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PubMed | National Center for Rare Skin disorders
Type: | Journal: Journal of the European Academy of Dermatology and Venereology : JEADV | Year: 2017

Anti-tumor necrosis factor alpha (TNF) therapies are used with success in many chronic inflammatory diseases. A wide range of adverse events has been reported in patients receiving these agents


PubMed | Hopital Pellegrin, National Center for Rare Skin Disorders and French Institute of Health and Medical Research
Type: Journal Article | Journal: Clinical and experimental dermatology | Year: 2016

Skin induration remains the major clinical symptom of systemic sclerosis (SSc), an autoimmune disease with potentially life-threatening visceral involvement. However, skin induration can be absent in some patients, making the diagnosis difficult to confirm and leading to delay in management. Skin pigmentation abnormalities have been reported in patients with SSc, and can be important to recognize for diagnosis. We report two patients who developed hyperpigmented skin patches without any sign of scleroderma, as a major clinical skin symptom of incipient SSc.


Ezzedine K.,National Center for Rare Skin Disorders | Ezzedine K.,University of Bordeaux 1 | Lim H.W.,Ford Motor Company | Suzuki T.,Yamagata University | And 19 more authors.
Pigment Cell and Melanoma Research | Year: 2012

During the 2011 International Pigment Cell Conference (IPCC), the Vitiligo European Taskforce (VETF) convened a consensus conference on issues of global importance for vitiligo clinical research. As suggested by an international panel of experts, the conference focused on four topics: classification and nomenclature; definition of stable disease; definition of Koebner's phenomenon (KP); and 'autoimmune vitiligo'. These topics were discussed in seven working groups representing different geographical regions. A consensus emerged that segmental vitiligo be classified separately from all other forms of vitiligo and that the term 'vitiligo' be used as an umbrella term for all non-segmental forms of vitiligo, including 'mixed vitiligo' in which segmental and non-segmental vitiligo are combined and which is considered a subgroup of vitiligo. Further, the conference recommends that disease stability be best assessed based on the stability of individual lesions rather than the overall stability of the disease as the latter is difficult to define precisely and reliably. The conference also endorsed the classification of KP for vitiligo as proposed by the VETF (history based, clinical observation based, or experimentally induced). Lastly, the conference agreed that 'autoimmune vitiligo' should not be used as a separate classification as published evidence indicates that the pathophysiology of all forms of vitiligo likely involves autoimmune or inflammatory mechanisms. © 2012 John Wiley & Sons A/S.


Ezzedine K.,National Center for Rare Skin Disorders | Ezzedine K.,Ghent University | Eleftheriadou V.,Institut Universitaire de France | Whitton M.,Institut Universitaire de France | Van Geel N.,University of Nottingham
The Lancet | Year: 2015

Vitiligo, an acquired pigmentary disorder of unknown origin, is the most frequent cause of depigmentation worldwide, with an estimated prevalence of 1%. The disorder can be psychologically devastating and stigmatising, especially in dark skinned individuals. Vitiligo is clinically characterised by the development of white macules due to the loss of functioning melanocytes in the skin or hair, or both. Two forms of the disease are well recognised: segmental and non-segmental vitiligo (the commonest form). To distinguish between these two forms is of prime importance because therapeutic options and prognosis are quite different. The importance of early treatment and understanding of the profound psychosocial effect of vitiligo will be emphasised throughout this Seminar. © 2015 Elsevier Ltd.


Gey A.,National Center for Rare Skin Disorders | Diallo A.,National Center for Rare Skin Disorders | Diallo A.,French Institute of Health and Medical Research | Seneschal J.,National Center for Rare Skin Disorders | And 7 more authors.
British Journal of Dermatology | Year: 2013

