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1.
JAAD Int ; 10: 6-13, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36387063

RESUMO

Background: Alopecia is a complication of autoimmune blistering diseases (AIBDs) that affects patients' quality of life; however, it has generally been overlooked in patients with severe disease because it is regarded as a cosmetic issue. Objective: To study the epidemiologic data and clinical presentations of alopecia in our cohort of patients with AIBDs. Methods: Forty-one patients with AIBDs were assessed in this cross-sectional study. An assessment tool to collate patient information, including AIBD scalp involvement, trichoscopic findings, and Severity of Alopecia Tool II scores, was used. Results: More than 70% of patients in our cohort had at least 1 type of alopecia, with 10% presenting with a nonspecific (end-stage) scarring alopecia. Elevated Dsg1 ratios were predictive of hair loss in pemphigus vulgaris (P < .001) and increased alopecia was associated with worse disease severity in bullous pemphigoid (P = .001). Limitations: The small sample size and lack of severe cases. Conclusion: There is a likelihood that 1 in 10 patients with AIBDs have a scarring alopecia related to their disease. To our knowledge, this is the first study including alopecia prevalence in patients with bullous pemphigoid, which was not significantly increased despite providing clues to disease severity.

2.
Pediatr Dermatol ; 2022 Nov 13.
Artigo em Inglês | MEDLINE | ID: mdl-36372450

RESUMO

Median canaliform nail dystrophy (MCD) is a rare nail abnormality with an unknown etiology. We report the case of MCD of both great toenails in a 2-year-old boy presenting with a fir tree nail pattern and longitudinal splits. MCD was treated with topical marigold therapy (Tagetes sp.). By 15 weeks, the proximal 50% of the MCD had normalized. The report highlights a potential new treatment of marigold therapy for MCD.

3.
Front Med (Lausanne) ; 9: 1057096, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36405625

RESUMO

Introduction: Bullous pemphigoid (BP) is the most common subtype of autoimmune blistering diseases that primarily affects the elderly and is classically defined by the presence of IgG and/or complement C3 against the BP180 and BP230 hemidesmosome proteins. However, most recent studies have introduced the role of specific eosinophil receptors and chemokine mediators in the pathogenesis of BP which are helpful in identifying new targets for future treatments. Areas covered: This review will focus on the involvement of eosinophils in BP, including the processes that lead to their recruitment, activation, and regulation. Subsequently, covering new therapeutic options in relation to the role of eosinophils. Eotaxin enhances the recruitment of eosinophils in BP, with CCR3 chemoreceptor that is expressed on eosinophils being identified as a key binding site for eotaxin-1. The pathogenic role of IgE and IL-4 in BP is corroborated by successful treatments with Omalizumab and Dupilumab, respectively. IL-5, IL-17 and IL-23 inhibitors may be effective given their roles in promoting eosinophilia. Expert opinion: Further research into inhibitors of eotaxin, IL-4, IL-5, IL-17, IL-23, CCR3, and specific complement factors are warranted as preliminary studies have largely identified success in treating BP with these agents. Learning from novel treatments for other IgG-mediated autoimmune diseases may be beneficial.

5.
Int J Womens Dermatol ; 8(3): e053, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36225612

RESUMO

Autoimmune bullous diseases are a group of skin disorders resulting from an autoimmune reaction against intercellular adhesion molecules or components of the basement membrane of skin and mucosa. Autoimmune disorders often occur in patients with a history of another autoimmune disease and most autoimmune diseases have a striking female predominance. In this review, we aim to analyze the different associations of autoimmune bullous diseases with other autoimmune diseases and highlight the distinctiveness of the female gender in these associations.

6.
Dermatol Ther ; : e15902, 2022 Oct 06.
Artigo em Inglês | MEDLINE | ID: mdl-36200713

RESUMO

Rituximab (RTX) combined with short-term glucocorticoids (GC) is an effective therapeutic option for pemphigus. The newly developed Glucocorticoid Toxicity Index (GTI) tool provides the possibility to measure GC toxicities over time. To compare 1-year GTI between two groups of RTX-treated and RTX-naïve patients with pemphigus. The responsiveness of the GTI was also investigated. A prospective cohort of 129 adults with newly diagnosed pemphigus was conducted. GC-related toxicities were assessed at 3-month intervals according to Composite and Specific lists of the GTI. Of the patients, 76.7% (n = 99) received RTX. Throughout the time intervals, RTX-treated patients had lower GTI compared to RTX-naïve ones (p = 0.036). The mean GTI at 1-year was 34.3 in the RTX-treated group and 50.8 in the RTX-naïve group (p = 0.04). The most commonly observed GC-related toxicity was neuropsychiatric manifestations for 34% (224 events). The relapse rate of RTX-treated patients (1%) was significantly lower than RTX-naïve patients (10%) (p = 0.037). The GTI showed no correlation with cumulative GC consumption in both groups (p > 0.05, both). Patients treated with GC alone had remarkably higher GTI than patients treated with GC plus RTX. The GTI is an applicable tool to quantitatively capture GC toxicities at the patient level in pemphigus.

