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6. Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

Author

Greenblatt, D.T., primary, Pillay, E., additional, Snelson, K., additional, Saad, R., additional, Torres Pradilla, M., additional, Widhiati, S., additional, Diem, A., additional, Knight, C., additional, Thompson, K., additional, Azzopardi, N., additional, Werkentoft, M., additional, Moore, Z., additional, Patton, D., additional, Mayre‐Chilton, K.M., additional, Murrell, D.F., additional, and Mellerio, J.E., additional

Published
2021
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7. Phase 2 BELIEVE study part B: Efficacy and safety of rilzabrutinib for patients with pemphigus vulgaris.

Author

Murrell, D.F., Patsatsi, A., Stavropoulos, P., Baum, S., Zeeli, T., Kern, J.S., Sinclair, R., Neale, A., Arora, P., Sugerman, P.B., Shi, G., Werth, V.P., Caux, F., Joly, P., and Stavropolous, P.

Subjects
*PEMPHIGUS vulgaris, *IRRITABLE colon, *PEMPHIGUS, *PATIENT safety, *BRUTON tyrosine kinase, *EMPLOYEE ownership
Abstract

Keywords: bullous disorders; disease control; pemphigus EN bullous disorders disease control pemphigus 852 855 4 09/19/22 20221001 NES 221001 Pemphigus vulgaris (PV) is a potentially life-threatening, blistering autoimmune disease commonly treated with first-line corticosteroids (CS).1,2 The oral, covalent Bruton tyrosine kinase (BTK) inhibitor rilzabrutinib3 was validated in proof-of-concept BELIEVE part A phase 2 study (NCT02704429) at 400-600 mg bid doses (±CS) for 12 weeks (mean CS = 0.18 mg/kg/day) plus 12 weeks follow-up in newly diagnosed/relapsing, mild-to-severe PV.4 Part B expanded these findings in patients aged 18-80 years with biopsy-proven, newly diagnosed (<=6 months from diagnosis) or relapsing PV, and Pemphigus Disease Area Index (PDAI) score 8-60.5 Patients initially received oral rilzabrutinib 400 mg qd with optional dose escalation to 400 mg bid at/after week 2, and further to a maximum 600 mg bid dose at/after week 4. Although results were limited by a small number of patients, rilzabrutinib (±CS) demonstrated a consistently favourable benefit-risk profile with broader rilzabrutinib doses and longer treatment in newly diagnosed/relapsing pemphigus patients. One patient with chronic, relapsing pemphigus (PDAI 36 at baseline) and worsening disease failed to achieve CDA despite rilzabrutinib dose escalation, was hospitalized at week 9 to receive intravenous immunoglobulin/rituximab, and discontinued the study. [Extracted from the article]

Published
2022
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8. European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I

Author

Rashid, H., primary, Lamberts, A., additional, Borradori, L., additional, Alberti‐Violetti, S., additional, Barry, R.J., additional, Caproni, M., additional, Carey, B., additional, Carrozzo, M., additional, Caux, F., additional, Cianchini, G., additional, Corrà, A., additional, Diercks, G.F.H., additional, Dikkers, F.G., additional, Di Zenzo, G., additional, Feliciani, C., additional, Geerling, G., additional, Genovese, G., additional, Hertl, M., additional, Joly, P., additional, Marzano, A.V., additional, Meijer, J.M., additional, Mercadante, V., additional, Murrell, D.F., additional, Ormond, M., additional, Pas, H.H., additional, Patsatsi, A., additional, Prost, C., additional, Rauz, S., additional, van Rhijn, B.D., additional, Roth, M., additional, Schmidt, E., additional, Setterfield, J., additional, Zambruno, G., additional, Zillikens, D., additional, and Horváth, B., additional

Published
2021
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10. 157 A comparison study of Outcome Measures for Epidermolysis Bullosa; Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and the Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB).

Author

Daniel B.S., Gibson M., Kern J.S., Martin L.K., Robertson S.J., Su J.C., Murrell D.F., Rogers C., Daniel B.S., Gibson M., Kern J.S., Martin L.K., Robertson S.J., Su J.C., Murrell D.F., and Rogers C.

