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1.
J Eur Acad Dermatol Venereol ; 38(5): 904-909, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38112226

RESUMEN

BACKGROUND: Adalimumab monotherapy for hidradenitis suppurativa (HS) is often insufficient with a maximum clinical efficacy of 60% in Hidradenitis Suppurativa Clinical Response (HiSCR) and limited effect on draining tunnels. Data suggest that adalimumab therapy could be improved by concomitant antibiotics. OBJECTIVE: To compare the clinical effectiveness of adalimumab with clindamycin and rifampicin versus adalimumab monotherapy after 12 weeks. METHODS: This retrospective study included patients who started adalimumab with additional clindamycin and rifampicin and patients treated with adalimumab monotherapy, matched on sex and refined Hurley score. The primary outcome measure was the difference in change in the International Hidradenitis Suppurativa Severity Score System (IHS4) at 12 weeks. RESULTS: In total, 62 patients were included in the combination therapy group (n = 31) and adalimumab monotherapy group (n = 31), showing comparable IHS4 scores; 32.5 versus 29, p = 0.87 at baseline respectively. The combination therapy demonstrated greater clinical effectiveness expressed in median IHS4 improvement (-20 vs. -9, p < 0.001), IHS4-55 (74% vs. 36%, p = 0.002), median draining tunnel reduction (-4 vs. -2, p < 0.001) and pain response (47% vs. 27%, p = 0.02). CONCLUSION: Adalimumab initiated with clindamycin and rifampicin shows greater clinical effectiveness than adalimumab monotherapy. An important difference in effect was observed in the decrease of draining tunnels, addressing a serious limitation of adalimumab monotherapy.


Asunto(s)
Adalimumab , Clindamicina , Quimioterapia Combinada , Hidradenitis Supurativa , Rifampin , Humanos , Hidradenitis Supurativa/tratamiento farmacológico , Adalimumab/uso terapéutico , Adalimumab/administración & dosificación , Rifampin/uso terapéutico , Rifampin/administración & dosificación , Clindamicina/uso terapéutico , Clindamicina/administración & dosificación , Estudios Retrospectivos , Femenino , Masculino , Adulto , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Antibacterianos/uso terapéutico , Antibacterianos/administración & dosificación , Resultado del Tratamiento
2.
J Eur Acad Dermatol Venereol ; 34(5): 1050-1056, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-31630445

RESUMEN

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic skin disease characterized by inflammatory lesions that flare unpredictably. The impact of weekly adalimumab (ADAew) on HS flare is not well-characterized. OBJECTIVE: To evaluate the impact of disease flare on health-related quality of life (HRQOL) in moderate-to-severe HS patients and to determine the effect of ADAew on disease flare using integrated data from two phase 3 trials over 36 weeks. METHODS: In period A (12 weeks), Dermatology Life Quality Index (DLQI) score change from baseline was compared in patients who flared and those who did not, regardless of treatment. The proportion of patients experiencing flare, duration of flare and time to flare was evaluated for ADAew vs. placebo (PBO). In period B (24 weeks), proportion of patients experiencing flare who received continuous ADAew treatment through 36 weeks was assessed. RESULTS: HRQOL was markedly improved among those who did not experience flare. In period A, the proportion of patients who experienced flare was significantly lower with ADAew vs. PBO (12.3% vs. 35.3%, P < 0.001). ADAew patients also had longer time to first flare (101 days vs. 57 days; P < 0.001) and shorter flare duration (18.9 days vs. 32.0 days, respectively; P = 0.001) vs. PBO. Through 36 weeks of treatment, 20.2% of ADAew patients flared, and for those who achieved at least a partial clinical response to ADAew at 12 weeks, only 5.7% flared. CONCLUSIONS: Flare reduction is an important measure in HS that correlates with clinically meaningful improvement in HRQOL. ADAew reduces HS flare through 12 and subsequent 36 weeks of treatment.