Background Vitiligo/nonsegmental vitiligo (NSV) is often associated with thyroid dysimmunity although very few reports have studied this association using multivariate logistic regression. Objective To identify weighted factors associated with the presence of autoimmune thyroid disease (AITD) in a large cohort of patients with vitiligo/NSV. Methods This was a prospective observational study in 626 patients with a confirmed diagnosis of vitiligo/NSV attending the vitiligo clinic of the University Hospital Department of Dermatology, Bordeaux, France, from 1 January 2006 to 1 May 2012. The Vitiligo European Task Force (VETF) questionnaire was completed for each consecutive patient. AITD was defined as the presence of significant levels of serum antithyroperoxidase antibodies or evidence of autoimmune thyroiditis. Univariate and multivariate logistic regression procedures were conducted to identify factors associated with AITD in this cohort of patients with vitiligo/NSV. Results A total of 626 patients with vitiligo/NSV were included, of whom 131 had AITD (AITD-vitiligo). Stress as an onset factor, familial history of AITD, body surface involvement and duration of the disease were positively associated with AITD-vitiligo using univariate analysis, whereas female sex, age at onset of vitiligo, personal history of autoimmune disease and localization on the trunk were found to be independently associated with AITD-vitiligo. Conclusion Vitiligo associated with AITD has clinical features distinct from vitiligo without AITD. In particular, female patients, and patients with longer duration of disease and greater body surface involvement are more likely to present with AITD and should thus be monitored for thyroid function and antithyroid antibodies on a regular basis. © 2012 The Authors. BJD © 2012 British Association of Dermatologists.


Ezzedine K.,National Center for Rare Skin Disorders | Ezzedine K.,French Institute of Health and Medical Research | Droitcourt C.,National Center for Rare Skin Disorders | Ged C.,French Institute of Health and Medical Research | And 6 more authors.
British Journal of Dermatology | Year: 2012

Background Loss of function FLG alleles were first identified as causative of ichthyosis vulgaris (IV) and were subsequently found to be major predisposing factors for atopic dermatitis (AD) and atopic disorders. Objectives To identify independent factors associated with the clinical IV phenotype in adult caucasian patients with AD and to assess the performance of a global clinical severity score of IV in predicting common FLG null mutations. Patients and methods This was a prospective study conducted from January 2007 to June 2008. Adult patients attending the department of dermatology with a diagnosis of AD with or without IV were eligible to participate. For each patient, five clinical signs of IV were scored from 0 to 3 - diffuse xerosis, hyperlinearity of palms, scales on legs, scalp desquamation and keratosis pilaris - and a global IV clinical severity score was derived (0-15). Age of onset of AD, SCORAD (SCORing of Atopic Dermatitis), family and personal history for other signs of atopy, and total immunoglobulin E were recorded. Genotyping was performed for R501X and 2282del4. Univariate and multivariate analysis for factors associated with AD or AD + IV were conducted. Results In univariate analysis, family history of atopy, global clinical severity scoring and 2282del4 FLG mutation were positively correlated with the AD + IV phenotype. Using multivariate analysis, SCORAD for AD (OR 0·94, P = 0·01) and global clinical severity scoring for AD + IV (OR 2·62, P < 0·0001) were found to be independent factors. Conclusions The 2282del4 FLG mutation was confirmed as a good marker of early-onset disease. Moreover, our global clinical severity score yielded a good negative predictive value of common caucasian null FLG mutations. © 2012 The Authors. BJD © 2012 British Association of Dermatologists.


Diallo A.,National Center for Rare Skin Disorders | Boniface K.,National Center for Rare Skin Disorders | Boniface K.,French Institute of Health and Medical Research | Jouary T.,National Center for Rare Skin Disorders | And 13 more authors.
Pigment Cell and Melanoma Research | Year: 2013

The relation of vitiligo/non-segmental vitiligo (NSV) to Koebner's phenomenon is variably appreciated. Our objective was to develop and validate a simple clinical score for Koebner's phenomenon (KP) in patients with vitiligo/NSV. The study population was composed of 351 individuals in the development sample and 285 patients in the validation sample. Seven variables were independently associated with the presence of KP: disease duration of more than 3 yr, forehead + scalp areas, eyelids, wrists, genital + belt areas, knees and tibial crests. The score computed by the weighted sum of the rounded coefficients of these seven variables ranged from 0 to 56 (mean 38.39 ± 22.93). The probability of having KP was computed as follows: exp (-2.37 + 0.1*score)/exp [1 + (-2.37 + 0.1*score)]. When applying the score to each patient in the validation and the development sample, the score maintained adequate discrimination and calibration (AUC-ROC = 0.78), arguing that KP can be adequately predicted using our score. Further studies should evaluate KP assessed by the K-VSCOR in clinical practice with the aim to determine its association with clinical profile, course and treatment response of vitiligo. © 2013 John Wiley & Sons A/S.


