title: The course and prognosis of pemphigus in 47 Tunisian patients.
abstract: BACKGROUND
  Pemphigus is a severe and life-threatening autoimmune bullous dermatosis.
  
  
  OBJECTIVE
  We have analysed parameters that may influence prognosis of pemphigus (P).
  
  
  METHODS
  It was a retrospective study (2002-2010), with pemphigus considered as severe if body surface involvement ≥ 30%. Disease control and relapse-free survival (Kaplan-Meier) were analysed and compared according to several parameters (P < 0.05).
  
  
  RESULTS
  47 cases of pemphigus were collected, mean age 51 years ± 16.8 (F/H = 3.27). There were 30 pemphigus profundus and 17 superficial pemphigus. The median remission period was of 9 months (1.2 months-5 years). The mean healing time was of 40 days (6 days-4 months), which did not depend on type of P, its severity or infectious complications, whereas it was shorter in aged patient (≥ 65 years) compared to non aged ones (P = 0.018). 36.2% of patients had relapsed. Relapses were significantly more frequently observed only in the presence of mucosal involvement at presentation (P = 0.015). The median overall 1st relapse-free survival was of 2.33 years. Only mucosal involvement at presentation was associated with a shorter median 1st relapse-free survival time (1.28 years vs. 3 years) (P = 0.0017). Mortality rate was of 10.6% (n = 5); in four patients the death was directly related to pemphigus and occurred rapidly after the onset of lesions.
  
  
  CONCLUSIONS
  Our study illustrates the poor prognosis of pemphigus by a long duration to disease control, a high initial dose of oral steroid, a high rate of relapse and a short remission period. Only mucosal involvement at presentation was identified as a poor prognostic factor.
authors.affiliation: Department of Dermatology, Charles Nicolle Hospital Tunis, Tunisia. aida.khaled@rns.tn
authors.forename: A
authors.initials: A
authors.lastname: Khaled
authors.forename: S Ben
authors.initials: SB
authors.lastname: Taazayet
authors.forename: N
authors.initials: N
authors.lastname: Ben Alaya
authors.forename: A
authors.initials: A
authors.lastname: Souissi
authors.forename: F
authors.initials: F
authors.lastname: Zeglaoui
authors.forename: N
authors.initials: N
authors.lastname: Kaffel
authors.forename: M
authors.initials: M
authors.lastname: Kharfi
authors.forename: R
authors.initials: R
authors.lastname: Zermani
authors.forename: B
authors.initials: B
authors.lastname: Fazaa
mesh_terms: D000284:Administration, Oral; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D016001:Confidence Intervals; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy* / Q000453:epidemiology* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use*; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D015996:Survival Rate; D016896:Treatment Outcome; D014416:Tunisia / Q000453:epidemiology; D055815:Young Adult
chemical_list: D011241:Prednisone