Asunto(s)
Hidradenitis Supurativa , Adalimumab/uso terapéutico , Antiinflamatorios/uso terapéutico , Hidradenitis Supurativa/tratamiento farmacológico , Humanos , Calidad de Vida , Brote de los Síntomas , Resultado del Tratamiento
3.
J Eur Acad Dermatol Venereol ; 34(10): 2353-2361, 2020 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-32078194

RESUMEN

BACKGROUND: Mutations in the γ-secretase enzyme subunits have been described in multiple kindreds with familial hidradenitis suppurativa (HS). OBJECTIVE: In this study, we report a novel nicastrin (NCSTN) mutation causing HS in a Dutch family. We sought to explore the immunobiological function of NCSTN mutations using data of the Immunological Genome Project. METHODS: Blood samples of three affected and two unaffected family members were collected. Whole-genome sequencing was performed using genomic DNA isolated from peripheral blood leucocytes. Sanger sequencing was done to confirm the causative NCSTN variant and the familial segregation. The microarray data set of the Immunological Genome Project was used for thorough dissection of the expression and function of wildtype NCSTN in the immune system. RESULTS: In a family consisting of 23 members, we found an autosomal dominant inheritance pattern of HS and detected a novel splice site mutation (c.1912_1915delCAGT) in the NCSTN gene resulting in a frameshift and subsequent premature stop. All affected individuals had HS lesions on non-flexural and atypical locations. Wildtype NCSTN appears to be upregulated in myeloid cells like monocytes and macrophages, and in mesenchymal cells such as fibroblastic reticular cells and fibroblasts. In addition, within the 25 highest co-expressed genes with NCSTN we identified CAPNS1, ARNT and PPARD. CONCLUSION: This study reports the identification a novel NCSTN gene splice site mutation which causes familial HS. The associated immunobiological functions of NCSTN and its co-expressed genes ARNT and PPARD link genetics to the most common environmental and metabolic HS risk factors which are smoking and obesity.


Asunto(s)
Hidradenitis Supurativa , Secretasas de la Proteína Precursora del Amiloide/genética , Calpaína , Hidradenitis Supurativa/genética , Humanos , Glicoproteínas de Membrana , Mutación , Factores de Transcripción
4.
Br J Dermatol ; 181(2): 314-323, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-30657173

RESUMEN

BACKGROUND: Biologics targeting inflammatory mediators can achieve clinical improvements in hidradenitis suppurativa (HS). However, their clinical efficacy shows great interpatient variability in daily practice. OBJECTIVES: To investigate the anti-inflammatory potency of a selection of currently available biologics and prednisolone for the treatment of HS in an ex vivo skin culture system using lesional HS biopsies. METHODS: Lesional skin samples from 10 patients with HS and skin samples from five healthy controls were cultured ex vivo and exposed to prednisolone or biologics targeting tumour necrosis factor (TNF)-α, interleukin (IL)-17A, IL-12/23p40 or CD20 (adalimumab, infliximab, secukinumab, ustekinumab and rituximab, respectively). Real-time quantitative polymerase chain reaction and cytokine bead arrays were used to measure the inhibitory effect of the biologics on cytokines and antimicrobial peptides (AMPs). RESULTS: The relative mRNA expression of all tested cytokines and AMPs was significantly downregulated by all anti-inflammatory agents (P < 0·001). The protein production of the proinflammatory cytokines TNF-α, interferon γ, IL-1ß, IL-6 and IL-17A was significantly inhibited by adalimumab, infliximab, ustekinumab, prednisolone (all P < 0·001) and rituximab (P = 0·0071), but not by secukinumab (P = 0·0663). On both mRNA and protein levels, adalimumab, infliximab and prednisolone reduced the levels of a broader mix of individual cytokines than secukinumab, ustekinumab and rituximab. Moreover, a significant inhibitory effect on mRNA expression levels of inflammatory markers in healthy control skin was observed only for TNF-α inhibitors (P < 0·001) and prednisolone (P = 0·0015). CONCLUSIONS: This ex vivo study suggests that TNF-α inhibitors and prednisolone are the most powerful inhibitors of proinflammatory cytokines and AMPs in HS lesional skin, which concurs with our clinical experience in patients with HS.


