Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults - 16/05/19

Doi : 10.1016/j.jaad.2018.03.043

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

Abstract

Background

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.

Objective

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

Methods

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

Results

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.

Limitation

Only case reports with sufficient mortality data were included.

Conclusion

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.

Le texte complet de cet article est disponible en PDF.

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

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

Plan

Material and methods

Results

Patient selection and general characteristics

Factors associated with mortality and survival time

Discussion

	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.

Export

Vol 80 - N° 6

P. 1544-1549 - juin 2019 Retour au numéro

Article précédent

Focal atrichia: A diagnostic clue in female pattern hair loss
Elise A. Olsen, David A. Whiting

| Article suivant

Potential of narrow-band ultraviolet B to induce sustained durable complete remission off-therapy in patients with stage I mycosis fungoides
Felix Pavlotsky, Marwan Dawood, Aviv Barzilai

Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.

Déjà abonné à cette revue ?

connectez-vous ou créez un compte

Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults - 16/05/19

Abstract

Background

Objective

Methods

Results

Limitation

Conclusion

Plan

Export citations

Fichier

Contenu

Accès rapides

Mon compte

Aide & support

Plateformes Elsevier Masson

Déclaration CNIL