Search

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.

International audience; Pruritus is a common symptom of bullous pemphi-goid (BP), but has been poorly studied. The aim of this study was to analyse the characteristics of pruritus in patients with BP and its impact on their quality of life. A multicentre prospective observational study (in 15 French hospitals) was performed. A total of 60 patients were included, with a mean age of 77.4 years. Pru-ritus occurred daily in 85% of patients, with a mean pruritus intensity of 5.2/10. Tingling sensations were present in 72.4% of patients and burning sensations in 68.9%. Pruritus was exacerbated by stress, fatigue and xerosis. The mean ItchyQol score was 56.2/110 and the mean 5-D Itch Scale score was 16.5/25. The severity of pruritus was not related to age, sex, BP activity score, eosinophilia, or anti-BP230 and anti-BP180 autoantibodies. This study revealed that pruri-tus in BP is poorly tolerated and is an important cause of impaired quality of life. .

Introduction La dermatomyosite (DM) est une maladie auto-immune, atteignant classiquement le muscle et la peau, caracterisee par la presence d’auto-anticorps et une infiltration lymphocytaire des tissus cibles. La DM amyopathique (DMa) est une forme sans atteinte musculaire. L’objectif etait d’etudier les populations lymphocytaires conventionnelles et innees dans le sang et les cytokines pro-inflammatoires seriques des patients ayant une DMa. Materiel et methodes Nous avons inclus 12 patients atteints de DMa, nous presentons les resultats preliminaires sur 6. Les criteres d’inclusion etaient un diagnostic de DMa selon les criteres de l’European NeuroMuscular Center et l’absence de traitement anterieur. Les caracteristiques cliniques (âge, sexe, duree d’evolution, score Cutaneous Dermatomyositis Activity Severity Index, association a une neoplasie et atteinte pulmonaire) et biologiques (anticorps specifiques de myosites, CPK) etaient recueillies. Les sous-populations de lymphocytes T circulants chez les patients ayant une DMa etaient etudies en cytometrie en flux a l’aide de 18 fluorochromes et comparees a 19 donneurs sains. La mesure des cytokines seriques etait faite dans les serums par technique Luminex ® et etait comparee a 14 donneurs sains. Les resultats etaient exprimes en mediane des % pour les populations cellulaires et en mediane des concentrations (pg/mL) pour les cytokines. Le test bilateral de Student etait utilise, significatifs lorsque p Resultats Les patients ayant une DMa avaient une diminution des populations CD3 + CD4 - CD8 − (0,79 vs 1,49, p + CD28 null (78,1 vs 23,3, p + CD26 + et CD3 + CD26 + CD161 + (0,65 vs 7,04, p p p p p Discussion Il s’agit de la premiere etude sur les modifications phenotypiques lymphocytaires T specifique a la DMa. Il s’agit d’un groupe homogene, naif de traitement sans neoplasie associee. Nous confirmons certains resultats trouves chez les patients ayant une DM classique : augmentation des populations CD3 + CD8 + CD28 null et des taux seriques d’IL-6 et d’IL17A, en faveur d’une physiopathologie commune. Nous avons identifie de nouvelles voies immunitaires perturbees, connues dans le lupus ou la polyarthrite rhumatoide : diminution de l’expression du CD26 parmi les lymphocytes CD3 et diminution de la frequence des iNKT. Nous rapportons une augmentation significative de l’IL-10 liee au score d’activite clinique et regressant apres traitement.