institutes of Health, Bethesda, Md.
Grand Rounds at the Clinical Center of the National
Institutes of Health Section Editors: John I. Gallin, MD, the Clinical
Center of the National Institutes of Health, Bethesda, Md; David S. Cooper,
MD, Contributing Editor, JAMA.
Autoimmune blistering diseases are generally distinct entities characterized
by relatively consistent clinical, histologic, and immunopathologic findings.
These disorders may cause impaired adhesion of epidermis to epidermal basement
membrane (eg, the pemphigoid group of disorders [bullous, gestational, and
mucous membrane]) or impaired adhesion of epidermal cells to each other (eg,
the pemphigus group of disorders). Recent studies have shown that these disorders
are characterized by autoantibodies that often display pathogenic (ie, blister-forming)
activity in passive transfer models. Interestingly, the autoantigens targeted
by these patients' autoantibodies represent important structural proteins
that promote cell matrix (eg, pemphigoid) or cell-to-cell (eg, pemphigus)
adhesion in skin. Autoimmune blistering diseases are characterized by substantial
morbidity (pruritus, pain, disfigurement), and in some instances, mortality
(secondary to loss of epidermal barrier function). Treatment with systemic
immunosuppressives has reduced morbidity and mortality in patients with these
Yancey KB, Egan CA. Pemphigoid: Clinical, Histologic, Immunopathologic, and Therapeutic Considerations. JAMA. 2000;284(3):350–356. doi:10.1001/jama.284.3.350