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Chair, John S. Strauss Professor and HeadProfessor of Dermatology
Office: 40040 Pomerantz Family PavilionIowa City, IA 52242
Office Phone: 1319-356-3609
BS, Zoology, Michigan State UniversityMD, Medicine, University of Michigan Medical School
Internship, Hennepin County Medical Center, Minneapolis, MNResidency, Dermatology, University of Michigan, Ann Arbor, MIFellowship, NIH Research, Cell Physiology, Dermatology, University of Michigan, Ann Arbor, MI
IowaMichiganDiplomate, dermatopathology, Board of DermatopathologyDiplomate, Dermatology, American Board of Dermatology
Interdisciplinary Graduate Program in Translational Biomedicine
The overall goal of the Fairley laboratory is to better understand the pathogenesis of the autoimmune blistering diseases of the skin. These disorders are primarily autoantibody-mediated and through a better understanding of how these antibodies develop and lead to blistering, we hope to develop new therapeutic interventions. Bullous pemphigoid (BP) is a blistering disease of the skin characterized by autoantibodies directed against BP180, also termed collagen XVII, a cell-substrate attachment protein of the hemisdesmosome. Antibodies of the IgG1 and IgG4 subclass historically have been the focus of most studies of the pathogenesis of this disorder. Work in the Fairley laboratory has shown that during the initial urticarial phase of the disease 70% of BP patients have elevated total IgE levels. IgE autoantibodies that specifically target BP180 have been identified in 90% of these patients. These IgE autoantibodies predominantly target the same region of the BP antigen as the IgG class autoantibodies (NC16A). Basophils from untreated BP patients will degranulate when exposed to the NC16A region of BP180. Utilizing human skin grafted to nu/nu mice the Fairley laboratory has demonstrated that purified IgE from BP patients results in formation of urticarial plaques with histologic separation of the skin through the basement membrane zone. Electron microscopy of these lesions indicates degranulation of mast cells and infiltration of lymphocytes, PMNs and eosinophils. On going studies aim to differentiate the IgE effects on mast cells and keratinocytes using real-time PCR, cytokine antibody arrays and ELISA. In addition, the development of IgE class autoantibodies is other blistering disorders is being studied. A pilot project examining the effect of the humanized anti-IgE monoclonal antibody, omalizumab, in BP patients has been developed. Pemphigus vulgaris (PV) is characterized by development of autoantibodies that target the desmosomal protein desmoglein 3. These autoantibodies lead to loss of cell-cell attachment between keratinocytes that results in epidermal separation and blister formation. TNF has been hypothesized to play a role in B cell maturation and on-going production of autoantibodies. The Fairley laboratory is part of a multicenter trial of infliximab in the treatment of PV patients. This study will also include an assessment of B cells maturation and autoantibody production
Missing the target: characterization of BP180 ELISA-negative bullous pemphigoid patients.
Jour Am Acad Derm.
The Dermatology Foundation: partnerships and programs focused on the future.
Journal of Investigative Dermatology.
Erythema migrans: a spectrum of histopathologic changes.
AM J Dermatopath.
2012 December. 34(8):834-7.
Cryptic esophageal pemphigus vulgaris despite apparent clinical remission.
J Am Acad Dermatol.
FA role for IgE receptor expression on eosinophils in BP.
J Invest Derm.
FceRI expression by eosinophils in BP and a potential pathogenic role in eosinophil degranulation.
J Invest Dermatol.
Functional characterization of an IgE-class monoclonal antibody specific for the bullous pemphigoid autoantigen, BP180.
Monoclonal antibody BP180 against bullous pemphigoid antigen-2 type XVII collagen.
Numerous skin-colored papules on the face and neck.
TNF-α is critical for the autoimmune skin blistering disease bullous pemphigoid.
Date Last Modified: 11/14/2013 -
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