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Journal of the American Academy of Dermatology
Sous presse. Epreuves corrigées par l'auteur. Disponible en ligne depuis le samedi 13 juillet 2013
Doi : 10.1016/j.jaad.2013.05.016
accepted : 22 May 2013
Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids

Naif Almugairen, MD a, Valerie Hospital, MD c, Christophe Bedane, MD, PhD d, Sophie Duvert-Lehembre, MD a, Damien Picard, MD, PhD a, Anne-Fleur Tronquoy, MD a, Estelle Houivet, MD b, Michel D'incan, MD, PhD c, Pascal Joly, MD, PhD a,
a Department of Dermatology, INSERM U 905, Institute for Research and Innovation in Biomedicine, Rouen University Hospital, University of Rouen, Rouen, France 
b Department of Biostatistics, Rouen University Hospital, University of Rouen, Rouen, France 
c Department of Dermatology, Clermont-Ferrand University Hospital, Clermont-Ferrand, France 
d Department of Dermatology, Limoges University Hospital, Limoges, France 

Reprint requests: Pascal Joly, MD, PhD, Clinique Dermatologique, Rouen University Hospital, 1 Rue de Germont, F 76031 Rouen cedex, France.

Few studies have evaluated pemphigus treatments according to the definitions of the consensus statement. Prognostic factors for complete remission off therapy (CRoffT) remain unknown.


We sought to assess the rate of CRoffT in patients with pemphigus treated with different regimens.


In all, 134 patients with pemphigus were included in a retrospective, multicenter study. Primary end point was the rate of CRoffT. Prognostic factors for CRoffT were determined using univariate and multivariate analyses.


Eighty patients with pemphigus vulgaris, 47 with pemphigus foliaceus, and 7 with paraneoplastic pemphigus were included. Mean age was 60 ± 18 years. Patients were treated either with medium (≤0.5 mg/kg/d) (n = 32) or high (≥1 mg/kg/d) (n = 59) doses of prednisone, or without systemic corticosteroids (n = 43). Mean follow-up was 77 ± 64 months. In all, 68 patients (50.7%) achieved CRoffT (95% confidence interval 42.3%-59.2%) after a mean treatment duration of 36 ± 39 months, including 47 of 80 patients with pemphigus vulgaris (58.7%) and 21 of 47 with pemphigus foliaceus (44.7%). Main prognostic factors for CRoffT were initial mucosal involvement (hazard ratio 2.2; 95% confidence interval 1.05-4.58; P  = .036) and younger age (<61 years) (hazard ratio 2.5; 95% confidence interval 1.18-5.12; P  = .0167). The rate of long-lasting CRoffT was 44%, with a mean follow-up after treatment withdrawal of 59 ± 50 months.


This was a retrospective study.


The rate of CRoffT was 51%. Patients with pemphigus vulgaris were more likely to achieve CRoffT than those with pemphigus foliaceus.

The full text of this article is available in PDF format.

Key words : follow-up, pemphigus, treatment

Abbreviations used : CI, CR, CRoffT, CS, MMF, PF, PNP, PV

 Funding sources: None.
 Conflicts of interest: None declared.

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