Asunto(s)
Antiinflamatorios/farmacología , Productos Biológicos/farmacología , Hidradenitis Supurativa/tratamiento farmacológico , Prednisolona/farmacología , Piel/efectos de los fármacos , Adulto , Antiinflamatorios/uso terapéutico , Antígenos CD20/inmunología , Antígenos CD20/metabolismo , Péptidos Catiónicos Antimicrobianos/antagonistas & inhibidores , Péptidos Catiónicos Antimicrobianos/inmunología , Péptidos Catiónicos Antimicrobianos/metabolismo , Productos Biológicos/uso terapéutico , Biopsia , Femenino , Voluntarios Sanos , Hidradenitis Supurativa/inmunología , Hidradenitis Supurativa/patología , Hidradenitis Supurativa/cirugía , Humanos , Subunidad p40 de la Interleucina-12/antagonistas & inhibidores , Subunidad p40 de la Interleucina-12/inmunología , Subunidad p40 de la Interleucina-12/metabolismo , Interleucina-17/antagonistas & inhibidores , Interleucina-17/inmunología , Interleucina-17/metabolismo , Masculino , Persona de Mediana Edad , Técnicas de Cultivo de Órganos , Prednisolona/uso terapéutico , Piel/inmunología , Piel/metabolismo , Piel/patología , Factor de Necrosis Tumoral alfa/antagonistas & inhibidores , Factor de Necrosis Tumoral alfa/inmunología , Factor de Necrosis Tumoral alfa/metabolismo
5.
Br J Dermatol ; 180(5): 1214-1220, 2019 05.
Artículo en Inglés | MEDLINE | ID: mdl-30512186

RESUMEN

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, debilitating, heterogeneous disease requiring different treatment approaches. Recently, we refined the classic Hurley classification into a seven-stage classification in order to guide these treatment choices. This new classification subdivides Hurley stage I and II into three substages, namely mild (A), moderate (B) and severe (C) HS disease. Hurley stage III is not subcategorized and is always severe. OBJECTIVES: To investigate the correlation between the given severity grades of Hurley I and Hurley II in the refined Hurley classification, and the patient-reported quality of life and physician-assessed objective severity score. MATERIALS AND METHODS: In this cross-sectional study, patients with HS participating in the observational cohorts of two Dutch tertiary referral centres were included before June 2017. The patient-reported Dermatology Life Quality Index (DLQI) and physician-assessed International HS Severity Score System (IHS4) scores were compared between the refined Hurley stages. RESULTS: In total, 433 patients were analysed. DLQI and IHS4 scores increased within Hurley stage I and II from A through C. There was a significant positive correlation of DLQI and IHS4 with increasing refined Hurley substages [refined Hurley stage I (A, B and C) to DLQI: rs = 0·259, P < 0·001 and refined Hurley stage II (A, B and C) to DLQI: rs = 0·185, P = 0·010; refined Hurley stage I (A, B and C) to IHS4: rs = 0·603, P < 0·001 and refined Hurley stage II (A, B and C) to IHS4: rs = 0·532, P < 0·001]. CONCLUSIONS: The refined Hurley classification accurately correlates with HS severity assessed by both patients and clinicians. Therefore, the refined Hurley classification is a useful tool for the quick assessment of severity in HS.


