Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) with unusual manifestations and without detectable autoantibodies

Indian J Dermatol Venereol Leprol. 2014 Jul-Aug;80(4):328-30. doi: 10.4103/0378-6323.136898.

Abstract

We describe a patient with paraneoplastic autoimmune multiorgan syndrome (PAMS) secondary to a lymphoblastic T- cell lymphoma who presented with a lichenoid dermatitis and vitiligo, later developing bronchiolitis obliterans and autoimmune hepatitis. Notably, he had no detectable autoantibodies. The development of vitiligo and autoimmune hepatic involvement probably indicate a role for cytotoxic T- cell lymphocytes in the pathogenesis of this syndrome.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Agents, Hormonal / administration & dosage
  • Autoantibodies* / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / drug therapy
  • Humans
  • Leukemia, T-Cell / blood
  • Leukemia, T-Cell / diagnosis
  • Leukemia, T-Cell / drug therapy
  • Male
  • Paraneoplastic Syndromes / blood
  • Paraneoplastic Syndromes / diagnosis*
  • Paraneoplastic Syndromes / drug therapy
  • Pemphigus / blood
  • Pemphigus / diagnosis*
  • Pemphigus / drug therapy
  • Prednisone / administration & dosage
  • Young Adult

Substances

  • Antineoplastic Agents, Hormonal
  • Autoantibodies
  • Prednisone