Abstract

The success of clinical trials in Epidermolysis Bullosa (EB) is dependent upon the availability of a valid and reliable scoring tool that can accurately assess and monitor disease severity. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and Instrument for Scoring Clinical Outcomes of Research for Epidermolysis Bullosa (iscorEB) were independently developed and validated against the Birmingham Epidermolysis Bullosa Severity Score (BEBS), but have never been directly compared. Our objective was to compare the reliability, convergent validity and discriminant validity of the EBDASI and iscorEB scoring tools. An observational cohort study was conducted in 15 patients with EB. Each patient was evaluated by six dermatologists with expertise in EB using the EBDASI and iscorEB-clinician scoring tools. Quality of life was assessed using the iscorEB-patient and Quality of Life in EB measures. The intraclass correlation coefficients (ICC) for inter-rater reliability were: EBDASI 0.942 and the iscorEB-clinician 0.852. The ICC for intra-rater reliability was 0.99 for both scores. The two tools demonstrated strong convergent validity with each-other. In conclusion, both scoring tools demonstrate excellent reliability. The EBDASI appears to better discriminate between EB types and disease severities.Copyright © 2021

Published
2021

15. Nemolizumab is associated with a rapid improvement in atopic dermatitis signs and symptoms: subpopulation (EASI ≥ 16) analysis of randomized phase 2B study

Author

Silverberg, J.I., primary, Pinter, A., additional, Alavi, A., additional, Lynde, C., additional, Bouaziz, J.‐D., additional, Wollenberg, A., additional, Murrell, D.F., additional, Alpizar, S., additional, Laquer, V., additional, Chaouche, K., additional, Ahmad, F., additional, Armstrong, J.M., additional, and Piketty, C., additional

Published
2021
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17. International collaboration

Author

Goldman, M.P., Murrell, D.F., Al-Mutairi, N., Arenbergerova, M., Dreno, B., Alhaddad, M., Al-Suwaidan, S.N., Firooz, A., Kerkhof, P.C.M. van de, Feldman, S.R., Goldman, M.P., Murrell, D.F., Al-Mutairi, N., Arenbergerova, M., Dreno, B., Alhaddad, M., Al-Suwaidan, S.N., Firooz, A., Kerkhof, P.C.M. van de, and Feldman, S.R.

Abstract

Contains fulltext : 229445.pdf (Publisher’s version ) (Closed access)

Published
2020

18. Tacrolimus ointment does not affect the immediate response to vaccination, the generation of immune memory, or humoral and cell-mediated immunity in children

Author

Hofman, T., Cranswick, N., Kuna, P., Boznanski, A., Latos, T., Gold, M., Murrell, D.F., Gebauer, K., Behre, U., Machura, E., Olafsson, J., and Szalai, Z.

Subjects
Tacrolimus -- Dosage and administration, Tacrolimus -- Research, Atopic dermatitis -- Care and treatment, Immune response -- Research, Children -- Diseases, Children -- Care and treatment
Published
2006

19. Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*.

Author

Greenblatt, D.T., Pillay, E., Snelson, K., Saad, R., Torres Pradilla, M., Widhiati, S., Diem, A., Knight, C., Thompson, K., Azzopardi, N., Werkentoft, M., Moore, Z., Patton, D., Mayre‐Chilton, K.M., Murrell, D.F., and Mellerio, J.E.

Subjects
EPIDERMOLYSIS bullosa, CHILDBIRTH, PREGNANCY, POPES
Abstract

Linked Comment:A.W. Lucky and E. Pope. Br J Dermatol 2022; 186:602–603. Plain language summary available online [ABSTRACT FROM AUTHOR]

Published
2022
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21. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

