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The course and prognosis of pemphigus in 47 Tunisian patients

Authors


  • Conflict of interest
    None.

A. Khaled. E-mail:aida.khaled@rns.tn

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.

Conclusion  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.

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