Ezzedine K.,National Center for Rare Skin Disorders | Ezzedine K.,French Institute of Health and Medical Research | Diallo A.,National Center for Rare Skin Disorders | Leaute-Labreze C.,National Center for Rare Skin Disorders | And 12 more authors.
British Journal of Dermatology | Year: 2012

Background Limited epidemiological data exist that compare clinical features of pre- and post-pubertal nonsegmental vitiligo. Objectives To compare factors associated with pre- and post-pubertal onset vitiligo. Patients and methods A prospective observational study was conducted of patients with vitiligo attending the clinic between 1 January 2006 and 1 July 2011. The Vitiligo European Task Force questionnaire was completed for each patient and thyroid function and antithyroid antibodies were screened. Other forms of vitiligo (segmental, focal, mucosal, not classifiable) were excluded. Results A total of 679 patients were included; 422 had post-pubertal and 257 pre-pubertal onset of vitiligo. Vitiligo universalis was seen only in post-pubertal onset. In univariate analysis, there was no significant statistical difference for sex, Koebner phenomenon or disease activity between both groups; thyroid disease or presence of thyroid antibodies was more frequent in post-pubertal onset [odds ratio (OR) 0·31, P < 0·003] whereas atopic dermatitis was more often associated with or preceding pre-pubertal onset (OR 2·42, P = 0·006). In multivariate analysis, halo naevi, family history of vitiligo, premature hair greying, atopic dermatitis and previous episode of spontaneous repigmentation were independently associated with pre-pubertal onset. In contrast, stress as onset factor, personal history of thyroid disease and acrofacial type were associated with post-pubertal onset. Conclusions Pre-pubertal onset vitiligo is strongly associated with personal and family history of atopy, suggesting that the predisposing immune background in vitiligo is not limited to autoimmunity, as also noted in alopecia areata. This study also suggests reconsidering the epidemiological data on sex ratio in vitiligo. © 2012 British Association of Dermatologists.


PubMed | National Center for Rare Skin Disorders
Type: Consensus Development Conference | Journal: Pigment cell & melanoma research | Year: 2012

During the 2011 International Pigment Cell Conference (IPCC), the Vitiligo European Taskforce (VETF) convened a consensus conference on issues of global importance for vitiligo clinical research. As suggested by an international panel of experts, the conference focused on four topics: classification and nomenclature; definition of stable disease; definition of Koebners phenomenon (KP); and autoimmune vitiligo. These topics were discussed in seven working groups representing different geographical regions. A consensus emerged that segmental vitiligo be classified separately from all other forms of vitiligo and that the term vitiligo be used as an umbrella term for all non-segmental forms of vitiligo, including mixed vitiligo in which segmental and non-segmental vitiligo are combined and which is considered a subgroup of vitiligo. Further, the conference recommends that disease stability be best assessed based on the stability of individual lesions rather than the overall stability of the disease as the latter is difficult to define precisely and reliably. The conference also endorsed the classification of KP for vitiligo as proposed by the VETF (history based, clinical observation based, or experimentally induced). Lastly, the conference agreed that autoimmune vitiligo should not be used as a separate classification as published evidence indicates that the pathophysiology of all forms of vitiligo likely involves autoimmune or inflammatory mechanisms.


PubMed | National Center for Rare Skin Disorders
Type: Journal Article | Journal: The British journal of dermatology | Year: 2013

Vitiligo/nonsegmental vitiligo (NSV) is often associated with thyroid dysimmunity although very few reports have studied this association using multivariate logistic regression.To identify weighted factors associated with the presence of autoimmune thyroid disease (AITD) in a large cohort of patients with vitiligo/NSV.This was a prospective observational study in 626 patients with a confirmed diagnosis of vitiligo/NSV attending the vitiligo clinic of the University Hospital Department of Dermatology, Bordeaux, France, from 1 January 2006 to 1 May 2012. The Vitiligo European Task Force (VETF) questionnaire was completed for each consecutive patient. AITD was defined as the presence of significant levels of serum antithyroperoxidase antibodies or evidence of autoimmune thyroiditis. Univariate and multivariate logistic regression procedures were conducted to identify factors associated with AITD in this cohort of patients with vitiligo/NSV.A total of 626 patients with vitiligo/NSV were included, of whom 131 had AITD (AITD-vitiligo). Stress as an onset factor, familial history of AITD, body surface involvement and duration of the disease were positively associated with AITD-vitiligo using univariate analysis, whereas female sex, age at onset of vitiligo, personal history of autoimmune disease and localization on the trunk were found to be independently associated with AITD-vitiligo.Vitiligo associated with AITD has clinical features distinct from vitiligo without AITD. In particular, female patients, and patients with longer duration of disease and greater body surface involvement are more likely to present with AITD and should thus be monitored for thyroid function and antithyroid antibodies on a regular basis.

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