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Blood Transfusion = Trasfusione Del... Sep 2022Acquired platelet function disorders (PFD) are rare bleeding diseases that should be suspected in all patients with unexplained mucocutaneous bleedings of recent onset,... (Review)
Review
Acquired platelet function disorders (PFD) are rare bleeding diseases that should be suspected in all patients with unexplained mucocutaneous bleedings of recent onset, with no previous history of haemorrhages, and with normal coagulation test and platelet count. Drug-induced platelet function bleeding disorders are the most frequent PFDs and can easily be identified on the basis of recent administration of platelet-inhibiting drugs. Apart from these, the most challenging acquired PFDs are those caused by autoimmune mechanisms. In fact, demonstration of autoantibodies inhibiting platelet function may be difficult in most non-specialised centres. Among autoimmune PFDs (aPFDs), acquired Glanzmann thrombasthenia (aGT), which is caused by autoantibodies that bind to platelet αIIbβ3 integrin, inhibiting its function, is the most frequent. aGT can be associated with underlying haematological malignancies or autoimmune diseases but can also be idiopathic. More rarely, other immune-mediated PFDs can occur, such as acquired delta storage pool disease (aδSPD). Treatment of aPFDs must rely on the control of acute and chronic bleedings, treatment of the underlying disease in secondary forms, and immunosuppressive treatment for autoantibody reduction or eradication. aPFDs may completely resolve upon treatment of any underlying disease that may be present. In primary aPFDs, and in the majority of secondary forms, treatment relies on immunosuppressive therapies.Here we present a systematic review of previously described immune-mediated aGT and aδSPD cases. Clinical and laboratory characteristics, treatments for the control of bleedings and for the eradication of autoantibodies, and responses to treatments are also discussed. Although no guidelines are available for the management of these very rare conditions, presentation of all cases reported so far can help clinicians in the diagnosis and treatment of these life-threatening diseases.
Topics: Albinism; Autoantibodies; Autoimmune Diseases; Hemorrhagic Disorders; Hermanski-Pudlak Syndrome; Humans; Platelet Glycoprotein GPIIb-IIIa Complex; Thrombasthenia
PubMed: 34369869
DOI: 10.2450/2021.0119-21 -
PloS One 2021Checkpoint inhibitors have revolutionized advanced melanoma care; however, their cutaneous side effects have not been definitively elucidated. (Meta-Analysis)
Meta-Analysis
BACKGROUND
Checkpoint inhibitors have revolutionized advanced melanoma care; however, their cutaneous side effects have not been definitively elucidated.
OBJECTIVE
To identify the prevalence of cutaneous toxicity in patients with melanoma treated with immune checkpoint inhibitors as monotherapy and/or in combination with chemotherapy and/or radiotherapy.
MATERIALS AND METHODS
We performed a systematic review and meta-analysis, which encompassed both clinical trials and observational studies describing the dermatological toxicities in patients treated with immune checkpoint inhibitors. The protocol was registered in the International Prospective Register of Systematic Review under the number CRD42018091915. The searches were performed using the CINAHL, Cochrane CENTRAL, LILACS, LIVIVO, PubMed, Scopus, and Web of Science databases. The methodological quality of the studies was evaluated with the JBI Critical Appraisal Checklist for Studies Reporting Prevalence Data.
RESULTS
A total of 9,802 articles were identified in the databases. The final sample comprised 39 studies. The evaluated drugs were ipilimumab, tremelimumab, pembrolizumab, and nivolumab. The results suggest that the most prevalent side effect was grade 1 and 2 pruritus (24%), followed by grade 1 and 2 rash (21%) and grade 1 and 2 vitiligo (10%).
CONCLUSION
The most prevalent side effects in patients treated with checkpoint inhibitors are pruritus, rash, and vitiligo, and they are rated mostly as grades 1 and 2 adverse events. Remarkably, vitiligo is most commonly found in patients treated with PD-1 inhibitors.
Topics: Antibodies, Monoclonal, Humanized; Clinical Trials as Topic; Exanthema; Female; Humans; Immune Checkpoint Inhibitors; Immunotherapy; Ipilimumab; Male; Melanoma; Middle Aged; Nivolumab; Observational Studies as Topic; Prevalence; Pruritus; Skin Neoplasms; Vitiligo
PubMed: 34358260
DOI: 10.1371/journal.pone.0255716 -
JAMA Dermatology Sep 2021Dermoscopy increases the diagnostic accuracy for melanoma. However, the accuracy of individual structures and patterns used in melanoma detection has not been... (Meta-Analysis)
Meta-Analysis
IMPORTANCE
Dermoscopy increases the diagnostic accuracy for melanoma. However, the accuracy of individual structures and patterns used in melanoma detection has not been systematically evaluated.
