-
Rheumatology International Jul 2024VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered autoinflammatory condition characterised by somatic mutation of the UBA1... (Review)
Review Meta-Analysis
BACKGROUND
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered autoinflammatory condition characterised by somatic mutation of the UBA1 gene. The syndrome leads to multi-system inflammation affecting predominantly the skin, lungs and bone marrow.
METHODS
We undertook a systematic review of the multisystem features and genotypes observed in VEXAS syndrome. Articles discussing VEXAS syndrome were included. Medline, Embase and Cochrane databases were searched. Information was extracted on: demographics, type and prevalence of clinical manifestations, genetic mutations and treatment. Meta-analysis using a random effects model was used to determine pooled estimates of serum markers.
RESULTS
From 303 articles, 90 were included, comprising 394 patients with VEXAS. 99.2% were male, with a mean age of 67.1 years (SD 8.5) at disease onset. The most frequent diagnoses made prior to VEXAS were: relapsing polychondritis (n = 59); Sweet's syndrome (n = 24); polyarteritis nodosa (n = 11); and myelodysplastic syndrome (n = 10). Fever was reported in 270 cases (68.5%) and weight loss in 79 (20.1%). Most patients had haematological (n = 342; 86.8%), dermatological (n = 321; 81.5%), pulmonary (n = 297; 75.4%%) and musculoskeletal (n = 172; 43.7%) involvement, although other organ manifestations of varying prevalence were also recorded. The most commonly reported mutations were "c.122T > C pMET41Thr" (n = 124), "c.121A > G pMET41Val" (n = 62) and "c.121A > C pMet41Leu" (n = 52). Most patients received glucocorticoids (n = 240; 60.9%) followed by methotrexate (n = 82; 20.8%) and IL-6 inhibitors (n = 61, 15.4%). One patient underwent splenectomy; 24 received bone marrow transplants.
CONCLUSION
VEXAS syndrome is a rare disorder affecting predominantly middle-aged men. This is the first systematic review to capture clinical manifestations, genetics and treatment of reported cases. Further studies are needed to optimise treatment and subsequently reduce morbidity and mortality.
Topics: Humans; Male; Ubiquitin-Activating Enzymes; Female; Mutation; Syndrome; Aged; Middle Aged; Myelodysplastic Syndromes; Sweet Syndrome; Polyarteritis Nodosa; Hereditary Autoinflammatory Diseases
PubMed: 38129348
DOI: 10.1007/s00296-023-05513-0 -
Journal of Plastic, Reconstructive &... Jan 2024Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis that can develop at a surgical site. Diagnosis can be challenging at its presentation causing... (Review)
Review
BACKGROUND
Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic dermatosis that can develop at a surgical site. Diagnosis can be challenging at its presentation causing delays in appropriate treatment. The aim of this study is to review the current literature as well as to describe the clinical presentation, diagnostic pathway, and treatment of PG after reduction mammaplasty in order to define a standardized multidisciplinary diagnostic and therapeutic approach. In the future, this may ease early identification and prompt treatment, and eventually minimize severe morbidity and long-term sequelae.
METHODS
The entire PubMed/Medline database was screened following the PRISMA guidelines to identify studies describing PG that have occurred after reduction mammoplasty.
RESULTS
Twenty-eight articles including 31 patients reported a PG after breast reduction surgery between January 1988 and March 2022. Twenty-one (68%) patients presented with skin ulcerations, 14 (45%) with erythema, and 5 (16%) with vesicles. Out of the 30 cases that underwent bilateral surgery, 18 (60%) developed PG bilaterally. In 12 out of 31 patients, nipple-areolar complex (NAC) involvement was evaluated, though in 10 patients (83%) the NAC was spared. Of the 20 patients (65%) who underwent skin biopsies for histopathological examination, 18 (90%) showed neutrophilic infiltration of the dermal layers. All 31 patients (100%) showed rapid clinical improvement after the introduction of immunosuppressive therapy.
CONCLUSIONS
PG can result in devastating skin alterations also after reduction mammoplasty, if misdiagnosed. However, it presents with constant yet unspecific local and general signs and symptoms that can be recognized to early initiate an appropriate pharmacological treatment.