7.
JAAD Int ; 9: 33-43, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36089938

RESUMO

Background: Systemic glucocorticoids are first-line treatment options for autoimmune blistering diseases; however, their long-term use is associated with significant toxicities. Objective: To evaluate the side effects of steroid-sparing agents and compare them with those of steroids. Methods: We searched Cochrane Reviews, Embase, MEDLINE, and Scopus between October 1978 and May 2020 using the keywords "bullous pemphigoid," "pemphigus," "autoimmune blistering diseases," and "side effects." A total of 31 randomized controlled trials and retrospective case series were critically appraised. Results: This review includes a total of 1685 patients with autoimmune blistering diseases, of whom 781 had bullous pemphigoid and 904 had either pemphigus vulgaris or pemphigus foliaceous. Limitations: A major limitation is that because adjuvants are generally used in combination with steroids, only 12 of the studies reviewed included a "steroid-only" arm to allow for a direct comparison of side effects. Additionally, there is inadequate literature and lack of standardized grade reporting of specific side effects of each steroid-sparing agent. Conclusion: In the future, researchers should consider implementing the Common Terminology Criteria for Adverse Events, version 5.0, for reporting of all side effects to allow for consistency and standardization. It would be useful to have an index similar to the Glucocorticoid Toxicity Index to quantify these side effects.

8.
Br J Dermatol ; 2022 Aug 17.
Artigo em Inglês | MEDLINE | ID: mdl-35975634

RESUMO

This study shows that gain-of-function variants in KLHL24 causing EBS and DCM, do not only originate in the start-codon and suggest that any nonsense-inducing variant affecting nucleotides c.4_84 will likely cause the same effect on protein level and a similar potential lethal phenotype.

10.
Orphanet J Rare Dis ; 17(1): 314, 2022 08 13.
Artigo em Inglês | MEDLINE | ID: mdl-35964087

RESUMO

BACKGROUND: Standardized assessments for dystrophic epidermolysis bullosa (DEB) are needed. This prospective, multicenter, 4-week, observational study was designed to evaluate DEB assessments for suitability as clinical trial endpoints. METHODS: Patients with confirmed DEB diagnosis and ≥ 5 measurable wounds were included. The primary outcome was change from baseline in wound surface area (WSA) of 5 selected wounds by 3-dimensional imaging. Secondary endpoints were change from baseline in clinician global assessment (CGA) of WSA, wound characteristics, disease-related questionnaires and instruments (disease severity, quality of life [QoL], pain and disability, and itch), and tolerability of procedures. RESULTS: Of 30 enrolled patients, 29 completed the study (of whom, 28 had recessive DEB). Median age was 17.8 years (range, 3.8-58.7). All patients developed new or recurrent wounds during the 4-week study. Of the wounds selected at baseline, 45/150 (30.0%) healed by week 2; an additional 38 healed by week 4, while 8 of those healed at week 2 had recurred by week 4 for a total of 75/150 (50.0%) healed wounds at week 4. Mean values for WSA, CGA, and disease-related questionnaire and instrument scores remained steady during this 4-week observational study. Of the 10 disease-related questionnaires and instruments assessed, the scores for the Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI) and the Instrument for Scoring Clinical Outcomes for Research of Epidermolysis Bullosa (iscorEB) did not substantially overlap between moderate and severe disease. Between mild and moderate disease, only the EBDASI scores did not substantially overlap. CONCLUSIONS: These results stress the dynamic nature of wounds, even during a 4-week period of observation, and suggest that a combination of clinician-assessed outcomes and patient-/caregiver-reported outcomes is needed to provide a comprehensive assessment of DEB severity and impact. In addition, these results support the use of EBDASI and iscorEB to monitor disease severity as both produced scores that did not substantially overlap between disease severity strata. Clinical trial registration ClinicalTrials.gov, NCT02178969 . Registered 4 June 2014, https://clinicaltrials.gov/ct2/show/NCT02178969 .


