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Pediatric Investigation Sep 2023Generalized pustular psoriasis (GPP) is a severe subtype of psoriasis, commonly combined with systemic inflammation. Gene mutations have been found to be associated with... (Review)
Review
Generalized pustular psoriasis (GPP) is a severe subtype of psoriasis, commonly combined with systemic inflammation. Gene mutations have been found to be associated with GPP and vary by ethnicity. Systemic treatments are usually required for the severity and potential complications of GPP. However, there is no common consensus in China, especially among pediatric patients, whose data are scarce. Acitretin, methotrexate, and cyclosporine are widely used in pediatrics with GPP, while the adverse effects should be highlighted. The emergence of different biological agents brings us into a new era. This article discusses the genetic background of Chinese patients and demonstrates the evidence of treatment in pediatrics with GPP.
PubMed: 37736368
DOI: 10.1002/ped4.12395 -
Vaccines Jul 2023From the beginning of public vaccinations until the relaxation of COVID-19 measures, many case reports, case series and case-control studies have been published... (Review)
Review
From the beginning of public vaccinations until the relaxation of COVID-19 measures, many case reports, case series and case-control studies have been published indicating cutaneous side effects of COVID-19 vaccination. Post-vaccination pustular eruption was reported as well, with a challenging differential diagnosis between pustular psoriasis, AGEP (acute generalized exanthematous pustulosis) and neutrophil pustular eruptions. We report a case of 56-year-old woman presented with acute generalized pustular flare up culminated 5 days after the second dose of BNT162b2(Pfizer) vaccination. She was diagnosed with pustular psoriasis flare and due to the regulating role of IL-1 in pustular psoriasis and in the cytokine storm observed in cases of COVID-19 postvaccination inflammation; we decided to treat the patient with an IL-1 antagonist, subcutaneous anakinra (100 mg daily) along with acitretin. One week later, after anakinra withdrawal, she presented a pustular psoriasis flare and a 7-day anakinra re-administration led to a satisfactory improvement in the skin lesions. We also reviewed the medical literature and found 28 case reports with pustular eruption after the COVID-19 vaccination. We compared the patients reported, regarding sex, age, number of doses, post-vaccination period and vaccine brand, and compared those results with our patient. Finally, as indicated by our case and other cases with similarly treated pustular eruptions. targeted therapy to this cytokine imbalance such as anakinra (IL-1) antagonist can improve the clinical course of the patient.
PubMed: 37631866
DOI: 10.3390/vaccines11081298 -
The Journal of Dermatological Treatment Dec 2024Generalized pustular psoriasis (GPP) is a rare auto-inflammatory disease. Patients with GPP may develop life-threatening complications, including sepsis, acute renal...
Generalized pustular psoriasis (GPP) is a rare auto-inflammatory disease. Patients with GPP may develop life-threatening complications, including sepsis, acute renal failure, neutrophilic cholangitis, high-output congestive heart failure, acute respiratory distress syndrome and death. The therapy of GPP is very limited and the course of the disease is unpredictable. We report a 60-year-old woman presenting with widespread and confluent erythematous-desquamative plaques with numerous small pustules covering almost 70% of the body surface area. Over the past years patient had undergone different types of conservative treatment regimens including topical therapy, acitretin, cyclosporin, methotrexate and long-term treatment with systemic corticosteroids. Considering the patient's overall clinical condition, we proceed to initiate the biologic therapy with guselkumab. Guselkumab (anti-IL-23) in the standard dose of 100 mg was administered subcutaneously at weeks 0, 4 and followed by a maintenance dose every 8 weeks. The remission of GPP was observed already after 12 weeks of treatment. The maintenance treatment in the period of 18 months shows stable clinical response. Our results support the evidence that guselkumab could provide an effective therapeutic approach in the treatment of GPP.
