title: Prurigo pigmentosa in a patient with primary biliary cirrhosis and Sjögren syndrome.
abstract: A 44-year-old Japanese woman suddenly developed severely pruritic erythematous papules on her trunk in a symmetrical distribution. Biopsy specimens showed the typical histopathological findings of prurigo pigmentosa. She had had recurrent episodes of high fever spikes for several years, and lost 10 kg in the last year. She was diagnosed as primary biliary cirrhosis (PBC) associated with subclinical Sjögren syndrome (SjS). Predonisolone (60 mg/day) for two weeks was effective for the PBC and fever, but not for the prurigo pigmentosa. PBC may be involved in the pathogenesis of this rare skin disease.
authors.affiliation: Department of Dermatology, Chiba University, Chiba, Japan.
authors.forename: Yaei
authors.initials: Y
authors.lastname: Togawa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shinkai
authors.forename: Atsushi
authors.initials: A
authors.lastname: Utani
mesh_terms: D000284:Administration, Oral; D000328:Adult; D001707:Biopsy, Needle; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017495:Hyperpigmentation / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D007150:Immunohistochemistry; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis*; D011239:Prednisolone / Q000627:therapeutic use; D011536:Prurigo / Q000150:complications / Q000188:drug therapy* / Q000473:pathology*; D018570:Risk Assessment; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis*; D016896:Treatment Outcome
chemical_list: D011239:Prednisolone