{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T13:02:43Z","timestamp":1792501363247,"version":"4.5.0"},"reference-count":28,"publisher":"American Academy of Pediatrics (AAP)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,10,1]]},"abstract":"<jats:p>Paraneoplastic autoimmune multiorgan syndrome, also known as paraneoplastic pemphigus, has been observed only rarely among children. We describe a 10-year-old boy with typical clinical and histologic findings of paraneoplastic pemphigus associated with Castleman\u2019s disease. His disease was refractory to resection of the tumor and aggressive combination immunosuppressive therapies. The patient died 1 year after presentation, as a result of complications of bronchiolitis obliterans. This case is unusual because of the young age of the patient.<\/jats:p>","DOI":"10.1542\/peds.2004-0436","type":"journal-article","created":{"date-parts":[[2004,10,13]],"date-time":"2004-10-13T18:57:26Z","timestamp":1097693846000},"page":"e513-e516","source":"Crossref","is-referenced-by-count":43,"title":["Paraneoplastic Autoimmune Multiorgan Syndrome (Paraneoplastic Pemphigus) in a Child: Case Report and Review of the Literature"],"prefix":"10.1542","volume":"114","author":[{"given":"Joshua E.","family":"Lane","sequence":"first","affiliation":[{"name":"Section of Dermatology, Department of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol","family":"Woody","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loretta S.","family":"Davis","sequence":"additional","affiliation":[{"name":"Section of Dermatology, Department of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret F.","family":"Guill","sequence":"additional","affiliation":[{"name":"Section of Pulmonology, Department of Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita S.","family":"Jerath","sequence":"additional","affiliation":[{"name":"Section of Rheumatology, Department of Pediatrics, Medical College of Georgia, Augusta, Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2004,10,1]]},"reference":[{"key":"2021120906070197200_R1","doi-asserted-by":"crossref","unstructured":"Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus: an autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med.\u20081990;323:1729\u20131735","DOI":"10.1056\/NEJM199012203232503"},{"key":"2021120906070197200_R2","unstructured":"Borradori L, Lombardi T, Samson J, Girardet C, Saurat JH, Hugli A. Anit-CD20 monoclonal antibody (rituximab) for refractory erosive stomatitis secondary to CD20+ follicular lymphoma-associated paraneoplastic pemphigus. Arch Dermatol.\u20082001;137:269\u2013272"},{"key":"2021120906070197200_R3","doi-asserted-by":"crossref","unstructured":"Heizmann M, Itin P, Wernli M, Borradori L, Bargetzi MJ. Successful treatment of paraneoplastic pemphigus in follicular NHL with rituximab: report of a case and review of treatment for paraneoplastic pemphigus in NHL and CLL. Am J Hematol.\u20082001;66:142\u2013144","DOI":"10.1002\/1096-8652(200102)66:2<142::AID-AJH1032>3.0.CO;2-0"},{"key":"2021120906070197200_R4","doi-asserted-by":"crossref","unstructured":"Kiyokawa C. Envoplakin and periplakin and the paraneoplastic pemphigus antigens. Kurume Med J.\u20081999;46:71\u201378","DOI":"10.2739\/kurumemedj.46.71"},{"key":"2021120906070197200_R5","doi-asserted-by":"crossref","unstructured":"Mahoney MG, Aho S, Uitto J, Stanley JR. The members of the plakin family of proteins recognized by paraneoplastic pemphigus. J Invest Dermatol.