Topics: Female; Humans; Middle Aged; Psoriasis; Antibodies, Monoclonal, Humanized; Acitretin; Methotrexate; Chronic Disease; Acute Disease
PubMed: 38522861
DOI: 10.1080/09546634.2024.2331807 -
Dermatology and Therapy May 2024Hidradenitis suppurativa (HS) is a debilitating chronic skin disorder characterized by painful inflammatory nodules, abscesses and sinus tracts involving intertriginous... (Review)
Review
Hidradenitis suppurativa (HS) is a debilitating chronic skin disorder characterized by painful inflammatory nodules, abscesses and sinus tracts involving intertriginous areas and has an adverse impact on patient quality of life. Over the past decade, the therapeutic options of HS have increased significantly to comprise multiple modalities, including topical medication, systemic therapies (mainly antibiotics, retinoids, and biologics), surgical approaches, and lifestyle modifications. Biologics alone or in combination with surgery remain the treatment of choice for moderate to severe disease. However, non-biologic therapies (including retinoids) may be used as monotherapy for mild disease and in combination with biologics and surgical treatment in moderate to severe disease. Retinoids, specifically isotretinoin, acitretin, and alitretinoin, are historically used in the management of HS, supported by anecdotal evidence and with variable treatment response. Although the current American and European guidelines offer different recommendations on the use of retinoids in HS, retinoids remain a valuable ally in HS management. This review provides a comprehensive analysis of the current scientific literature on retinoid therapy (topical and systemic) in HS, highlighting disparities in mechanisms of action, efficacy, and safety to clarify their role in HS treatment.
PubMed: 38700645
DOI: 10.1007/s13555-024-01169-1 -
Frontiers in Immunology 2024Fatty liver disease (FLD) is a common comorbidity of psoriasis and is often referred to as non-alcoholic fatty liver disease (NAFLD). However, the role of inflammation...
BACKGROUND
Fatty liver disease (FLD) is a common comorbidity of psoriasis and is often referred to as non-alcoholic fatty liver disease (NAFLD). However, the role of inflammation or insulin resistance (IR) in FLD is inconclusive. The study aims to explore whether FLD in psoriasis patients is more related to insulin resistance or systemic inflammation level.
METHODS
Data for this study were collected from the Shanghai Psoriasis Effectiveness Evaluation Cohort, a prospective cohort that examines psoriasis characteristics in the Chinese population. IR was assessed using the triglyceride glucose (TyG) and TyG-body mass index (TyG-BMI) indicators. Systemic non-specific inflammation was assessed using the neutrophil-to-lymphocyte ratio (NLR), derived neutrophil-to-lymphocyte ratio (dNLR), and systemic immune inflammation index (SII).
RESULTS
The analysis included a total of 647 patients. Subsequent logistic regression analysis revealed that NLR, dNLR, and SII were not significantly associated with FLD in psoriasis patients, while TyG and TyG-BMI showed significant associations with FLD. Subgroup analysis indicated that in the majority of subgroups, TyG and TyG-BMI were significantly associated with FLD, particularly TyG-BMI. Excluding individuals with methotrexate and acitretin resulted in consistent findings with the main analysis. Further analysis revealed a significantly higher diagnosis rate of metabolic-associated fatty liver disease (MAFLD) compared to NAFLD.
CONCLUSIONS
Metabolic factors play a crucial role in FLD in patients with psoriasis, and TyG and TyG-BMI are potential predictors of FLD. Therefore, MAFLD can be recommend as a term to describe FLD in psoriasis patients.
TRIAL REGISTRATION
https://www.chictr.org.cn/showproj.html?proj=58256, identifier ChiCTR2000036186. A multi-center clinical study of systemic treatment strategies for psoriasis in Chinese population. Registered 31 August 2020.
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; China; Cross-Sectional Studies; Insulin Resistance; Neutrophils; Non-alcoholic Fatty Liver Disease; Prospective Studies; Psoriasis
PubMed: 38715604
DOI: 10.3389/fimmu.2024.1388967 -
Indian Journal of Dermatology 2023Generalised pustular psoriasis(GPP) is a rare, potentially life-threatening variant of psoriasis with acute onset, widely spread pustular lesions on an inflamed base...
Generalised pustular psoriasis(GPP) is a rare, potentially life-threatening variant of psoriasis with acute onset, widely spread pustular lesions on an inflamed base associated with a systemic inflammatory state. In the setting of the current COVID-19 pandemic, flares in pustular psoriasis have been reported, however these flares leading to mortality in SARS-CoV-2 infected patients is hitherto unreported. We present two cases of GPP flare following SARS-CoV-2 infection with fatal outcome. A 20 year old male, known case of GPP since 5 years presented with an exacerbation of his existing disease for 2 months. He had received methotrexate, cyclosporine, acitretin, apremilast, infliximab and secukinumab in the past. He was admitted and started on inj. methotrexate (subcutaneous) and cap. acitretin. During admission, he developed COVID-19 associated severe acute respiratory distress syndrome(ARDS). Despite timely intervention with life-saving measures, the patient could not be saved. The second case was a 52-year-old female, a case of GPP on treatment for the last 10 years, being maintained on cap. acitretin and cyclosporine. She also developed ARDS due to COVID infection. Despite being on acitretin for GPP and the appropriate management of severe COVID infection, her condition worsened and she expired within one day of admission. Both of our patients were not vaccinated against COVID-19. Awareness of potential risk of mortality in patients of GPP getting co-infected by COVID-19 is thus essential for dermatologists.
