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Journal of the American Academy of Dermatology
Volume 80, n° 6
pages 1544-1549 (juin 2019)
Doi : 10.1016/j.jaad.2018.03.043
accepted : 25 Mars 2018
Original Articles

Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults

Elise Ouedraogo, MD a, Jeremy Gottlieb, MD a, Adèle de Masson, MD, PhD a, b, Clémence Lepelletier, MD a, b, Marie Jachiet, MD a, b, Camille Salle de Chou, MD a, b, Lionel Galicier, MD b, c, Martine Bagot, MD, PhD a, b, Sylvie Chevret, MD, PhD b, d, Jean-David Bouaziz, MD, PhD a, b,
a Assistance Publique-Hôpitaux de Paris, Département de Dermatologie, Hôpital Saint-Louis, Paris, France 
b Université Paris-Diderot, Sorbonne Paris Cité, Paris, France 
c Assistance Publique-Hôpitaux de Paris, Service d'Immuno-Hématologie, Hôpital Saint-Louis, Paris, France 
d Assistance Publique-Hôpitaux de Paris, Service de Santé Publique, Hôpital Saint-Louis, Paris, France 

Correspondence to: Jean-David Bouaziz, MD, PhD, Department of Dermatology, AP-HP, Université Paris VII Sorbonne Paris Cité, Hôpital Saint Louis, 1 avenue Claude Vellefaux, 75475, Paris, France.Department of DermatologyAP-HPUniversité Paris VII Sorbonne Paris CitéHôpital Saint Louis1 avenue Claude VellefauxParis75475France

Paraneoplastic pemphigus (PNP) occurs more often in patients with hematologic malignancies (HMs) than in patients with solid cancer. Lung bronchiolitis obliterans (BO) is a severe complication of PNP.


To determine the precise clinical and biologic features of HM-associated PNP and identify factors associated with mortality and survival.


Systematic review of previously described cases of PNP associated with HMs.


A total of 144 patients were included. The HMs were non-Hodgkin lymphoma (52.78%), chronic lymphocytic leukemia (22.92%), Castleman disease (18.60%), and other underlying hematologic malignancy (5.70%). The mortality rate was 57%, and most deaths occurred within the first year after the diagnosis of PNP. Multivariate analysis showed that (1) the presence of antienvoplakin antibodies and BO were significantly associated with death, and (2) a toxic epidermal necrolysis–like clinical pattern, bullous pemphigoid–like clinical pattern, and BO were significantly associated with decreased survival.


Only case reports with sufficient mortality data were included.


PNP associated with HM has a high mortality rate. The toxic epidermal necrolysis–like and BO-associated forms are independent survival factors in PNP associated with HMs.

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

Key words : bullous pemphigoid, Castleman disease, lymphoma, pemphigus

Abbreviations used : BO, BP, CI, HM, HR, OR, PNP, TEN

 Dr Chevret and Dr Jean-David Bouaziz contributed equally and share last author senior status.
 Funding sources: None.
 Conflicts of interest: None disclosed.
 Reprints not available from the authors.

© 2018  American Academy of Dermatology, Inc.@@#104156@@
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