Assuntos
Epidermólise Bolhosa Distrófica , Epidermólise Bolhosa , Adolescente , Epidermólise Bolhosa/complicações , Humanos , Estudos Prospectivos , Qualidade de Vida , Índice de Gravidade de Doença
12.
Australas J Dermatol ; 63(3): 312-320, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35816576

RESUMO

BACKGROUND: Psoriasis imposes a disease burden that can have a profound negative impact on patients' quality of life (QoL). HOPE was the first non-interventional study conducted in patients with severe chronic plaque psoriasis in Australia that evaluated health-related QoL in response to treatment with secukinumab. METHODS: HOPE was a prospective, open-label, single-arm, multicentre, non-interventional, exploratory study in patients with severe chronic plaque psoriasis in Australia. The study investigated the change in QoL, using the Dermatology Life Quality Index (DLQI), Assessment Quality of Life-8 Dimension questionnaire (AQoL-8D) and Psoriasis Area and Severity Index (PASI), and safety profile in response to treatment with secukinumab 300 mg SC weekly for 4 weeks followed by monthly maintenance for 58 weeks. RESULTS: At Week 14, the mean percentage reduction in total DLQI score from baseline was -82.4% (n = 65), which indicates a substantial improvement in QoL. This level of improvement was sustained up to Week ≥58, with a mean percentage change of -87.4%. The mean percentage change from baseline for AQoL-8D weighted total score decreased from Week 14 (41.1%) to Week 58 (35.2%), indicating an improvement in patients' QoL. A high proportion of patients achieved PASI 75/90/100 responses at Week 14 (97.0%/71.2%/34.8%), with rates sustained up to Week ≥58 (100%/87.9%/43.1%). The safety profile of secukinumab was favourable, with no cumulative or unexpected safety concerns. CONCLUSION: Secukinumab treatment demonstrated a striking improvement in patients' QoL in the HOPE study, the first real-world study in patients with severe chronic plaque psoriasis in the Australian clinical setting.


Assuntos
Anticorpos Monoclonais Humanizados , Psoríase , Anticorpos Monoclonais Humanizados/efeitos adversos , Austrália , Humanos , Estudos Prospectivos , Psoríase/tratamento farmacológico , Qualidade de Vida , Resultado do Tratamento
14.
JID Innov ; 2(4): 100129, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35860447

RESUMO

Many treatments are currently proposed for treating patients with bullous pemphigoid (BP). We assessed treatment modalities of BP depending on the different countries, BP extent, and patients' comorbidities. We surveyed worldwide experts about how they treat patients with BP. A total of 61 experts from 27 countries completed the survey. Severe and moderate BP were treated with oral prednisone (61.4 and 53.7%, respectively) or superpotent topical corticosteroids (CSs) (38.6 and 46.3%, respectively). Conventional immunosuppressants were more frequently combined with oral prednisone (74.5%) than with superpotent topical CS (37.5%) in severe BP. Topical CSs were mainly used in Europe in mild (81.1%), moderate (55.3%), and severe (54.3%) BP. In the United States of America and Asia, systemic CSs were mainly proposed for treating severe (77.8 and 100%, respectively), moderate (70 and 77.8%, respectively), and also mild (47.1 and 33.3%, respectively) BP. Most experts reduced the initial dose of oral CS in patients with diabetes mellitus (48.1%) or cardiac insufficiency (40.2%) but rarely changed BP treatment in patients with neurological disorders or neoplasia. This survey showed major differences in the way patients with BP are treated between AmeriPac countries (United State of America, Latin America, and Australia) and Asia on the one hand and Europe and the Middle East on the other hand.

