Prognostic factors of paraneoplastic pemphigus.
Fiche publication
Date publication
octobre 2012
Auteurs
Membres identifiés du Cancéropôle Est :
Pr AUBIN François
Tous les auteurs :
Leger S, Picard D, Ingen-Housz-Oro S, Arnault JP, Aubin F, Carsuzaa F, Chaumentin G, Chevrant-Breton J, Chosidow O, Crickx B, D'incan M, Dandurand M, Debarbieux S, Delaporte E, Dereure O, Doutre MS, Guillet G, Jullien D, Kupfer I, Lacour JP, Leonard F, Lok C, Machet L, Martin L, Paul C, Pignon JM, Robert C, Thomas L, Weiller PJ, Ferranti V, Gilbert D, Courville P, Houivet E, Benichou J, Joly P
Lien Pubmed
Résumé
OBJECTIVE: To identify the prognostic factors of overall survival in a series of patients with paraneoplastic pemphigus (PNP). DESIGN: Multicenter retrospective cohort study. SETTING: Twenty-seven dermatology departments in France. PATIENTS: A total of 53 patients (31 men and 22 women; median age, 59 years; age range, 30-88 years) were diagnosed as having PNP between 1992 and 2010. MAIN OUTCOME MEASURES: Overall Kaplan-Meier survival rates were estimated, and features associated with survival were assessed using univariate (log-rank test) and multivariate (Cox regression) analyses. RESULTS: The study included 53 patients with PNP. Thirty-six patients (68%) died during the study. The 1-, 3-, and 5-year overall survival rates were 49%, 41%, and 38%, respectively. The main causes of death were infections (n=21) and evolution of neoplasia (n=6). In univariate analysis, the main detrimental prognostic factors identified were erythema multiforme-like skin lesions (P=.05) and histologic keratinocyte necrosis (P=.03). None of the 5 patients with Castleman disease died during the study. After adjustment for age and sex in multivariate analysis, erythema multiforme-like skin lesions remained predictive of fatal outcome, with a 2-fold increase in death rate (hazard ratio [HR], 2.3; 95% CI, 1.05-5.03; P=.04). The prognosis of patients with PNP was even poorer when erythema multiforme-like skin lesions were associated with severe skin or mucosal involvement at presentation (HR of death, 3.0; 95% CI, 1.01-8.92; P=.049). CONCLUSION: Patients with PNP with erythema multiforme-like skin lesions and histologic keratinocyte necrosis, especially when associated with extensive lesions at presentation, are likely to have a more severe and rapid fatal outcome and should be managed very carefully.
Référence
Arch Dermatol. 2012 Oct;148(10):1165-72.