PubMed: 38371580
DOI: 10.4103/ijd.ijd_914_22 -
Frontiers in Pharmacology 2023Psoriasis vulgaris (PV) is a longstanding, inflammatory, immune-responsive skin condition. Chinese herbal medicine injections (CHMIs) have been utilized for treating PV...
Psoriasis vulgaris (PV) is a longstanding, inflammatory, immune-responsive skin condition. Chinese herbal medicine injections (CHMIs) have been utilized for treating PV in Asian countries. This study aims to conduct a thorough systematic review and meta-analysis to comprehensively appraise the efficacy of CHMIs in treating PV. Seven databases were searched for randomized controlled trials that evaluated the effect of CHMIs in treating PV, ranging from 2004 to June 2022. The meta-analysis was undertaken based on outcome measures, treatment options, and treatment durations using Review Manager 5.4. The primary outcome measure of this study was a 60% or higher reduction in the Psoriasis Area and Severity Index score (PASI 60). A descriptive analysis was performed for the assessment of adverse events. This systematic review incorporated 33 studies, comprising 3,059 participants. The main findings indicated significant differences based on the PASI 60 (RR = 1.30, 95% CI: 1.24 to 1.37, Z = 10.72, < 0.00001), PASI 30 (RR = 1.25, 95% CI: 1.13 to 1.38, Z = 4.48, < 0.00001), and PASI 20 (RR = 1.28, 95% CI: 1.13 to 1.45, Z = 3.82, = 0.0001) outcome measures. Evaluating the treatment options, CHMIs in combination with monotherapies like narrowband ultraviolet B (NB-UVB) and the acitretin capsule (AC) showed a greater reduction in PASI 60 (RR = 1.33, 95% CI: 1.25 to 1.43, Z = 8.32, < 0.00001). In terms of treatment duration, no significant difference was observed when the duration extended beyond 56 days. Furthermore, the results suggested that CHMIs might reduce the incidence of adverse events in the treatment of PV. This systematic review revealed preliminary clinical evidence supporting the use of CHMIs for treating PV, considering outcome measures, treatment options, and treatment durations. However, due to the low methodological quality and limited sample size of the included studies, there is an urgent need for high-quality, multi-center and larger-scale studies of CHMIs for PV to provide robust evidence for their clinical application. : [https://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=326531], identifier [CRD42022326531].
PubMed: 37465524
DOI: 10.3389/fphar.2023.1148445 -
Dermatology (Basel, Switzerland) 2024Acitretin, a synthetic vitamin A derivative, is the most studied and widely used oral retinoid for ichthyoses. Its major disadvantage is the need for contraceptive... (Review)
Review
BACKGROUND
Acitretin, a synthetic vitamin A derivative, is the most studied and widely used oral retinoid for ichthyoses. Its major disadvantage is the need for contraceptive measures during 3 years after discontinuation. An alternative is needed for women of childbearing age. With alitretinoin, another retinoid, pregnancy is considered safe 1 month after discontinuation.
OBJECTIVES
The aim of this study was to provide evidence for alitretinoin as an alternative for acitretin for ichthyosis in women of childbearing age. Our experience is shared in a case series combined with an overview of the current literature.
METHODS
Nine women of childbearing age (19-31 years, median 21) with different subtypes of ichthyosis (autosomal recessive congenital ichthyosis, (superficial) epidermolytic ichthyosis, erythrokeratoderma variabilis, and epidermolytic epidermal nevi, a mosaic form of epidermolytic ichthyosis) were included and treated with 30 mg alitretinoin during 2-28 months. Severity was measured by Ichthyosis Area Severity Index (IASI) and Investigator Global Assessment (IGA). A literature search in Pubmed using the Mesh terms "alitretinoin," "skin diseases, genetic" and "ichthyosis" was performed.