Author

Joly, P., primary, Horvath, B., additional, Patsatsi, Α, additional, Uzun, S., additional, Bech, R., additional, Beissert, S., additional, Bergman, R., additional, Bernard, P., additional, Borradori, L., additional, Caproni, M., additional, Caux, F., additional, Cianchini, G., additional, Daneshpazhooh, M., additional, De, D, additional, Dmochowski, M., additional, Drenovska, K., additional, Ehrchen, J., additional, Feliciani, C., additional, Goebeler, M., additional, Groves, R., additional, Guenther, C., additional, Hofmann, S., additional, Ioannides, D., additional, Kowalewski, C., additional, Ludwig, R., additional, Lim, Y.L., additional, Marinovic, B., additional, Marzano, A.V., additional, Mascaró, J.M., additional, Mimouni, D., additional, Murrell, D.F., additional, Pincelli, C., additional, Squarcioni, C.P., additional, Sárdy, M., additional, Setterfield, J., additional, Sprecher, E., additional, Vassileva, S., additional, Wozniak, K., additional, Yayli, S., additional, Zambruno, G., additional, Zillikens, D., additional, Hertl, M., additional, and Schmidt, E., additional

Published
2020
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23. Sensitivity to change and correlation between the autoimmune bullous disease quality‐of‐life questionnaires ABQOL and TABQOL , and objective severity scores

Author

Ferries, L., primary, Gillibert, A., additional, Duvert‐Lehembre, S., additional, Corbaux, C., additional, Alexandre, M., additional, Prost‐Squarcioni, C., additional, Caux, F., additional, Delaporte, E., additional, Litrowski, N., additional, Boulard, C., additional, Murrell, D.F., additional, Joly, P., additional, and Hébert, V., additional

Published
2020
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24. Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility

Author

Has, C., primary, Bauer, J.W., additional, Bodemer, C., additional, Bolling, M.C., additional, Bruckner‐Tuderman, L., additional, Diem, A., additional, Fine, J.‐D., additional, Heagerty, A., additional, Hovnanian, A., additional, Marinkovich, M.P., additional, Martinez, A.E., additional, McGrath, J.A., additional, Moss, C., additional, Murrell, D.F., additional, Palisson, F., additional, Schwieger‐Briel, A., additional, Sprecher, E., additional, Tamai, K., additional, Uitto, J., additional, Woodley, D.T., additional, Zambruno, G., additional, and Mellerio, J.E., additional

Published
2020
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26. Proof of concept for the clinical effects of oral rilzabrutinib, the first Bruton tyrosine kinase inhibitor for pemphigus vulgaris: the phase II BELIEVE study*.

Author

Murrell, D.F., Patsatsi, A., Stavropoulos, P., Baum, S., Zeeli, T., Kern, J.S., Roussaki‐Schulze, A.‐V., Sinclair, R., Bassukas, I.D., Thomas, D., Neale, A., Arora, P., Caux, F., Werth, V.P., Gourlay, S.G., and Joly, P.

Subjects
*PEMPHIGUS vulgaris, *PROTEIN-tyrosine kinase inhibitors, *DESMOGLEINS, *RITUXIMAB, *ADVERSE health care events, *PROOF of concept, *PROTEIN-tyrosine kinases
Abstract