Asunto(s)
Hidradenitis Supurativa/diagnóstico , Medición de Resultados Informados por el Paciente , Calidad de Vida , Índice de Severidad de la Enfermedad , Adulto , Estudios Transversales , Estudios de Factibilidad , Femenino , Hidradenitis Supurativa/complicaciones , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Países Bajos , Sistema de Registros/estadística & datos numéricos
6.
Br J Dermatol ; 181(3): 483-491, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-30724351

RESUMEN

BACKGROUND: Monitoring disease activity over time is a prerequisite for clinical practice and research. Valid and reliable outcome measurement instruments (OMIs) and staging systems provide researchers and clinicians with benchmark tools to assess the primary and secondary outcomes of interventional trials and to guide treatment selection properly. OBJECTIVES: To investigate inter-rater reliability and agreement in instruments currently used in hidradenitis suppurativa (HS), with dermatologists experienced in HS as the rater population of interest. METHODS: In a prospective completely balanced design, 24 patients with HS underwent a physical examination by 12 raters (288 assessments) using nine instruments. The results were analysed using generalized linear mixed models. RESULTS: For the staging systems, the study found good inter-rater reliability for Hurley staging in the axillae and gluteal region, moderate inter-rater reliability for Hurley staging in the groin and for Physician's Global Assessment, and fair inter-rater reliability for refined Hurley staging and the International HS Severity Scoring System. For all the tested OMIs, the observed intervals for limits of agreement were very wide relative to the ranges of the scales. CONCLUSIONS: The very wide intervals for limits of agreement imply that substantial changes are needed in clinical research in order to rule out measurement error. The results illustrate a difficulty, even for experienced HS experts, to agree on the type and number of lesions when evaluating disease severity. The apparent caveats call for global efforts, such as the HIdradenitis SuppuraTiva cORe outcomes set International Collaboration (HISTORIC) to reach consensus on how best to measure physical signs of HS reliably in randomized trials. What's already known about this topic? Without valid and reliable instruments to measure outcomes, researchers and clinicians lack the necessary benchmarks to assess primary and secondary end points of interventional trials properly. Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease. Several outcome measure instruments exist for HS, but their validation is generally incomplete or of relatively low methodological quality. What does this study add? Using a prospective completely balanced design this study examined inter-rater reliability with HS-experienced dermatologists as the rater population of interest. The study did not find very good reliability for any included instrument or lesion counts. This study illustrates the difficulty in finding agreement on the type and number of HS lesions, even among experts. The results question whether physical signs are best measured by a traditional physician lesion count instrument. What are the clinical implications of this work? For staging, Hurley staging and physician global visual analogue scale proved to be acceptable instruments in terms of inter-rater reliability. For the instruments designed to measure changes in health status, our study illustrates how difficult it is, even for experts, to measure the physical signs of HS using a simple rater counting. Consequently, other assessment methods of physicals signs, such as ultrasound evaluation, require consideration.


Asunto(s)
Hidradenitis Supurativa/diagnóstico , Evaluación del Resultado de la Atención al Paciente , Índice de Severidad de la Enfermedad , Adulto , Femenino , Hidradenitis Supurativa/terapia , Humanos , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Estudios Prospectivos , Ensayos Clínicos Controlados Aleatorios como Asunto , Reproducibilidad de los Resultados
7.
Br J Dermatol ; 181(6): 1198-1206, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-30919434

RESUMEN

BACKGROUND: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. OBJECTIVES: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. METHODS: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. CONCLUSIONS: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies. What's already known about this topic? The occurrence of pilonidal sinus disease has not been clearly reported among patients with hidradenitis suppurativa/acne inversa. What does this study add? This is the first study that investigated the prevalence of pilonidal sinus disease among a large cohort of patients and identified the patient characteristics. Risk factors that might help to improve the management of patients were identified.


