title: A case of bullous pemphigoid arising in juvenile hyaline fibromatosis with oral squamous cell carcinoma.
abstract: We experienced a case of bullous pemphigoid (BP) arising in juvenile hyaline fibromatosis (JHF) associated with oral squamous cell carcinoma (SCC). JHF is a very rare disease inherited in an autosomal recessive fashion. Skin-colored, elastic hard nodules up to 10 cm in size were scattered mainly on the trunk and scalp. Gingival hypertrophy and an ill-defined mass with an irregular surface on the hard palate were noticed. A skin biopsy and a gingival biopsy confirmed the diagnosis of JFH and oral SCC, respectively. The patient was receiving anti-cancer drugs for the treatment of oral SCC and the treatment was successful. In the meantime, bean to nail sized bullas were noticed on the hands and feet. Histological examination, immunofluorescence method and ELISA confirmed the diagnosis of BP. The pathophysiological mechanisms are discussed in the present case.
authors.affiliation: Department of Dermatology, Nagasaki University, Graduate School of Biomedical Sciences, 1-7-1, Sakamoto, 852-8501 Nagasaki, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Shimizu
authors.forename: Fumihide
authors.initials: F
authors.lastname: Ogawa
authors.forename: Yoichiro
authors.initials: Y
authors.lastname: Hamasaki
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Murota
authors.forename: Ichiro
authors.initials: I
authors.lastname: Katayama
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002294:Carcinoma, Squamous Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D003241:Consanguinity; D003937:Diagnosis, Differential; D005260:Female; D005351:Fibromatosis, Gingival / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D005887:Gingival Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage; D012535:Scalp; D013909:Thorax
chemical_list: D011239:Prednisolone