Summary: Background: Bruton tyrosine kinase (BTK) inhibition targets B‐cell and other non‐T‐cell immune cells implicated in the pathophysiology of pemphigus, an autoimmune disease driven by anti‐desmoglein autoantibodies. Rilzabrutinib is a new reversible, covalent BTK inhibitor demonstrating preclinical efficacy as monotherapy in canine pemphigus foliaceus. Objectives: To evaluate the efficacy and safety of oral rilzabrutinib in patients with pemphigus vulgaris in a multicentre, proof‐of‐concept, phase II trial. Methods: Patients with Pemphigus Disease Area Index severity scores 8–45 received 12 weeks of oral rilzabrutinib 400–600 mg twice daily and 12 weeks of follow‐up. Patients initially received between 0 and ≤ 0·5 mg kg−1 prednisone‐equivalent corticosteroid (CS; i.e. 'low dose'), tapered after control of disease activity (CDA; no new lesions, existing lesions healing). The primary endpoints were CDA within 4 weeks on zero‐to‐low‐dose CS and safety. Results: In total, 27 patients with pemphigus vulgaris were included: nine newly diagnosed (33%) and 18 relapsing (67%); 11 had moderate disease (41%) and 16 moderate to severe (59%). The primary endpoint, CDA, was achieved in 14 patients (52%, 95% confidence interval 32–71): 11 using low‐dose CS and three using no CS. Over 12 weeks of treatment, mean CS doses reduced from 20·0 to 11·8 mg per day for newly diagnosed patients and from 10·3 to 7·8 mg per day for relapsing patients. Six patients (22%) achieved complete response by week 24, including four (15%) by week 12. Treatment‐related adverse events were mostly mild (grade 1 or 2); one patient experienced grade 3 cellulitis. Conclusions: Rilzabrutinib alone, or with much lower CS doses than usual, was safe, with rapid clinical activity in pemphigus vulgaris. These data suggest that BTK inhibition may be a promising treatment strategy and support further investigation of rilzabrutinib for the treatment of pemphigus. What is already known about this topic?Standard pemphigus treatment relies on systemic high‐dose corticosteroids (CS), rituximab and/or immunosuppressives, which are limited by delayed onset of action and potential toxicities.Immune‐mediated mechanisms that are fast acting on both the innate and adaptive immune systems, are steroid sparing, and have safety profiles well suited for chronic administration are greatly needed for patients with pemphigus. What does this study add?Rilzabrutinib is an oral Bruton tyrosine kinase (BTK) inhibitor targeting B‐cell and other non‐T‐cell immune cells implicated in pemphigus pathophysiology.Treatment with rilzabrutinib (with or without low‐dose CS) demonstrated rapid disease control and a well‐tolerated safety profile in patients with newly diagnosed and relapsing pemphigus vulgaris.BELIEVE provides evidence for a promising treatment strategy via BTK inhibition, supporting further investigation of rilzabrutinib in other immune‐mediated diseases. Linked Comment: A.M. Drucker and N.H. Shear. Br J Dermatol 2021; 185:691–692. Plain language summary available online [ABSTRACT FROM AUTHOR]

Published
2021
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28. Inter‐rater reliability of the BIOCHIP indirect immunofluorescence dermatology mosaic in bullous pemphigoid and pemphigus patients

Author

Yang, A., primary, Xuan, R.R., additional, Melbourne, W., additional, Hashimoto, T., additional, Uzun, S., additional, Daneshpazhooh, M., additional, Yamagami, J., additional, Di Zenzo, G., additional, Mascaro, J.M., additional, Mahmoudi, H., additional, Patsatsi, A., additional, Drenovska, K., additional, Vassileva, S., additional, and Murrell, D.F., additional

Published
2019
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31. International Bullous Diseases Group: consensus on diagnostic criteria for epidermolysis bullosa acquisita

Author

Prost-Squarcioni, C., Caux, F., Schmidt, E., Jonkman, M.F., Vassileva, S., Kim, S.C., Iranzo, P., Daneshpazhooh, M., Terra, J., Bauer, J., Fairley, J., Hall, R., Hertl, M., Lehman, J.S., Marinovic, Branka, Patsatsi, A., Zillikens, D., Werth, V., Woodley, D.T., Murrell, D.F., and the International Bullous Diseases Group

Subjects
Epidermolysis bullosa acquisita, SERRATION PATTERN-ANALYSIS, Microscopy, Electron, Scanning Transmission, medicine.medical_specialty, epidermolysis bullosa acquisita, diagnostic criteria, consensus conference, Consensus, media_common.quotation_subject, education, Immunoblotting, MEDLINE, Fluorescent Antibody Technique, Enzyme-Linked Immunosorbent Assay, Dermatology, Disease, Review, Epidermolysis Bullosa Acquisita, VII COLLAGEN ANTIBODIES, Diagnosis, Differential, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Voting, medicine, Journal Article, Bullous disease, Humans, Intensive care medicine, Microscopy, Immunoelectron, health care economics and organizations, media_common, SPLIT-SKIN, LINKED-IMMUNOSORBENT-ASSAY, DIRECT IMMUNOFLUORESCENCE MICROSCOPY, business.industry, Clinical Laboratory Techniques, Consensus conference, OUTCOME MEASURES, medicine.disease, SCANNING CONFOCAL MICROSCOPY, CROHNS-DISEASE, Flow chart, 030220 oncology & carcinogenesis, Differential diagnosis, business, CHLORIDE-SEPARATED SKIN, LINEAR IGA DISEASE
Abstract

