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Lichenoid Dermatitis in Paraneoplastic Pemphigus
A Pathogenic Trigger of Epitope Spreading?
Glen M. Bowen, MD;
Neill T. Peters, MD;
David P. Fivenson, MD;
Lyndon D. Su, MD;
Hossein C. Nousari, MD;
Grant J. Anhalt, MD;
Kevin D. Cooper, MD;
Seth R. Stevens, MD
Arch Dermatol. 2000;136:652-656.
Background In select cases, lichen planus has been observed to be a paraneoplastic condition sometimes associated with paraneoplastic pemphigus, a disease featuring autoantibodies directed against plakin proteins, desmogleins 3 and 1, and a still uncharacterized 170-kd antigen. Epitope spreading describes the phenomenon where underlying chronic inflammation leads to the sequential recognition of new epitopes on self-proteins over time.
Observations Five of 6 patients diagnosed as having paraneoplastic pemphigus had concomitant clinical and histological features of lichen planus. In 1 patient, results of the initial indirect immunofluorescence on rat bladder were negative and only 2 of the 5 antigens were identified by immunoprecipitation. After 1 year of worsening disease, repeated testing confirmed the presence of antibodies directed against all 6 of the implicated antigens, supportive of our hypothesis that epitope spreading may occur in paraneoplastic pemphigus.
Conclusions Lichenoid eruptions may predispose to an early evolutionary stage of paraneoplastic pemphigus. Cell-mediated autoimmunity at the dermoepidermal junction may promote the exposure of self-antigens and the development of subsequent and progressive humoral autoimmunity. As such, paraneoplastic pemphigus may demonstrate epitope spreading in a human, humoral-mediated autoimmune disease.
From the Departments of Dermatology, University of Utah, Salt Lake City (Dr Bowen), Henry Ford Hospital, Detroit, Mich (Dr Fivenson), and University of Michigan, Ann Arbor (Dr Su); Division of Dermatoimmunology, Johns Hopkins Medical Institutions, Baltimore, Md (Drs Nousari and Anhalt); and Departments of Dermatology, Case Western Reserve University, University Hospitals of Cleveland, and the Louis Stokes Veterans Affairs Medical Center, Cleveland, Ohio (Drs Cooper and Stevens). Dr Peters is in private practice in Chicago, Ill.
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