Topics: Female; Humans; Pyoderma Gangrenosum; Mammaplasty; Skin; Mastectomy; Immunosuppression Therapy
PubMed: 38118291
DOI: 10.1016/j.bjps.2023.11.041 -
Archives of Dermatological Research Dec 2023There is increasing demand for natural and sustainable products for the treatment of dermatologic conditions. This systematic review aims to critically analyze published... (Review)
Review
There is increasing demand for natural and sustainable products for the treatment of dermatologic conditions. This systematic review aims to critically analyze published randomized controlled trials (RCTs) and provide evidence-based recommendations on the therapeutic use of curcumin for a variety of dermatological diseases. A systematic search of published literature was performed on July 18, 2023 using PRISMA guidelines for turmeric or curcumin for the treatment of skin diseases. Clinical recommendations were made based on the Oxford Centre for Evidence-Based Medicine guidelines. We identified 18 original randomized controlled trials for use of turmeric or curcumin for psoriasis, radiation dermatitis, oral lichen planus, pruritis, vitiligo, tinea capitis, facial erythema, and scarring. Psoriasis, cesarean section scar, and pruritus received grade of recommendation B. Radiation dermatitis, oral lichen planus, vitiligo, tinea capitis, and facial redness received grade of recommendation C or D. Curcumin was demonstrated to have an excellent safety profile in all clinical trials analyzed. Further research is required to determine optimal dosing and treatment parameters of turmeric. Additional, larger, RCTs and non-RCTs should be conducted to further investigate the safety and efficacy of curcumin as a treatment option for dermatological diseases.
Topics: Humans; Curcumin; Lichen Planus, Oral; Vitiligo; Psoriasis; Tinea Capitis; Dermatitis
PubMed: 38085369
DOI: 10.1007/s00403-023-02754-8 -
The Australasian Journal of Dermatology Mar 2024Toxic epidermal necrolysis (TEN) involves extensive mucocutaneous loss, and care is supportive. The approach to wound care includes surgical debridement or using... (Meta-Analysis)
Meta-Analysis Review
Toxic epidermal necrolysis (TEN) involves extensive mucocutaneous loss, and care is supportive. The approach to wound care includes surgical debridement or using dressings while leaving the epidermis intact. Robust evidence for either approach is lacking. We compared surgical debridement to the use of dressings while leaving the epidermis in situ (referred to hereon as dressings) in adult patients with TEN. The primary outcome assessed was mortality. The secondary outcome was time to re-epithelialisation. The impact of medications was evaluated. An individual patient data (IPD) systematic review and meta-analysis was undertaken. A random effects meta-analysis and survival analysis for IPD data examined mortality, re-epithelisation time and the effect of systemic medications. The quality of evidence was rated per the Grading of Recommendations Assessment, Development and Evaluation (GRADE). PROSPERO: CRD42021266611 Fifty-four studies involving 227 patients were included in the systematic review and meta-analysis, with a GRADE from very low to moderate. There was no difference in survival in patients who had surgical debridement or dressings (univariate: p = 0.91, multivariate: p = 0.31). Patients who received dressings re-epithelialised faster than patients who underwent debridement (multivariate HR: 1.96 [1.09-3.51], p = 0.023). Intravenous immunoglobulin (univariate HR: 0.21 [0.09-0.45], p < 0.001; multivariate HR: 0.22 [0.09-0.53], p < 0.001) and cyclosporin significantly reduced mortality (univariate HR: 0.09 [0.01-0.96], p = 0.046; multivariate HR: 0.06 [0.01-0.73], p = 0.028) irrespective of the wound care. This study supports the expert consensus of the dermatology hospitalists, that wound care in patients with TEN should be supportive with the epidermis left intact and supported with dressings, which leads to faster re-epithelialisation.
Topics: Adult; Humans; Stevens-Johnson Syndrome; Bandages; Cyclosporine; Immunoglobulins, Intravenous
PubMed: 38063272
DOI: 10.1111/ajd.14193 -
Journal of Drugs in Dermatology : JDD Dec 2023Porokeratosis is a group of disorders characterized by aberrant skin keratinization secondary to genetic alterations in the mevalonate pathway, which participates in... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Porokeratosis is a group of disorders characterized by aberrant skin keratinization secondary to genetic alterations in the mevalonate pathway, which participates in cholesterol synthesis. While a rare disorder, malignant transformation to squamous cell carcinoma is seen in up to 11% of cases. Recently, topical cholesterol and topical statin therapy have been suggested as a pathogenesis-directed treatment for porokeratosis.
METHODS
A PubMed/MEDLINE and Embase literature search was performed using the search terms: "porokeratosis" AND "cholesterol" OR "lovastatin" OR "simvastatin" OR "atorvastatin" OR "fluvastatin" OR "pitavastatin" OR "pravastatin" OR "rosuvastatin" OR "statin." Peer-reviewed clinical trials, case series, and case reports of all porokeratosis subtypes were included.
RESULTS
Eleven articles were included in the systematic review and 9 articles in the meta-analysis. The systematic review consisted of an aggregate of 33 patients, most of whom (n=31, 93.9%) applied the treatment twice daily for an average of 9.4 weeks (median=8 weeks), with 93.9% (n=31) experiencing improvement or resolution of porokeratosis. Sixteen patients (48.5%) used lovastatin and 16 (48.5%) used simvastatin with concurrent cholesterol therapy. Mild adverse events including erythema and contact dermatitis were experienced by 12.1% of patients. Our meta-analysis yielded a random effects model supporting a robust reduction in porokeratosis severity (OR = .076, 95% CI [0.022, 0.262]).