OBJECTIVE
To assess the diagnostic accuracy of individual dermoscopic structures and patterns used in melanoma detection.
DATA SOURCES
A search of Ovid Medline, Embase, Cochrane CENTRAL, Scopus, and Web of Science was conducted from inception to July 2020.
STUDY SELECTION
Studies evaluating the dermoscopic structures and patterns among melanomas in comparison with nonmelanoma lesions were included. Excluded were studies with fewer than 3 patients, studies in languages other than English or Spanish, studies not reporting dermoscopic structures per lesion type, and studies assessing only nail, mucosal, acral, facial, or metastatic melanomas or melanomas on chronically sun-damaged skin. Multiple reviewers applied these criteria, and 0.7% of studies met selection criteria.
DATA EXTRACTION AND SYNTHESIS
The Preferred Reporting Items for Systematic Reviews and Meta-analyses reporting guideline and Meta-analysis of Observational Studies in Epidemiology reporting guideline were followed. Guidelines were applied via independent extraction by multiple observers. Data were pooled using a random-effects model.
MAIN OUTCOMES AND MEASURES
The prespecified outcome measures were diagnostic accuracy (sensitivity and specificity) and risk (odds ratio [OR]) of melanoma for the following dermoscopic structures/patterns: atypical dots/globules, atypical network, blue-white veil, negative network, off-centered blotch, peripheral-tan structureless areas, atypical vessels (eg, linear irregular, polymorphous), pseudopods, streaks, regression (ie, peppering, scarlike areas), shiny white structures, angulated lines, irregular pigmentation, and a multicomponent pattern.
RESULTS
A total of 40 studies including 22 796 skin lesions and 5736 melanomas were evaluated. The structures and patterns with the highest ORs were shiny white structures (OR, 6.7; 95% CI, 2.5-17.9), pseudopods (OR, 6.7; 95% CI, 2.7-16.1), irregular pigmentation (OR, 6.4; 95% CI, 2.0-20.5), blue-white veil (OR, 6.3; 95% CI, 3.7-10.7), and peppering (OR, 6.3; 95% CI, 2.4-16.1). The structures with the highest specificity were pseudopods (97.3%; 95% CI, 94.3%-98.7%), shiny white structures (93.6%; 95% CI, 85.6%-97.3%), peppering (93.4%; 95% CI, 81.9%-97.8%), and streaks (92.1%; 95% CI, 88.4%-94.7%), whereas features with the highest sensitivity were irregular pigmentation (62.3%; 95% CI, 31.2%-85.8%), blue-white veil (60.6%; 95% CI, 46.7%-72.9%), atypical network (56.8%; 95% CI, 43.6%-69.2%), and a multicomponent pattern (53.7%; 95% CI, 40.4%-66.4%).
CONCLUSIONS AND RELEVANCE
The findings of this systematic review and meta-analysis support the diagnostic importance of dermoscopic structures associated with melanoma detection (eg, shiny white structures, blue-white veil), further corroborate the importance of the overall pattern, and may suggest a hierarchy in the significance of these structures and patterns.
Topics: Dermoscopy; Humans; Melanoma; Pigmentation Disorders; Retrospective Studies; Skin Diseases; Skin Neoplasms
PubMed: 34347005
DOI: 10.1001/jamadermatol.2021.2845 -
Frontiers in Endocrinology 2021Proopiomelanocortin (POMC) deficiency is an extremely rare inherited autosomal recessive disorder characterized by severe obesity, adrenal insufficiency, skin...
Proopiomelanocortin (POMC) deficiency is an extremely rare inherited autosomal recessive disorder characterized by severe obesity, adrenal insufficiency, skin hypopigmentation, and red hair. It is caused by pathogenic variants in the gene that codes the proopiomelanocortin polypeptide which is cleaved to several peptides; the most notable ones are adrenocorticotropic hormone (ACTH), alpha- and beta-melanocyte-stimulating hormones (-MSH and -MSH); the latter two are crucial in melanogenesis and the energy balance by regulating feeding behavior and energy homeostasis through melanocortin receptor 4 (MC4R). The lack of its regulation leads to polyphagia and early onset severe obesity. A novel MC4R agonist, setmelanotide, has shown promising results regarding weight loss in patients with POMC deficiency. A systematic review on previously published clinical and genetic characteristics of patients with POMC deficiency and additional data obtained from two unrelated patients in our care was performed. A 25-year-old male patient, partly previously reported, was remarkable for childhood developed type 1 diabetes (T1D), transient growth hormone deficiency, and delayed puberty. The second case is a girl with an unusual presentation with central hypothyroidism and normal pigmentation of skin and hair. Of all evaluated cases, only 50% of patients had characteristic red hair, fair skin, and eye phenotype. Central hypothyroidism was reported in 36% of patients; furthermore, scarce adolescent data indicate possible growth axis dysbalance and central hypogonadism. T1D was unexpectedly prevalent in POMC deficiency, reported in 14% of patients, which could be an underestimation. POMC deficiency reveals to be a syndrome with several endocrinological abnormalities, some of which may become apparent with time. Apart from timely diagnosis, careful clinical follow-up of patients through childhood and adolescence for possible additional disease manifestations is warranted.