\u20081998;111:308\u2013313","DOI":"10.1046\/j.1523-1747.1998.00279.x"},{"key":"2021120906070197200_R6","doi-asserted-by":"crossref","unstructured":"Amagai M, Nishikawa T, Nousari HC, Anhalt GJ, Hashimoto T. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest.\u20081998;102:775\u2013782","DOI":"10.1172\/JCI3647"},{"key":"2021120906070197200_R7","doi-asserted-by":"crossref","unstructured":"Reich K, Brink U, Lefschert M, et al. Graft-versus-host disease-like immunophenotype and apoptotic keratinocyte death in paraneoplastic pemphigus. Br J Dermatol.\u20081999;141:739\u2013746","DOI":"10.1046\/j.1365-2133.1999.03123.x"},{"key":"2021120906070197200_R8","doi-asserted-by":"crossref","unstructured":"Martel P, Loiseau P, Joly P, et al. Paraneoplastic pemphigus is associated with the DRB1*03 allele. J Autoimmun.\u20082003;20:91\u201395","DOI":"10.1016\/S0896-8411(02)00092-6"},{"key":"2021120906070197200_R9","doi-asserted-by":"crossref","unstructured":"Liu AY, Valenzuela R, Helm TN, Camisa C, Melton AL, Bergfield WF. Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus. J Am Acad Dermatol.\u20081993;28:696\u2013699","DOI":"10.1016\/0190-9622(93)70095-B"},{"key":"2021120906070197200_R10","doi-asserted-by":"crossref","unstructured":"Helou J, Allbritton J, Anhalt GJ. Accuracy of indirect immunofluorescence testing in the diagnosis of paraneoplastic pemphigus. J Am Acad Dermatol.\u20081995;32:441\u2013447","DOI":"10.1016\/0190-9622(95)90066-7"},{"key":"2021120906070197200_R11","doi-asserted-by":"crossref","unstructured":"Mehregan DR, Oursler JR, Leiferman KM, Muller SA, Anhalt GJ, Peters MS. Paraneoplastic pemphigus: a subset of patients with pemphigus and neoplasia. J Cutan Pathol.\u20081993;20:203\u2013210","DOI":"10.1111\/j.1600-0560.1993.tb00644.x"},{"key":"2021120906070197200_R12","doi-asserted-by":"crossref","unstructured":"Chorzelski T, Hashimoto T, Maciejewska B, Amagai M, Anhalt GJ, Jablonska S. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis, and bronchiolitis obliterans. J Am Acad Dermatol.\u20081999;41:393\u2013400","DOI":"10.1016\/S0190-9622(99)70111-8"},{"key":"2021120906070197200_R13","doi-asserted-by":"crossref","unstructured":"van Mook WNK, Fickers MM, Theunissen PH, et al. Paraneoplastic pemphigus as the initial presenting sign of chronic lymphocytic leukemia. Ann Oncol.\u20082001;12:115\u2013118","DOI":"10.1023\/A:1008324929876"},{"key":"2021120906070197200_R14","doi-asserted-by":"crossref","unstructured":"Hartz RS, Daroca PJ. Clinical-pathological conference: cutaneous paraneoplastic pemphigus associated with benign encapsulated thymoma. J Thorac Cardiovasc Surg.\u20082003;125:400\u2013406","DOI":"10.1067\/mtc.2003.60"},{"key":"2021120906070197200_R15","doi-asserted-by":"crossref","unstructured":"Leyn J, Degreef H. Paraneoplastic pemphigus in a patient with a thymoma. Dermatology.\u20082001;202:151\u2013154","DOI":"10.1159\/000051623"},{"key":"2021120906070197200_R16","doi-asserted-by":"crossref","unstructured":"Takahashi M, Shimatsu Y, Kazama T, Kimura K, Otsuka T, Hashimoto T. Paraneoplastic pemphigus associated with bronchiolitis obliterans. Chest.\u20082000;117:603\u2013607","DOI":"10.1378\/chest.117.2.603"},{"key":"2021120906070197200_R17","doi-asserted-by":"crossref","unstructured":"Matz H, Milner Y, Frusic-Zlotkin M, Brenner S. Paraneoplastic pemphigus associated with pancreatic carcinoma. Acta Derm Venereol (Stockh).