Asunto(s)
Hidradenitis Supurativa/complicaciones , Seno Pilonidal/epidemiología , Adulto , Nalgas , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Seno Pilonidal/etiología , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Adulto Joven
8.
J Eur Acad Dermatol Venereol ; 33(4): 761-765, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30451329

RESUMEN

BACKGROUND: Treatment with apremilast has recently demonstrated clinically meaningful improvement in moderate hidradenitis suppurativa (HS). OBJECTIVE: To evaluate the change in expression of inflammatory markers in lesional skin of HS patients receiving apremilast 30 mg twice daily (n = 15) for 16 weeks compared with placebo (n = 5). METHODS: At baseline, 5-mm punch biopsies were obtained from an index lesion (HSL) and non-lesional (HSN) skin in the same anatomical area. Subsequent HSL samples were taken as close as possible to the previously biopsied site at week 4 and week 16. After sampling, biopsies were split; one half was processed for in vivo mRNA analysis using real-time quantitative PCR; the other half was cultured for ex vivo protein analysis using a proximity extension assay (Olink). Linear mixed effects models were calculated to compare the levels of inflammatory markers in HSL skin between apremilast and placebo over time. RESULTS: At baseline, 17 proteins with a fold change >2 in HSL vs. HSN skin were identified in 20 patients. The top five were IL-17A (5), S100A12, CST5, IL-12/23p40, CD6 (1) with fold changes ranging from 6.6 to 1638, respectively (FDR <0.044). Linear mixed effects models for 75 assays were calculated. Protein levels of S100A12 decreased during treatment in the apremilast group compared with the placebo group (p = 0.014, FDR = 0.186). None of the 14 genes exhibited significant changes in expression over time. However, an evident downward trend in relative mRNA expression of IL-17A and IL-17F was demonstrated in patients receiving apremilast. CONCLUSION: We did not detect statistically significant changes in inflammatory markers in HSL skin of HS patients receiving apremilast compared with placebo, despite clinical improvement in the apremilast group. Nonetheless, S100A12 and IL-17A were significantly elevated in HSL skin and showed a decrease in response to apremilast. The translational model in clinical trials involving HS clearly needs further improvement.


Asunto(s)
Antiinflamatorios no Esteroideos/uso terapéutico , Hidradenitis Supurativa/tratamiento farmacológico , Hidradenitis Supurativa/metabolismo , ARN Mensajero/metabolismo , Talidomida/análogos & derivados , Adulto , Antígenos CD/genética , Antígenos CD/metabolismo , Antígenos de Diferenciación de Linfocitos T/genética , Antígenos de Diferenciación de Linfocitos T/metabolismo , Biomarcadores/metabolismo , Cistatinas/genética , Cistatinas/metabolismo , Femenino , Hidradenitis Supurativa/genética , Humanos , Interleucina-12/genética , Interleucina-12/metabolismo , Interleucina-17/genética , Interleucina-17/metabolismo , Masculino , Persona de Mediana Edad , Proteína S100A12/genética , Proteína S100A12/metabolismo , Talidomida/uso terapéutico , Adulto Joven
9.
J Eur Acad Dermatol Venereol ; 33(9): 1633-1643, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31037773

RESUMEN

This paper is organized jointly by the European Academy of Dermatology and Venereology (EADV) Task Force (TF) on Quality of Life (QoL) and Patient-Oriented Outcomes and the EADV TF on acne, rosacea and hidradenitis suppurativa (ARHS). The purpose of this paper was to present current knowledge about QoL assessment in HS, including data on HS-specific health-related (HR) QoL instruments and HRQoL changes in clinical trials, and to make practical recommendations concerning the assessment of QoL in people with HS. HS results in significant quimp that is higher than in most other chronic skin diseases. HS impact in published studies was assessed predominantly (84% of studies) by the Dermatology Life Quality Index (DLQI). There is a lack of high-quality clinical trials in HS patients where HRQoL instruments have been used as outcome measures. One double-blind randomized placebo-controlled trial on infliximab with low number of participants reported significantly better HRQoL improvement in the treatment group than in the placebo group. Well-designed clinical studies in HS patients to compare different treatment methods, including surgical methods and assessing long-term effects, are needed. Because of lack of sufficient validation, the Task Forces are not at present able to recommend existing HS-specific HRQoL instruments for use in clinical studies. The EADV TFs recommend the dermatology-specific DLQI questionnaire for use in HS patients. The EADV TFs encourage the further development, validation and use of other HS-specific, dermatology-specific and generic instruments but such use should be based on the principles presented in the previous publications of the EADV TF on QoL and Patient-Oriented Outcomes.