Topics: Adrenal Insufficiency; Adult; Child, Preschool; Diabetes Mellitus, Type 1; Female; Humans; Male; Obesity; Pro-Opiomelanocortin; Skin Pigmentation
PubMed: 34177811
DOI: 10.3389/fendo.2021.689387 -
BioMed Research International 2021Vitiligo is a disfiguring skin disease with profound psychosocial impacts, such as anxiety, but the reported effect sizes of associations vary. We aimed to conduct a... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Vitiligo is a disfiguring skin disease with profound psychosocial impacts, such as anxiety, but the reported effect sizes of associations vary. We aimed to conduct a meta-analysis to quantify the strength of association between anxiety and vitiligo and to estimate the prevalence of anxiety among individuals with vitiligo.
METHODS
A systematic literature search was performed in five online databases (MEDLINE, Embase, Web of Science, Cochrane Library, and PsycINFO) from inception until March 20, 2020. All of the eligible studies were comprehensively reviewed, and all of the available data were analyzed according to our predefined criteria.
RESULTS
Twenty-one studies involving 3259 patients in 11 countries were included in this meta-analysis. Compared with the healthy control group, patients with vitiligo often had concomitant anxiety (OR = 6.14 [95% CI: 3.35-11.24], = 30.1%). The pooled prevalence of anxiety in female patients was significantly higher than that in males (OR = 2.24 [95% CI: 1.31-3.84], = 0.0%). Subgroup analysis showed that the pooled prevalence of clinical anxiety disorder and anxiety symptoms was 12% (95% CI: 7%-16%, = 76.3%) and 34% (95% CI: 21%-46%, = 94.7%), respectively. No publication bias has been detected by Begg's funnel plot and Egger's test.
CONCLUSION
Patients with vitiligo have high anxiety comorbidity, with female predominance. Dermatologists and psychiatrists should be vigilant to the presence of anxiety, apply appropriate interventions to reduce the psychological impacts in a timely manner, and thus promote recovery in vitiligo patients. However, due to some objective limitations (poor information about the OR and diversity in assessment tools among included studies), findings should be interpreted with caution.
Topics: Anxiety; Anxiety Disorders; Comorbidity; Databases, Factual; Depression; Female; Humans; Male; Prevalence; Vitiligo
PubMed: 34055993
DOI: 10.1155/2021/6663646 -
Anais Brasileiros de Dermatologia 2021The serum Vitamin D status in patients with vitiligo is ambiguous when compared to controls. A systematic review and updated meta-analysis were conducted to evaluate the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The serum Vitamin D status in patients with vitiligo is ambiguous when compared to controls. A systematic review and updated meta-analysis were conducted to evaluate the association between Vitamin D and vitiligo.
METHODS
Relevant studies were identified by searching PubMed and other databases. The random effects model was used to obtain standardized mean differences and pooled correlation coefficients. Meta-regression and sub-group analyses were conducted to explore heterogeneity. The presence of publication bias and the study robustness were tested using funnel plot and sensitivity analyses, respectively.
RESULTS
This meta-analysis finally included 31 studies. Compared with controls, vitiligo patients showed significantly decreased serum Vitamin D levels (standardized mean difference = -1.03; p < 0.0001). The sub-group analysis showed that vitiligo patients with indoor/urban work had a significantly lower Vitamin D level when compared to their outdoor/rural counterparts (standardized mean differences = -0.45; p = 0.03). The sensitivity analysis indicated that no single study had a significant influence on the overall outcome, suggesting the robustness of this meta-analysis.
STUDY LIMITATIONS
Varied sample sizes and heterogeneous study populations from different countries are the limitations of this study. However, the between-study heterogeneity has been addressed by the random-effects model with meta-regression and sensitivity analyses.
CONCLUSIONS
This meta-analysis showed significantly decreased Vitamin D level in vitiligo, and its association with indoor/outdoor type of work of vitiligo patients. This study highlights the need to assess Vitamin D status for improving its level in vitiligo.