\u20081997;77:289\u2013291","DOI":"10.2340\/0001555577289291"},{"key":"2021120906070197200_R18","doi-asserted-by":"crossref","unstructured":"Niimi Y, Kawana S, Hashimoto T, Kusunoki T. Paraneoplastic pemphigus associated with uterine carcinoma. J Am Acad Dermatol.\u20082003;48:S69\u2013S72","DOI":"10.1067\/mjd.2003.156"},{"key":"2021120906070197200_R19","doi-asserted-by":"crossref","unstructured":"Hinterhuaer G, Drach J, Riedl E, et al. Paraneoplastic pemphigus in association with hepatocellular carcinoma. J Am Acad Dermatol.\u20082003;49:538\u2013540","DOI":"10.1067\/S0190-9622(03)01581-0"},{"key":"2021120906070197200_R20","unstructured":"Rivollier C, Vaillant L, Machet MC, et al. Paraneoplastic pemphigus: a pustular form during chronic lymphoid leukemia. Ann Dermatol Venereol.\u20082001;128:644\u2013648"},{"key":"2021120906070197200_R21","unstructured":"Nguyen VT, Ndoye A, Bassler KD, et al. Classification, clinical manifestations, and immunpathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome. Arch Dermatol.\u20082001;137:193\u2013206"},{"key":"2021120906070197200_R22","doi-asserted-by":"crossref","unstructured":"Schoen H, Foedinger D, Derfler K, et al. Immunoapharesis in paraneoplastic pemphigus. Arch Dermatol.\u20081998;134:706\u2013710","DOI":"10.1001\/archderm.134.6.706"},{"key":"2021120906070197200_R23","doi-asserted-by":"crossref","unstructured":"Ng PP, Rencic A, Nousari HC. Paraneoplastic pemphigus: a refractory autoimmune mucocutaneous disease. J Cutan Med Surg.\u20082002;6:434\u2013437","DOI":"10.1177\/120347540200600505"},{"key":"2021120906070197200_R24","doi-asserted-by":"crossref","unstructured":"Rico MJ. Autoimmune blistering diseases in children. Semin Dermatol.\u20081995;14:54\u201359","DOI":"10.1016\/S1085-5629(05)80040-9"},{"key":"2021120906070197200_R25","doi-asserted-by":"crossref","unstructured":"Rybojad M, Leblanc T, Flageul B, et al. Paraneoplastic pemphigus in a child with a T-cell lymphoblastic lymphoma. Br J Dermatol.\u20081993;128:418\u2013422","DOI":"10.1111\/j.1365-2133.1993.tb00202.x"},{"key":"2021120906070197200_R26","doi-asserted-by":"crossref","unstructured":"Lemon MA, Weston WL, Huff JC. Childhood paraneoplastic pemphigus associated with Castleman\u2019s tumour. Br J Dermatol.\u20081997;136:115\u2013117","DOI":"10.1111\/j.1365-2133.1997.tb08759.x"},{"key":"2021120906070197200_R27","doi-asserted-by":"crossref","unstructured":"Mimouni D, Anhalt GJ, Lazarova Z, et al. Paraneoplastic pemphigus in children and adolescents. Br J Dermatol.\u20082002;147:725\u2013732","DOI":"10.1046\/j.1365-2133.2002.04992.x"},{"key":"2021120906070197200_R28","doi-asserted-by":"crossref","unstructured":"Mar WA, Glaesser R, Strubble K, Stephens-Groff S, Bangert J, Hansen RC. Paraneoplastic pemphigus with bronchiolitis obliterans in a child. Pediatr Dermatol.\u20082003;20:238\u2013242","DOI":"10.1046\/j.1525-1470.2003.20311.x"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/114\/4\/e513\/1019939\/zpe0100400e513.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/114\/4\/e513\/1019939\/zpe0100400e513.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,14]],"date-time":"2024-01-14T16:53:03Z","timestamp":1705251183000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/114\/4\/e513\/73241\/Paraneoplastic-Autoimmune-Multiorgan-Syndrome"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,10,1]]},"references-count":28,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2004,10,1]]},"published-print":{"date-parts":[[2004,10,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2004-0436","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,10,1]]}}}