Asunto(s)
Acné Vulgar/psicología , Acné Vulgar/terapia , Hidradenitis Supurativa/psicología , Hidradenitis Supurativa/terapia , Rosácea/psicología , Rosácea/terapia , Comités Consultivos , Europa (Continente) , Humanos , Medición de Resultados Informados por el Paciente , Guías de Práctica Clínica como Asunto , Calidad de Vida , Índice de Severidad de la Enfermedad
10.
J Eur Acad Dermatol Venereol ; 33(1): 19-31, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30176066

RESUMEN

Hidradenitis suppurativa (HS)/acne inversa is a debilitating chronic disease that remains poorly understood and difficult to manage. Clinical practice is variable, and there is a need for international, evidence-based and easily applicable consensus on HS management. We report here the findings of a systematic literature review, which were subsequently used as a basis for the development of international consensus recommendations for the management of patients with HS. A systematic literature review was performed for each of nine clinical questions in HS (defined by an expert steering committee), covering comorbidity assessment, therapy (medical, surgical and combinations) and response to treatment. Included articles underwent data extraction and were graded according to the Oxford Centre for Evidence-based Medicine criteria. Evidence-based recommendations were then drafted, refined and voted upon, using a modified Delphi process. Overall, 5310 articles were screened, 171 articles were analysed, and 65 were used to derive recommendations. These articles included six randomized controlled trials plus cohort studies and case series. The highest level of evidence concerned dosing recommendations for topical clindamycin in mild disease (with systemic tetracyclines for more frequent/widespread lesions) and biologic therapy (especially adalimumab) as second-line agents (following conventional therapy failure). Good-quality evidence was available for the hidradenitis suppurativa clinical response (HiSCR) as a dichotomous outcome measure in inflammatory areas under treatment. Lower-level evidence supported recommendations for topical triclosan and oral zinc in mild-to-moderate HS, systemic clindamycin and rifampicin in moderate HS and intravenous ertapenem in selected patients with more severe disease. Intralesional or systemic steroids may also be considered. Local surgical excision is suggested for mild-to-moderate HS, with wide excision for more extensive disease. Despite a paucity of good-quality data on management decisions in HS, this systematic review has enabled the development of robust and easily applicable clinical recommendations for international physicians based on graded evidence.


Asunto(s)
Antibacterianos/uso terapéutico , Hidradenitis Supurativa/tratamiento farmacológico , Hidradenitis Supurativa/epidemiología , Fumar/epidemiología , Adalimumab/uso terapéutico , Antiinflamatorios/uso terapéutico , Productos Biológicos/uso terapéutico , Comorbilidad , Consenso , Técnica Delphi , Hidradenitis Supurativa/cirugía , Humanos , Guías de Práctica Clínica como Asunto
11.
Br J Dermatol ; 178(3): 715-721, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29080368

RESUMEN

BACKGROUND: A core outcomes set (COS) is an agreed minimum set of outcomes that should be measured and reported in all clinical trials for a specific condition. Hidradenitis suppurativa (HS) has no agreed-upon COS. A central aspect in the COS development process is to identify a set of candidate outcome domains from a long list of items. Our long list had been developed from patient interviews, a systematic review of the literature and a healthcare professional survey, and initial votes had been cast in two e-Delphi surveys. In this manuscript, we describe two in-person consensus meetings of Delphi participants designed to ensure an inclusive approach to generation of domains from related items. OBJECTIVES: To consider which items from a long list of candidate items to exclude and which to cluster into outcome domains. METHODS: The study used an international and multistakeholder approach, involving patients, dermatologists, surgeons, the pharmaceutical industry and medical regulators. The study format was a combination of formal presentations, small group work based on nominal group theory and a subsequent online confirmation survey. RESULTS: Forty-one individuals from 13 countries and four continents participated. Nine items were excluded and there was consensus to propose seven domains: disease course, physical signs, HS-specific quality of life, satisfaction, symptoms, pain and global assessments. CONCLUSIONS: The HISTORIC consensus meetings I and II will be followed by further e-Delphi rounds to finalize the core domain set, building on the work of the in-person consensus meetings.