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a complex autoimmune bullous disease disease with variable clinical presentations and multiple possible diagnostic tests, making an international consensus on the diagnosis of EBA essential. ----- OBJECTIVES: To obtain an international consensus on the clinical and diagnostic criteria for EBA. ----- METHODS: The International Bullous Diseases Group (IBDG) met three times to discuss the clinical and diagnostic criteria for EBA. For the final voting exercise, 22 experts from 14 different countries voted on 50 different items. When > 30% disagreed with a proposal, a discussion was held and re-voting carried out. ----- RESULTS: In total, 48 of 50 proposals achieved consensus after discussion. This included nine diagnostic criteria, which are summarized in a flow chart. The IBDG was unable to determine one procedure that would be applicable worldwide. A limitation of the study is that differential diagnosis of bullous systemic lupus erythematosus has not been addressed. ----- CONCLUSIONS: This first international consensus conference established generally agreed-upon clinical and laboratory criteria defining the clinical classification of and diagnostic testing for EBA. Holding these voting exercises in person with the possibility of discussion prior to voting has advantages in reaching consensus over Delphi exercises with remote voting.

Published
2017

32. Validation of the BIOCHIP test for the diagnosis of bullous pemphigoid, pemphigus vulgaris and pemphigus foliaceus.

Author

Yang, A., Xuan, R., Melbourne, W., Tran, K., and Murrell, D.F.

Subjects
IMMUNOFLUORESCENCE, PEMPHIGUS, DIAGNOSIS, BULLOUS pemphigoid
Abstract

Background: The BIOCHIP is a novel multiplex indirect immunofluorescence technique used in the serological diagnosis of bullous pemphigoid and pemphigus. The BIOCHIP method combines the screening of autoantibodies and target antigen‐specific substrates in a single miniature incubation field. Objective: To evaluate the diagnostic accuracy of the new immunofluorescence BIOCHIP multiplex tool in pemphigus and bullous pemphigoid. Methods: For the validation of the BIOCHIP, sera from patients with BP (n = 38), PF (n = 8) and pemphigus vulgaris (PV) (n = 23) were used. In addition, sera from disease control patients (n = 63) and healthy volunteers (n = 39) were used. The multiplex BIOCHIP and direct immunofluorescence (DIF) were performed for all BP, PF and PV patients. Additional indirect immunofluorescence (IIF) was performed on patients with BP, and ELISA was performed on patients with pemphigus. Results: The BIOCHIP mosaic showed a sensitivity of 86.8% and specificity of 85% for BP180 or BP230 being positive in BP. It demonstrated a sensitivity of 75% and specificity of 97.7% for Dsg1 in PF. The BIOCHIP was found to have a sensitivity of 60.9% and specificity of 73.6% for Dsg3 in PV. Conclusion: The BIOCHIP mosaic‐based immunofluorescence test is potentially a simple, time and effort saving test that can aid in the diagnosis and screening of BP, PV and PF. However, there is potential for interpretation bias and a learning curve that needs to be taken into consideration. [ABSTRACT FROM AUTHOR]

Published
2020
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38. Calculation of cut-off values based on the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus

Author

Boulard, C., Duvert Lehembre, S., Picard-Dahan, C., Kern, J.S., Zambruno, G., Feliciani, C., Marinovic, B., Vabres, P., Borradori, L., Prost-Squarcioni, C., Labeille, B., Richard, M.A., Ingen-Housz-Oro, S., Houivet, E., Werth, V.P., Murrell, D.F., Hertl, M., Bénichou, J., Joly, P., Service de Dermatologie [Rouen], Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Service de Dermatologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de dermatologie [Avicenne], Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre de Recherches en Oncologie biologique et Oncopharmacologie (CRO2), Aix Marseille Université (AMU)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie, vénéreologie et cancérologie cutanée [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service de dermatologie [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Unité de biostatistiques [CHU Rouen], Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Pharmacoepidemiologie et évaluation de l'impact des produits de santé sur les populations, Université Bordeaux Segalen - Bordeaux 2-Université de Rouen Normandie (UNIROUEN), Hôpital Charles Nicolle [Rouen]-CHU Rouen, CHU Saint-Etienne, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Bordeaux Segalen - Bordeaux 2-Université de Rouen Normandie (UNIROUEN), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 (UPD7), Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Avicenne, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), and Unité de biostatistiques [Rouen]

Subjects
integumentary system, skin and connective tissue diseases, [SDV.MHEP.DERM]Life Sciences [q-bio]/Human health and pathology/Dermatology
Abstract

International audience; BACKGROUND:Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus.OBJECTIVES:To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores.METHODS:In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment (PGA) and Dermatology Life Quality Index (DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups.RESULTS:Ninety-six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (P < 0·001), PDAI (P < 0·001), PGA (P < 0·001) and DLQI (P = 0·03) scores.CONCLUSIONS:This study suggests cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus.

Published
2016

39. Calculation of cut‐off values based on the Autoimmune Bullous Skin Disorder Intensity Score ( <scp>ABSIS</scp> ) and Pemphigus Disease Area Index ( <scp>PDAI</scp> ) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus

Author

Boulard, C., Duvert Lehembre, S., Picard‐Dahan, C., Kern, J.S., Zambruno, G., Feliciani, C., Marinovic, B., Vabres, P., Borradori, L., Prost‐Squarcioni, C., Labeille, B., Richard, M.A., Ingen‐Housz‐Oro, S., Houivet, E., Werth, V.P., Murrell, D.F., Hertl, M., Benichou, J., Joly, P., Hofmann, S., Camaioni, B., Didona, B., Fania, L., Della Torre, R., Caux, F., Alexandre, M., Paul, C., Bulai Livideanu, C., Delaporte, E., Dupuy, A., Avenel‐Audran, M., Schmidt, E., Schumacher, N., Bagot, M., Tancrede‐Bohin, E., Bouaziz, J.D., D'Incan, M., Quereux, G., Skowron, F., Beylot‐Barry, M., Doutre, M.S., Bedane, C., Bernard, P., Machet, L., Jullien, ., Debarbieux, S., Dereure, O., Hünefeld, C., Schanz, S., Franck, N., Maillard, H., Misery, L., Sassolas, B., Abasq, C., Bouyeure, A.C., Lagoutte, P., Ferranti, V., and the International Pemphigus Study Group

Subjects
pemphigus vulgaris, pemphigus foliaceus, severity scores
Abstract

Background: Two pemphigus severity scores, Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values that allow differentiation between moderate, significant and extensive types of pemphigus. Objectives: To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. Methods: In 31 dermatology departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using ABSIS, PDAI, Physician's Global Assessment (PGA) and Dermatology Life Quality Index (DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25th and 75th percentiles of the ABSIS and PDAI scores. The median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared in order to validate these subgroups. Results: Ninety-six patients with pemphigus vulgaris (n = 77) or pemphigus foliaceus (n = 19) were included. The median PDAI activity and ABSIS total scores were 27·5 (range 3-84) and 34·8 points (range 0·5-90·5), respectively. The respective cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (P < 0·001), PDAI (P < 0·001), PGA (P < 0·001) and DLQI (P = 0·03) scores. Conclusions: This study suggests cut-off values of 15 and 45 for PDAI and 17 and 53 for ABSIS, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage patients with pemphigus.