Topics: Humans; Sample Size; Vitamin D; Vitamin D Deficiency; Vitiligo
PubMed: 33863565
DOI: 10.1016/j.abd.2020.10.002 -
The Journal of Dermatological Treatment Jun 2022Melasma is a common chronic refractory disorder of pigmentation affecting people with darker skin types. Overall prevalence varies between 8.8% and 40%, depending on the... (Review)
Review
Melasma is a common chronic refractory disorder of pigmentation affecting people with darker skin types. Overall prevalence varies between 8.8% and 40%, depending on the ethnicity of the population and the geographical area. Therapeutic management of melasma is challenging, with high recurrence rates which significant impacts on the quality of life. No single treatment is universally efficacious. Systemic treatments with tranexamic acid and polypodium leucotmatous had promising results, although the former was related to systemic side effects. Microneedling and peeling were also efficacious, although their superiority to topical hydroquinone, the gold standard in melasma treatment, remains to be established. Similarly, laser and light devices have been beneficial. However, recurrence rates remain high in all treatment groups. Combination therapies, either in double or triple combinations yielded the best results when compared to single terapies. Treatment choice should be made after Wood's lamp examination, as well as dermatoscopic evaluation, in order to select the best treatment option, targeted at each melasma subtype.
Topics: Administration, Cutaneous; Humans; Melanosis; Quality of Life; Tranexamic Acid; Treatment Outcome
PubMed: 33849384
DOI: 10.1080/09546634.2021.1914313 -
Clinical, Cosmetic and Investigational... 2021Vitiligo is disfiguring and devastating condition that can humans feel stigmatic and devalued. Melasma is a general condition of hyperpigmentation particularly involving... (Review)
Review
INTRODUCTION
Vitiligo is disfiguring and devastating condition that can humans feel stigmatic and devalued. Melasma is a general condition of hyperpigmentation particularly involving the face. The pigmentation disorders of vitiligo (hypopigmentation or de-pigmentation) and melasma (Hypermelanosis) are common among the world's population (around 1% for vitiligo).
OBJECTIVE
The identification of medicinal plants used in the treatment of vitiligo and hypermelanosis. A systematic literature review on harms associated with the medicinal plants used in the treatment of vitiligo and hypermelanosis. To review and summarize information on reported adverse drug reactions (ADRs) associated with these medicinal plants contained in (where access is available) national and global individual case safety report databases.
METHODS
A systematic review of the literature with special reference to all types of clinical trial and case reports using biomedical databases including Medline, EMBASE, Scopus, International Pharmaceutical Abstracts and so forth to identify medicinal plants alone or as an adjuvant with other treatments and their safety/tolerability in the treatment of vitiligo and Hypermelanosis. Other sources of this search were medicinal plants text books, pharmacopoeias and authentic websites discussing possible treatments for vitiligo/hypermelanosis. It also included databases such as VigiAccess containing data from spontaneous reporting schemes for ADRs.
RESULTS
A total of 55 articles (47 clinical trials and 8 case reports) met the inclusion criteria. Some trials did not reported safety information, some did report, but not very well. Reports of blistering, erythema, acute hepatitis and mutagenesis with . Adverse effects of erythema (mild to severe), phototoxic reactions, mild raise in liver transaminases, gastrointestinal disturbances, burns, itching, scaling, depigmented macules, pruritis, and giddiness with the use of psoralens. Khellin-related erythema, perilesional hyperpigmentation, gastrointestinal disturbances, mild raise in liver transaminases and orthostatic complaints. Infrequent side effects with Ginkgo biloba. Lower grade of erythema and edema reported with the use of
CONCLUSION
Primarily the retrieved clinical studies were efficacy oriented and safety parameters were secondary in priority whilst the general protocol of clinical trials requires the screening of drugs/medicinal plants on the basis of safety studies before testing the clinical aspects of efficacy. Thereby it is recommended that efficacy studies may be followed once the safety has been established for a particular medicinal plant in treating vitiligo and hypermelanosis.
PubMed: 33790609
DOI: 10.2147/CCID.S298342 -
Indian Journal of Dermatology,... 2021Xeroderma pigmentosum is a rare hereditary autosomal recessive genodermatosis. At present, there are many treatment options for xeroderma pigmentosum, covering...