Asunto(s)
Hidradenitis Supurativa/terapia , Ensayos Clínicos como Asunto , Consenso , Conferencias de Consenso como Asunto , Técnica Delphi , Salud Global , Humanos , Resultado del Tratamiento
12.
J Eur Acad Dermatol Venereol ; 32(3): 459-462, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29265421

RESUMEN

BACKGROUND: Surgery is an important treatment modality for hidradenitis suppurativa (HS). Various methods of HS surgery have been described. Even though wide excision is a common surgical procedure for HS, data on the recurrence rate and patient satisfaction are scarce. OBJECTIVE: To determine the recurrence rate and patient satisfaction of HS lesional wide excision (complete excision) with secondary intention healing. METHODS: A single centre retrospective study. Hundred and twenty eligible patients were identified from our medical files, and an individualized questionnaire was sent. RESULTS: Eighty-six patents responded to our questionnaire (71.7%). Of whom, 84 patients underwent in total 253 procedures. The mean follow-up time per procedure was 36.2 months. In 37.6% of the procedures, recurrence occurred within a mean follow-up period of 3 years (after a median of 6.0 months). Total remission of an anatomical area was achieved in 49% of the procedures, whereas natural disease progression occurred in 13%. The genital region was the most prone to recurrence. The majority of the patients were glad that they had undergone the procedure and would recommend the surgical procedure to other HS patients. CONCLUSION: Lesional wide excision (complete excision) with secondary intention healing yields a meaningful local cure rate for HS and is well tolerated.


Asunto(s)
Hidradenitis Supurativa/cirugía , Satisfacción del Paciente , Cicatrización de Heridas , Adolescente , Adulto , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Hidradenitis Supurativa/complicaciones , Humanos , Masculino , Recurrencia , Inducción de Remisión , Adulto Joven
14.
Br J Dermatol ; 176(4): 1042-1047, 2017 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-27534591

RESUMEN

BACKGROUND: Hidradenitis suppurativa (HS) has a major impact on patients' quality of life (QoL). Although it has commonly been assumed that HS impairs sexual health, only a single case-control study has been performed on sexual functioning in a small group of patients with HS. OBJECTIVES: To investigate the QoL with a particular focus on sexual health in a substantial population of patients with HS. METHODS: In total 916 patients with HS received an invitation to participate in this multicentre cross-sectional survey. RESULTS: Three hundred patients completed the questionnaires. This study showed a diminished QoL and sexual health in patients with HS (Female Sexual Function Index: 21·6 ± 9·6, International Index of Erectile Function: 49·7 ± 20·7, Arizona Sexual Experience Scale: 16·7 ± 5·3, Dermatology Life Quality Index: 12·5 ± 7·5). Sexual health was associated with QoL in women but not in men. Female sex and late onset of HS were associated with poor sexual function. Impairment of QoL was associated with anogenital involvement, early onset of HS, disease severity and disease activity. CONCLUSIONS: HS is associated with impaired sexual health and QoL. Physicians should not hesitate to ask patients with HS about their sexual function and, when needed, offer them psychological support.