Published
2016

40. Report from the fourth international consensus meeting to harmonize core outcome measures for atopic eczema/dermatitis clinical trials (HOME initiative): British Journal of Dermatology

Author

Chalmers, J. R., Simpson, E., Apfelbacher, C. J., Thomas, S.K., von Kobyletzki, L., Schmitt, J., Singh, J.A., Svensson, A., Williams, H.C., Abuabara, K., Aoki, V., Ardeleanu, M., Awici-Rasmussen, M., Barbarot, S., Berents, T. L., Block, J., Bragg, A., Burton, T., Clemmensen, K. K. B., Creswell-Melville, A., Dinesen, M., Drucker, A., Eckert, R.L., Flohr, C., Garg, M., Gerbens, L. A. A., Graff, A. L. B., Hanifin, J., Heinl, D., Humphreys, R., Ishii, H.A., Kataoka, Y., Leshem, Y. A., Marquort, B., Massuel, M. A., Merhand, S., Mizutani, H., Murota, H., Murrell, D.F., Nakahara, T., Nasr, I., Nograles, K., Ohya, Y., Osterloh, I., Pander, J., Prinsen, C., Purkins, L., Ridd, M.J., Sach, T., Schuttelaar, M. L. A., Shindo, S., Smirnova, J., Sulzer, A., Gjerde, E. S., Takaoka, R., Talmo, H. V., Tauber, M., Torchet, F., Volke, A., Wahlgren, C.F., Weidinger, S., Weisshaar, E., Wollenberg, A., Yamaga, K., Zhao, C. Y., Spuls, P.I., Epidemiology and Data Science, and EMGO - Quality of care

Abstract

This article is a report of the fourth meeting of the Harmonising Outcome Measures for Eczema (HOME) initiative held in Malmo, Sweden on 23-24 April 2015 (HOME IV). The aim of the meeting was to achieve consensus over the preferred outcome instruments for measuring patient-reported symptoms and quality of life for the HOME core outcome set for atopic eczema (AE). Following presentations, which included data from systematic reviews, consensus discussions were held in a mixture of whole group and small group discussions. Small groups were allocated a priori to ensure representation of different stakeholders and countries. Decisions were voted on using electronic keypads. For the patient-reported symptoms, the group agreed by vote that itch, sleep loss, dryness, redness/inflamed skin and irritated skin were all considered essential aspects of AE symptoms. Many instruments for capturing patient-reported symptoms were discussed [ including the Patient-Oriented SCOring Atopic Dermatitis index, Patient-Oriented Eczema Measure (POEM), Self-Administered Eczema Area and Severity Index, Itch Severity Scale, Atopic Dermatitis Quickscore and the Nottingham Eczema Severity Score] and, by consensus, POEM was selected as the preferred instrument to measure patient-reported symptoms. Further work is needed to determine the reliability and measurement error of POEM. Further work is also required to establish the importance of pain/soreness and the importance of collecting information regarding the intensity of symptoms in addition to their frequency. Much of the discussion on quality of life concerned the Dermatology Life Quality Index and Quality of Life Index for Atopic Dermatitis; however, consensus on a preferred instrument for measuring this domain could not be reached. In summary, POEM is recommended as the HOME core outcome instrument for measuring AE symptoms.

Published
2016

44. Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: Best clinical practice guidelines.

Author

Murrell D.F., McGrath J.A., Morris S., Palisson F., Salas-Alanis J.C., Robson A., Pillay E.I., Mellerio J.E., Robertson S.J., Bernardis C., Diem A., Fine J.D., George R., Goldberg D., Halmos G.B., Harries M., Jonkman M.F., Lucky A., Martinez A.E., Maubec E., Murrell D.F., McGrath J.A., Morris S., Palisson F., Salas-Alanis J.C., Robson A., Pillay E.I., Mellerio J.E., Robertson S.J., Bernardis C., Diem A., Fine J.D., George R., Goldberg D., Halmos G.B., Harries M., Jonkman M.F., Lucky A., Martinez A.E., and Maubec E.

Abstract

This article summarizes recommendations reached following a systematic literature review and expert consensus on the diagnosis and management of cutaneous squamous cell carcinomas in people with epidermolysis bullosa. The guidelines are intended to help inform decision making by clinicians dealing with this complex complication of a devastating disease.Copyright © 2015 The Authors. British Journal of Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.

Published
2016

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