Xeroderma pigmentosum is a rare hereditary autosomal recessive genodermatosis. At present, there are many treatment options for xeroderma pigmentosum, covering medical/procedural, surgical and combined modalities. However, the quality of these interventions has not been assessed. Our study aimed to perform a systematic review of the literature regarding the treatment of xeroderma pigmentosum. Multiple medical databases were accessed with the Medical Subject Headings terms; "xeroderma pigmentosum," "therapeutics" and "surgical procedures, operative" from January 2000 to April 2019, including articles published in Portuguese, Spanish and English (PROSPERO-CRD42018114858). Two hundred and ninety-eight studies were found in the databases researched, of which, after applying the inclusion criteria, only 33 studies remained. The 33 complete articles were read by three of the authors, having been found: 16 reported medical/procedural and 17 reported surgical treatments. Only one clinical study presented a good level of evidence (EL: 2): a randomized clinical trial using a T4 endonuclease V (T4N5) liposome lotion which reduced the development of skin lesions in patients with xeroderma pigmentosum. Amongst surgical modalities, all studies presented low evidence level (EL: 4). Three illustrative cases are also presented, to emphasize the multiple number of times that surgical modalities may be required in these patients. The therapeutic modalities, both clinical and surgical, for xeroderma pigmentosum presented a low level of scientific evidence which did not allow meta-analysis. More therapeutic studies, both clinical and surgical, with better scientific evidence are needed.
Topics: Antineoplastic Agents; Dermatologic Surgical Procedures; Fluorouracil; Humans; Imiquimod; Ointments; Photochemotherapy; Skin Neoplasms; Xeroderma Pigmentosum
PubMed: 33769755
DOI: 10.25259/IJDVL_431_19 -
JAMA Dermatology Mar 2021Surgical interventions are a key part of the therapeutic arsenal, especially in refractory and stable vitiligo. Comparison of treatment outcomes between the different... (Comparative Study)
Comparative Study Meta-Analysis
IMPORTANCE
Surgical interventions are a key part of the therapeutic arsenal, especially in refractory and stable vitiligo. Comparison of treatment outcomes between the different surgical procedures and their respective adverse effects has not been adequately studied.
OBJECTIVE
To investigate the reported treatment response following different surgical modalities in patients with vitiligo.
DATA SOURCES
A comprehensive search of the MEDLINE, Embase, Web of Science, and Cochrane Library databases from the date of database inception to April 18, 2020, was conducted. The key search terms used were vitiligo, surgery, autologous, transplantation, punch, suction blister, and graft.
STUDY SELECTION
Of 1365 studies initially identified, the full texts of 358 articles were assessed for eligibility. A total of 117 studies were identified in which punch grafting (n = 19), thin skin grafting (n = 10), suction blister grafting (n = 29), noncultured epidermal cell suspension (n = 45), follicular cell suspension (n = 9), and cultured epidermal cell suspension (n = 17) were used.
DATA EXTRACTION AND SYNTHESIS
Three reviewers independently extracted data on study design, patients, intervention characteristics, and outcomes. Random effects meta-analyses using generic inverse-variance weighting were performed.
MAIN OUTCOMES AND MEASURES
The primary outcomes were the rates of greater than 90%, 75%, and 50% repigmentation response. These rates were calculated by dividing the number of participants in an individual study who showed the corresponding repigmentation by the total number of participants who completed the study. The secondary outcomes were the factors associated with treatment response to the surgical intervention.
RESULTS
Among the 117 unique studies and 8776 unique patients included in the analysis, rate of repigmentation of greater than 90% for surgical interventions was 52.69% (95% CI, 46.87%-58.50%) and 45.76% (95% CI, 30.67%-60.85%) for punch grafting, 72.08% (95% CI, 54.26%-89.89%) for thin skin grafting, 61.68% (95% CI, 47.44%-75.92%) for suction blister grafting, 47.51% (95% CI, 37.00%-58.03%) for noncultured epidermal cell suspension, 36.24% (95% CI, 18.92%-53.57%) for noncultured follicular cell suspension, and 56.82% (95% CI, 48.93%-64.71%) for cultured epidermal cell suspension. The rate of repigmentation of greater than 50% after any surgical intervention was 81.01% (95% CI, 78.18%-83.84%). In meta-regression analyses, the treatment response was associated with patient age (estimated slope, -1.1418), subtype of vitiligo (estimated slope, 0.3047), and anatomical sites (estimated slope, -0.4050).
CONCLUSIONS AND RELEVANCE
The findings of this systematic review and meta-analysis suggest that surgical intervention can be an effective option for refractory stable vitiligo. An appropriate procedure should be recommended based on patient age, site and size of the lesion, and costs.
Topics: Age Factors; Blister; Epidermal Cells; Humans; Skin Transplantation; Treatment Outcome; Vitiligo
PubMed: 33595599
DOI: 10.1001/jamadermatol.2020.5756