15.
Orphanet J Rare Dis ; 17(1): 270, 2022 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-35841105

RESUMO

BACKGROUND: Epidermolysis bullosa simplex (EBS) comprises a group of rare, blistering genodermatoses. Prior work has been limited by small sample sizes, and much remains unexplored about the disease burden and health-related quality of life (QOL) of patients with EBS. The aim of this study was to characterize the most common patient-reported clinical manifestations and the health-related impact of QOL in EBS, and to examine differences in disease burden by age. METHODS: Patients with a diagnosis of epidermolysis bullosa (EB) or their caregivers completed a one-time online survey administered by EBCare, an international online EB registry. Survey data from respondents self-reporting a diagnosis of EBS were analyzed for clinical and wound manifestations, medication use, and QOL (using Quality of Life in Epidermolysis Bullosa [QOLEB] scores). Differences across age groups were assessed using Kruskal-Wallis and Fisher's exact tests. RESULTS: There were 214 survey respondents with EBS. The mean age was 32.8 years (standard deviation = 19.2). Many respondents reported blisters (93%), recurrent wounds (89%), pain (74%), chronic wounds (59%), itch (55%), and difficulty walking (44%). Mean QOLEB score was 14.7 (standard deviation = 7.5) indicating a "moderate" impact on QOL, and 12% of respondents required regular use of opiates. Findings were consistent in subgroup analyses restricted to respondents with diagnostic confirmation via genetic testing or skin biopsy (n = 63 of 214). Age-stratified analyses revealed differences in disease burden: younger respondents were more likely to self-report severe disease (24% vs. 19% vs. 5% for respondents aged 0-9 vs. 10-17 vs. 18 + , p = 0.001), failure to thrive (9% vs. 15% vs. 3%, p = 0.02), and use of gastrostomy tubes (15% vs. 12% vs. 1%, p < 0.001) and topical antibiotics (67% vs. 69% vs. 34%, p < 0.001), while older respondents were more likely to be overweight or obese (6% vs. 0% vs. 51%, p < 0.001) and have difficulty walking (24% vs. 46% vs. 48%, p = 0.04). CONCLUSIONS: In the largest international cross-sectional survey of EBS patients conducted, respondents reported extensive disease burden including significant wounding, pain, itch, difficulty walking, and impact on QOL. Age stratified disease manifestations. These findings suggest significant unmet need, and treatment and counseling for EBS patients should consider age-specific differences.


Assuntos
Epidermólise Bolhosa Simples , Epidermólise Bolhosa , Adulto , Efeitos Psicossociais da Doença , Estudos Transversais , Epidermólise Bolhosa/genética , Humanos , Limitação da Mobilidade , Dor , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida
16.
J Drugs Dermatol ; 21(5): 510-516, 2022 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-35533034

RESUMO

Malignant melanoma is one of the most aggressive solid tumors but has low morbidity if treated at an early stage. Over the past decade, the advent of targeted therapy and immunotherapy have productively enriched the treatment options for advanced melanomas and further improved the prognosis. The treatment of melanoma is a rapidly evolving field. In patients with sentinel lymph node (SLN)-positive (SLN+) melanoma, the role of complete lymph node dissection (CLND) is still a matter of debate. Sentinel lymph node biopsy (SLNB) is a staging procedure for melanoma that is routinely offered to patients with tumor thickness ≥1 mm or ≥0.8 mm with additional risk factors and is widely accepted as an important diagnostic and prognostic tool, since SLN+ patients can receive adjuvant targeted treatment or immunotherapy. Currently, the role of CLND has largely been replaced by often recommended adjuvant therapies since their approval. This article provides an overview of sentinel lymph-node surgery in cutaneous melanoma. J Drugs Dermatol. 2022;21(5):510-516. doi:10.36849/JDD.6198.


Assuntos
Melanoma , Linfonodo Sentinela , Neoplasias Cutâneas , Humanos , Fatores Imunológicos , Metástase Linfática/patologia , Melanoma/diagnóstico , Melanoma/patologia , Melanoma/cirurgia , Prognóstico , Estudos Retrospectivos , Linfonodo Sentinela/patologia , Linfonodo Sentinela/cirurgia , Biópsia de Linfonodo Sentinela/métodos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgia
18.
Int J Womens Dermatol ; 8(1): e004, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35620025

RESUMO

In autoimmune bullous diseases (AIBDs), autoantibodies loosen molecular adhesions in the skin and/or mucosa and lead to blisters and erosions. Immunosuppressive drugs reduce mortality of the AIBD; therefore, patients will have to live longer with comorbidities. Objective: This study aims to determine the quality of life of AIBD patients undergoing systemic treatment while investigating the survey's relationship with various factors. Methods: In this 2-step cross-sectional study, we initially included 53 consecutive pemphigus patients to investigate reliability and validity of the Persian version of Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaire. Then, we conducted the study on 119 AIBD patients, currently under treatment at an AIBD clinic in Iran. Results: The mean TABQOL score for our patients was 13.87 ± 7.51. The highest TABQOL was for epidermolysis bullosa acquisita (24 ± 8.485) followed by pemphigus foliaceus (20.5 ± 14.181) and the lowest for pemphigus vulgaris (13.24 ± 6.54). There was no significant difference between patients' TABQOL scores and their gender, history of rituximab injection, and disease severity scores. We only found a positive correlation between TABQOL and prednisolone dose. Conclusion: Treatments of AIBD considerably impact the quality of life of patients and an impairment in quality of life is correlated to higher doses of prednisolone.

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