title: Oral Ulcers as an Initial Presentation of Juvenile Pemphigus: A Case Report.
abstract: Pemphigus vulgaris (PV) is an autoimmune disease in which the autoantibody, immunoglobulin G, is directed against the keratinocytes in the epidermis. The classic presentations of PV are flaccid vesicles or bullae over the oral mucosa, trunk, groin, and extremities. The age of onset is usually between 40 and 60 years, and cases of PV in children or adolescent patients are rare. Here, we present a 17-year-old boy who had painful oral ulcers for 3 months initially and bullae spreading to the whole body in the following days. Paraneoplastic pemphigus was another differential diagnosis due to the atypical appearance of the skin lesion. However, PV was confirmed by hematoxylin and eosin staining and immunofluorescence examination of the skin biopsy specimens. The patient had a good response to corticosteroid treatment and the immunosuppressive agent, rituximab.
authors.affiliation: Department of Pediatrics, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
authors.forename: I-Hsuan
authors.initials: IH
authors.lastname: Chen
authors.affiliation: Department of Pediatrics, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
authors.forename: Shu-Chi
authors.initials: SC
authors.lastname: Mu
authors.affiliation: Department of Dermatology, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
authors.forename: Dino
authors.initials: D
authors.lastname: Tsai
authors.affiliation: Department of Pathology, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
authors.forename: Yuh-Yu
authors.initials: YY
authors.lastname: Chou
authors.affiliation: Department of Dermatology, National Taiwan University, Taipei, Taiwan.
authors.forename: Li-Fang
authors.initials: LF
authors.lastname: Wang
authors.affiliation: Department of Pediatrics, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan. Electronic address: jpcantw@yahoo.com.tw.
authors.forename: Ling-Jen
authors.initials: LJ
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D019226:Oral Ulcer / Q000209:etiology*; D010392:Pemphigus / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
keywords: oral ulcers; paraneoplastic pemphigus; pemphigus vulgaris; rituximab