Asunto(s)
Hidradenitis Supurativa/psicología , Calidad de Vida , Disfunciones Sexuales Fisiológicas/etiología , Edad de Inicio , Anciano , Estudios Transversales , Femenino , Humanos , Enfermedades de Inicio Tardío , Masculino , Persona de Mediana Edad , Salud Reproductiva/estadística & datos numéricos , Encuestas y Cuestionarios
15.
Br J Dermatol ; 177(5): 1401-1409, 2017 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-28636793

RESUMEN

BACKGROUND: A validated tool for the dynamic severity assessment of hidradenitis suppurativa/acne inversa (HS) is lacking. OBJECTIVES: To develop and validate a novel dynamic scoring system to assess the severity of HS. METHODS: A Delphi voting procedure was conducted among the members of the European Hidradenitis Suppurativa Foundation (EHSF) to achieve consensus towards an initial HS Severity Score System (HS4). Strengths and weaknesses of HS4 were examined by a multicentre prospective study. Multivariate logistic regression, discriminant analysis and receiver operating characteristic curves, as well as examination for correlation (Spearman's rho) and agreement (Cohen's kappa) with existing scores, were engaged to recognize the variables for a new International HS4 (IHS4) that was established by a second Delphi round. RESULTS: Consensus HS4 was based on number of skin lesions, number of skin areas involved and Dermatology Life Quality Index (DLQI), and was evaluated by a sample of 236 patients from 11 centres. Subsequently, a multivariate regression model calculated adjusted odds ratios for several clinical signs. Nodules, abscesses and draining tunnels resulted as the scoring variables. Three candidate scores were presented to the second Delphi round. The resulting IHS4 score is arrived at by the number of nodules (multiplied by 1) plus the number of abscesses (multiplied by 2) plus the number of draining tunnels (multiplied by 4). A total score of 3 or less signifies mild, 4-10 signifies moderate and 11 or higher signifies severe disease. Cohen's kappa was fair (κ = 0·32) compared with Hurley classification, and moderate (κ = 0·49) compared with Expert Opinion. Correlation was good (ρ > 0·6) with Hurley classification, Expert Opinion, Physician's Global Assessment and Modified Sartorius score, and moderate for DLQI (ρ = 0·36). CONCLUSIONS: The novel IHS4 is a validated tool to dynamically assess HS severity and can be used both in real-life and the clinical trials setting.


Asunto(s)
Hidradenitis Supurativa/patología , Índice de Severidad de la Enfermedad , Adulto , Consenso , Femenino , Humanos , Masculino , Estudios Prospectivos , Calidad de Vida
17.
J Eur Acad Dermatol Venereol ; 29(4): 619-44, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25640693

RESUMEN

Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillae, inguinal and anogenital regions. A mean disease incidence of 6.0 per 100,000 person-years and an average prevalence of 1% has been reported in Europe. HS has the highest impact on patients' quality of life among all assessed dermatological diseases. HS is associated with a variety of concomitant and secondary diseases, such as obesity, metabolic syndrome, inflammatory bowel disease, e.g. Crohn's disease, spondyloarthropathy, follicular occlusion syndrome and other hyperergic diseases. The central pathogenic event in HS is believed to be the occlusion of the upper part of the hair follicle leading to a perifollicular lympho-histiocytic inflammation. A highly significant association between the prevalence of HS and current smoking (Odds ratio 12.55) and overweight (Odds ratio 1.1 for each body mass index unit) has been documented. The European S1 HS guideline suggests that the disease should be treated based on its individual subjective impact and objective severity. Locally recurring lesions can be treated by classical surgery or LASER techniques, whereas medical treatment either as monotherapy or in combination with radical surgery is more appropriate for widely spread lesions. Medical therapy may include antibiotics (clindamycin plus rifampicine, tetracyclines), acitretin and biologics (adalimumab, infliximab). A Hurley severity grade-relevant treatment of HS is recommended by the expert group following a treatment algorithm. Adjuvant measurements, such as pain management, treatment of superinfections, weight loss and tobacco abstinence have to be considered.


Asunto(s)
Hidradenitis Supurativa/etiología , Hidradenitis Supurativa/terapia , Guías de Práctica Clínica como Asunto , Europa (Continente) , Hidradenitis Supurativa/diagnóstico , Hidradenitis Supurativa/epidemiología , Humanos
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