CONCLUSION
This underpowered meta-analysis provides limited, preliminary evidence supporting the efficacy of topical cholesterol/statin therapy. Overall, quality studies and aggregated sample size are limited; future large clinical trials are needed to further elucidate the role of topical cholesterol/statin therapy in the treatment of porokeratosis. J Drugs Dermatol. 2023;22(12):1160-1165. doi:10.36849/JDD.7775.
Topics: Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Porokeratosis; Lovastatin; Simvastatin; Cholesterol
PubMed: 38051843
DOI: 10.36849/JDD.7775 -
Skin Health and Disease Dec 2023Immunotherapy has become a mainstay of treatment for many cancers. Multiple immune checkpoint inhibitors have been used to treat malignancies, including anti-programed... (Review)
Review
Immunotherapy has become a mainstay of treatment for many cancers. Multiple immune checkpoint inhibitors have been used to treat malignancies, including anti-programed death-1 (PD1) and anti-cytotoxic T-lymphocyte-associated protein (anti-CTLA4). However, a significant percentage of patients develop resistance to these immunotherapy drugs. Therefore, novel strategies were developed to target other aspects of the immune response. Lymphocyte activation gene-3 (LAG-3) is a cell-surface molecule found on natural killer cells and activated T-cells which negatively regulates T-cell proliferation and function. LAG-3 inhibitors interact with LAG-3 ligands on the surface of T-cells to block T-regulatory (Treg) cell activity, suppress cytokine secretion and restore dysfunctional effector T-cells which subsequently attack and destroy cancer cells. This review reports the dermatologic side effects associated with LAG-3 inhibitors used in the treatment of melanomas. Using PRISMA 2022 guidelines, a comprehensive literature review of PubMed, Google Scholar, Embase, Cochrane, and Web of Science databases was conducted. Three studies were identified that demonstrated that the use of LAG-3 inhibitors, whether as a single agent or in combination with other immune checkpoint inhibitors, resulted in stomatitis, pruritus, rash, dry skin, erythema, and vitiligo. Further research is warranted to assess the cutaneous adverse events observed with LAG-3 inhibitors in treating melanoma and to identify populations most vulnerable to such side effects.
PubMed: 38047262
DOI: 10.1002/ski2.296 -
JMIR Dermatology Nov 2023Atopic dermatitis (AD), also known as eczema, is a chronic inflammatory skin condition that presents with symptoms of intense pruritus, dryness, and erythema.... (Review)
Review
BACKGROUND
Atopic dermatitis (AD), also known as eczema, is a chronic inflammatory skin condition that presents with symptoms of intense pruritus, dryness, and erythema. Dissatisfaction with first-line therapies for AD, the desire to avoid steroids, and the extreme cost of effective biologics have created a demand for alternative treatment options such as oral vitamins and nutritional supplements.
OBJECTIVE
The purpose of this review was to assess the effectiveness of oral nutritional supplements, pre- and probiotics, and vitamin deficiencies and supplements on AD symptomology and clinical course.
METHODS
We searched Scopus, PubMed, and MEDLINE (Ovid interface) for English-language articles published between 1993 and 2023. The final search was conducted on June 22, 2023. The search terms comprised the following: "(Atopic Dermatitis or Atopic Eczema) AND (supplement OR vitamin OR mineral OR micronutrients OR Fish Oil OR Omega Fatty Acid OR Probiotics OR Prebiotics OR apple cider vinegar OR collagen OR herbal OR fiber)."
RESULTS
A total of 18 studies-3 (17%) evaluating vitamins, 4 (22%) evaluating herbal medicine compounds, 2 (11%) evaluating single-ingredient nutritional supplements, and 9 (50%) evaluating pre- and probiotics-involving 881 patients were included in this review.
CONCLUSIONS
Overall, there is weak evidence to support any one nutritional supplement intervention for the alleviation of AD symptoms. Multiple trials (4/18, 22%) showed promise for supplements such as Zemaphyte, kefir, and freeze-dried whey with Cuscuta campestris Yuncker extract. The most evidence was found on the effectiveness of probiotics on the clinical course of AD. Lactiplantibacillus plantarum, Ligilactobacillus salivarius, and Lactobacillus acidophilus specifically showed evidence of efficacy and safety across multiple studies (6/18, 33%). However, larger, more extensive randomized controlled trials are needed to determine the true effectiveness of these supplements on the broader population.
TRIAL REGISTRATION
PROSPERO CRD42023470596; https://tinyurl.com/4a9477u7.
