title: Clinical analysis of 69 patients with familial benign chronic pemphigus.
abstract: OBJECTIVE
  To analyze the clinical feature, efficacy of treatment and prognosis in familial benign chronic pemphigus (FBCP).
  
  
  METHODS
  Sixty-nine cases of FBCP were retrospectively analyzed.
  
  
  RESULTS
  The ratio of male to female is 3.93:1 in 69 patients (55 males, 14 females). The mean age at the onset was 29.09 years (3-60 years). There was familial history in 27 families in all of the cases. The lesion usually involved in genital area, neck, axillae and popliteal fossa. Erythemas and vesicles on the soles were seen only in 1 case. Histopathologically 44 cases had special features of FBCP, and immunopathologically 8 cases were direct immunofluorescence (DIF) negative, in which one case had C3 linear deposition along dermoepidermal junction. The combined regimen was more effective. The low-dose X-ray could improve the effect.
  
  
  CONCLUSIONS
  The disease is transmitted as an irregular autosomal dominant trait. The condition in males is more frequent than that in females, probably owing to the different level of female hormone in both sexes. Our patients have the same clinical features as those reported in the literature, but the erythema, vesicle lesions on sole have not been documented in the literature. The combined therapy should be adopted in this condition.
authors.affiliation: Institute of Dermatology, Chinese Academy of Medical Sciences, Peking Union Medical College, Nanjing 210042, China.
authors.forename: H
authors.initials: H
authors.lastname: Gu
authors.forename: B
authors.initials: B
authors.lastname: Chang
authors.forename: W
authors.initials: W
authors.lastname: Chen
authors.forename: C
authors.initials: C
authors.lastname: Shao
mesh_terms: D000328:Adult; D000368:Aged; D000890:Anti-Infective Agents / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003622:Dapsone / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016506:Pemphigus, Benign Familial / Q000175:diagnosis / Q000532:radiotherapy / Q000628:therapy*; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D012737:Sex Factors
chemical_list: D000890:Anti-Infective Agents; D003622:Dapsone; D011241:Prednisone