Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies

J Am Acad Dermatol. 2007 Jan;56(1):153-9. doi: 10.1016/j.jaad.2006.06.007. Epub 2006 Sep 14.

Abstract

Paraneoplastic pemphigus (PNP) has been described as an antibody-mediated mucocutaneous disease occurring almost exclusively in patients with lymphocytic neoplasms. We describe 4 patients with the clinical features of the lichenoid variant of PNP in the absence of detectable autoantibodies. On the basis of these findings, we conclude that the spectrum of PNP likely includes patients with disease predominantly or exclusively mediated by cytotoxic T cells rather than autoantibodies. The pathophysiology and range of PNP disease are likely more complex than was initially believed.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies, Monoclonal / administration & dosage
  • Antibodies, Monoclonal / pharmacology
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • Antibody Formation / drug effects
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Autoantibodies / blood*
  • B-Lymphocytes / drug effects
  • Combined Modality Therapy
  • Cyclophosphamide / administration & dosage
  • Doxorubicin / administration & dosage
  • Esophageal Diseases / diagnosis
  • Esophageal Diseases / etiology
  • Esophageal Diseases / immunology
  • Etoposide / administration & dosage
  • Female
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immunity, Cellular
  • Interleukin-2 / administration & dosage
  • Leukemia, Lymphocytic, Chronic, B-Cell / complications
  • Leukemia, Lymphocytic, Chronic, B-Cell / drug therapy
  • Leukemia, Lymphocytic, Chronic, B-Cell / radiotherapy
  • Leukemia, Lymphocytic, Chronic, B-Cell / surgery
  • Lichenoid Eruptions / diagnosis
  • Lichenoid Eruptions / etiology*
  • Lichenoid Eruptions / immunology
  • Lymphoma, Follicular / complications
  • Lymphoma, Follicular / drug therapy
  • Lymphoma, Follicular / radiotherapy
  • Lymphoma, Non-Hodgkin / complications
  • Lymphoma, Non-Hodgkin / drug therapy
  • Lymphoma, Non-Hodgkin / radiotherapy
  • Male
  • Middle Aged
  • Mucositis / complications
  • Paraneoplastic Syndromes / etiology*
  • Paraneoplastic Syndromes / immunology
  • Pemphigus / diagnosis
  • Pemphigus / etiology*
  • Pemphigus / immunology
  • Prednisone / administration & dosage
  • Recurrence
  • Rituximab
  • T-Lymphocytes / immunology
  • Vidarabine / administration & dosage
  • Vidarabine / analogs & derivatives
  • Vincristine / administration & dosage

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Autoantibodies
  • Interleukin-2
  • Rituximab
  • Vincristine
  • Etoposide
  • Doxorubicin
  • Cyclophosphamide
  • Vidarabine
  • fludarabine
  • Prednisone

Supplementary concepts

  • CHOP protocol
  • EPOCH protocol