PubMed: 38019566
DOI: 10.2196/40857 -
International Wound Journal Nov 2023This meta-analysis aims to evaluate and compare the effect of surgical excision followed by adjuvant radiotherapy and laser combined with steroids on keloids. Relevant...
This meta-analysis aims to evaluate and compare the effect of surgical excision followed by adjuvant radiotherapy and laser combined with steroids on keloids. Relevant studies reporting the recurrence rate or incidence of adverse events (AEs) were retrieved from the PubMed, Web of Science, Embase and Cochrane Library databases through August 2023. The quality of noncomparative single-arm clinical trials was evaluated using the methodological index for nonrandomised studies (MINORS) Methodological items. This meta-analysis was conducted utilizing Stata 12.0 statistical software. 26 studies involving 989 patients were included in the analysis. The recurrence rate in the laser combined with steroids therapy group (12.2%, 95% confidence interval [CI]: 5.9%-18.5%) was lower than that of the surgical excision combined with radiotherapy group (13.5%, 95% CI: 6.6%-22.2%). For the incidence of AEs, relatively low incidence of atrophy (0.0%, 95% CI: 0.0%-1.2%), telangiectasia (3.2%, 95% CI: 0.4%-7.6%), erythema (2.3%, 95% CI: 0.0%-10.6%), infection (0.2%, 95% CI: 0.0%-1.6%) and high hyperpigmentation rate (8.3%, 95% CI: 4.2%-13.4%) were obtained in the surgical excision combined with radiotherapy group. Compared with surgical resection followed by radiotherapy, the combination of laser and steroids for keloids showed a lower hyperpigmentation rate (6.5%), as well as a higher incidence of atrophy (22.7%), telangiectasia (6.4%), erythema (3.3%) and infection (3.3%). Only a hypopigmentation rate of 2.9% was obtained in patients treated with surgical excision plus radiotherapy. Current evidence revealed that surgical excision followed by adjuvant radiotherapy and laser combined with steroids therapy were effective and safe treatments for keloids, with relatively low recurrence rate and complication rate. Comparative studies are needed to further compare the effects of these two combination therapies on keloids.
PubMed: 37967571
DOI: 10.1111/iwj.14449 -
Journal of the American Academy of... Feb 2024
Topics: Humans; Proto-Oncogene Proteins B-raf; Melanoma; Protein Kinase Inhibitors; Panniculitis; Antineoplastic Combined Chemotherapy Protocols; Mutation; Skin Neoplasms; Pyridones; Oximes
PubMed: 37943239
DOI: 10.1016/j.jaad.2023.10.008 -
Breast Care (Basel, Switzerland) Aug 2023Fibroadenomas are solid, mobile, and non-tender benign breast lumps, with highest prevalence amongst young women aged between 15 and 35. Symptoms can include discomfort,... (Review)
Review
INTRODUCTION
Fibroadenomas are solid, mobile, and non-tender benign breast lumps, with highest prevalence amongst young women aged between 15 and 35. Symptoms can include discomfort, and they can become problematic, particularly when they enlarge, resulting in many referrals for biopsies, with fibroadenomas accounting for 30-75% of the cases. Diagnosis is based on triple assessment that involves a clinical examination, ultrasound imaging, and mammography, as well as core needle biopsies. Current management includes observation for 6-12 months, with the indication of definitive surgery, in cases that are older than 35 years or with fibroadenoma persistence. Serious adverse effects of surgery might include nipple areolar distortion, scarring, and damage to the breast tissue, as well as the risks associated with surgery and anesthesia, making it a non-feasible option.
METHODS
A literature search was performed on the databases Embase, MEDLINE/PubMed, Google Scholar, and Ovid for English language papers published between January 1, 2000, and March 17, 2021. A structured protocol was employed to devise a comprehensive search strategy with keywords and Boolean operators defined by the research question. The keywords used for the search were "HIFU", "High Intensity Focused Ultrasound," "Fibroadenoma," "Breast," "Lesion." This review was carried out in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
Recently, a thermal ablative technique, high intensity focused ultrasound (HIFU), was found to be a safe, noninvasive, and technically successful alternative, having displayed promising outcomes in reducing the volume of fibroadenomas, pain experienced by patients, and the length of hospitalization. Quality of life improvement was also evidenced, exhibited by the disappearance of symptoms, and enhanced physical activity post-intervention, in addition to patients' satisfaction with the cosmetic results and future recommendation of the procedure to other patients.
CONCLUSION
Overall, HIFU is a well-tolerated treatment associated, with low risk of complications, that can potentially include erythema, skin discoloration, and bruising with the majority of these self-resolving shortly after the procedure.
PubMed: 37900548
DOI: 10.1159/000524738