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Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) with unusual manifestations and without detectable autoantibodies. 2014

Jimena Sanz-Bueno, and Daniella Cullen, and Carlos Zarco, and Francisco Vanaclocha
Department of Dermatology, 12 de Octubre University Hospital, Madrid, Spain.

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.

UI MeSH Term Description Entries
D008297 Male Males
D010257 Paraneoplastic Syndromes In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products. Paraneoplastic Syndrome,Syndrome, Paraneoplastic,Syndromes, Paraneoplastic
D010392 Pemphigus Group of chronic blistering diseases characterized histologically by ACANTHOLYSIS and blister formation within the EPIDERMIS. Pemphigus Vulgaris,Pemphigus Foliaceus,Foliaceus, Pemphigus
D011241 Prednisone A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver. Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D001327 Autoimmune Diseases Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides. Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune
D015458 Leukemia, T-Cell A malignant disease of the T-LYMPHOCYTES in the bone marrow, thymus, and/or blood. Leukemia, Lymphocytic, T-Cell,Lymphocytic Leukemia, T-Cell,T-Cell Leukemia,T-Lymphocytic Leukemia,Leukemia, Lymphocytic, T Cell,T Lymphocytic Leukemia,Leukemia, T Cell,Leukemia, T Lymphocytic,Leukemia, T-Cell Lymphocytic,Leukemia, T-Lymphocytic,Leukemias, T Lymphocytic,Leukemias, T-Cell,Leukemias, T-Cell Lymphocytic,Leukemias, T-Lymphocytic,Lymphocytic Leukemia, T,Lymphocytic Leukemia, T Cell,Lymphocytic Leukemias, T,Lymphocytic Leukemias, T-Cell,T Cell Leukemia,T Lymphocytic Leukemias,T-Cell Leukemias,T-Cell Lymphocytic Leukemia,T-Cell Lymphocytic Leukemias,T-Lymphocytic Leukemias
D055815 Young Adult A person between 19 and 24 years of age. Adult, Young,Adults, Young,Young Adults
D018931 Antineoplastic Agents, Hormonal Antineoplastic agents that are used to treat hormone-sensitive tumors. Hormone-sensitive tumors may be hormone-dependent, hormone-responsive, or both. A hormone-dependent tumor regresses on removal of the hormonal stimulus, by surgery or pharmacological block. Hormone-responsive tumors may regress when pharmacologic amounts of hormones are administered regardless of whether previous signs of hormone sensitivity were observed. The major hormone-responsive cancers include carcinomas of the breast, prostate, and endometrium; lymphomas; and certain leukemias. (From AMA Drug Evaluations Annual 1994, p2079) Hormonal Antineoplastic Agents,Antineoplastic Drugs, Hormonal,Antineoplastic Hormonal Agents,Antineoplastic Hormonal Drugs,Antineoplastics, Hormonal,Hormonal Agents, Antineoplastic,Hormonal Antineoplastic Drugs,Hormonal Antineoplastics,Agents, Antineoplastic Hormonal,Drugs, Antineoplastic Hormonal,Hormonal Drugs, Antineoplastic

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