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Year : 2014  |  Volume : 80  |  Issue : 4  |  Page : 328--330

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

Department of Dermatology, 12 de Octubre University Hospital, Madrid, Spain

Correspondence Address:
Jimena Sanz-Bueno
Hospital 12 de Octubre, Avenida de Córdoba s/n, Madrid - 28041
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0378-6323.136898

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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.


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Online since 15th March '04
Published by Wolters Kluwer - Medknow