RESULTS
Significant reduction in the mean scores of IGA, IASI-erythema, IASI-scaling, and IASI-total was seen. Seven patients are still being treated, 1 patient stopped to become pregnant, 1 patient discontinued due to financial reasons. Observed side effects were reversible headache (n = 6), asteatotic eczema (n = 1), "not feeling well" temporarily (n = 1), and easier blistering of the feet (n = 1). The literature search resulted in six case reports and case series about alitretinoin in ichthyosis and ichthyosis syndromes with in total 29 patients. The vast majority of articles (21/29) reported significant improvement or even complete remission of skin symptoms. However, validated outcome measures to support these results were lacking. Side effects (n = 16) were relatively mild, except for benign intracranial hypertension (n = 1) and autoimmune hypothyroidism (n = 1).
CONCLUSION
Our study shows, with validated outcome measures, that alitretinoin is effective to mitigate the symptoms of ichthyosis in women of childbearing age and a suitable alternative to acitretin.
Topics: Pregnancy; Humans; Female; Young Adult; Adult; Alitretinoin; Acitretin; Hyperkeratosis, Epidermolytic; Ichthyosis; Immunoglobulin A
PubMed: 37666225
DOI: 10.1159/000533934 -
Frontiers in Pharmacology 2023In March 2018, the European pregnancy prevention programme for oral retinoids was updated as part of risk minimisation measures (RMM), emphasising their...
Impact of the 2018 revised Pregnancy Prevention Programme by the European Medicines Agency on the use of oral retinoids in females of childbearing age in Denmark, Italy, Netherlands, and Spain: an interrupted time series analysis.
In March 2018, the European pregnancy prevention programme for oral retinoids was updated as part of risk minimisation measures (RMM), emphasising their contraindication in pregnant women. To measure the impact of the 2018 revision of the RMMs in Europe by assessing the utilisation patterns of isotretinoin, alitretinoin and acitretin, contraceptive measures, pregnancy testing, discontinuation, and pregnancy occurrence concomitantly with a retinoid prescription. An interrupted time series (ITS) analysis to compare level and trend changes after the risk minimisation measures implementation was conducted on a cohort of females of childbearing age (12-55 years of age) from January 2010 to December 2020, derived from six electronic health data sources in four countries: Denmark, Netherlands, Spain, and Italy. Monthly utilisation figures (incidence rates [IR], prevalence rates [PR] and proportions) of oral retinoids were calculated, as well as discontinuation rates, contraception coverage, pregnancy testing, and rates of exposed pregnancies to oral retinoids, before and after the 2018 RMMs. From 10,714,182 females of child-bearing age, 88,992 used an oral retinoid at any point during the study period (mean age 18.9-22.2 years old). We found non-significant level and trend changes in incidence or prevalence of retinoid use in females of child-bearing age after the 2018 RMMs. The reason of discontinuation was unknown in >95% of cases. Contraception use showed a significant increase trend in Spain; for other databases this information was limited. Pregnancy testing was hardly recorded thus was not possible to model ITS analyses. After the 2018 RMM, rates of pregnancy occurrence during retinoid use, and start of a retinoid during a pregnancy varied from 0.0 to 0.4, and from 0.2 to 0.8, respectively. This study shows a limited impact of the 2018 RMMs on oral retinoids utilisation patterns among females of child-bearing age in four European countries. Pregnancies still occur during retinoid use, and oral retinoids are still prescribed to pregnant women. Contraception and pregnancy testing information was limited in most databases. Regulators, policymakers, prescribers, and researchers must rethink implementation strategies to avoid any pregnancy becoming temporarily related to retinoid use.
PubMed: 37663263
DOI: 10.3389/fphar.2023.1207976 -
Acta Dermatovenerologica Alpina,... Dec 2023Darier disease is a rare autosomal dominant genodermatosis that initially first presents in adolescence with scaly reddish brown keratotic papules and plaques with a...
Darier disease is a rare autosomal dominant genodermatosis that initially first presents in adolescence with scaly reddish brown keratotic papules and plaques with a seborrheic and intertriginous distribution. The absence of specific targeted medications complicates the treatment process, and managing resistant cases can prove challenging due to recurrent exacerbations that may result in serious complications such as secondary bacterial and viral infections. Treatments of choice include antiseptics, topical corticosteroids, and systemic retinoids, mainly acitretin and isotretinoin. We report the case of a female patient with Darier disease that was unsuccessfully treated with acitretin and isotretinoin but showed significant improvement with alitretinoin. Previous reports on the efficacy of alitretinoin in Darier disease are reviewed.
Topics: Adolescent; Humans; Female; Darier Disease; Alitretinoin; Acitretin; Isotretinoin; Dermatologic Agents
PubMed: 38126103
DOI: No ID Found