학술논문
Prognostic Factors of Paraneoplastic Pemphigus
Document Type
Academic Journal
Author
Leger, Sandy; Picard, Damien; Ingen-Housz-Oro, Saskia; Arnault, Jean-Philippe; Aubin, François; Carsuzaa, Francis; Chaumentin, Gilles; Chevrant-Breton, Jacqueline; Chosidow, Olivier; Crickx, Béatrice; D’incan, Michel; Dandurand, Michel; Debarbieux, Stéphane; Delaporte, Emmanuel; Dereure, Olivier; Doutre, Marie-Sylvie; Guillet, Gérard; Jullien, Denis; Kupfer, Ingrid; Lacour, Jean-Philippe; Leonard, Fabienne; Lok, Catherine; Machet, Laurent; Martin, Ludovic; Paul, Carle; Pignon, Jean-Michel; Robert, Caroline; Thomas, Luc; Weiller, Pierre-Jean; Ferranti, Vincent; Gilbert, Danièle; Courville, Philippe; Houivet, Estelle; Benichou, Jacques; Joly, Pascal
Source
Archives of Dermatology. Oct 01, 2012 148(10):1165-1172
Subject
Language
English
ISSN
0003-987X
Abstract
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.