Lamellar ichthyosis with a novel NIPAL4 variant showing dramatic response to high-dose vitamin D therapy.

Therapeutic options are limited in cases of autosomal recessive congenital ichthyosis with inadequate response to topical agents. Acitretin is the current standard of care in these patients, but its use is limited by cumulative toxicity when prolonged therapy is needed in children. There is evidence to suggest that high doses of vitamin D can normalize keratinization and suppress inflammatory cytokines. Here, we report a patient with lamellar ichthyosis with a novel mutation in the Nipa-like Domain-Containing 4 (NIPAL4) gene. High dose short-term vitamin D therapy was administered with a dramatic and sustained clinical response.

Safety of current systemic therapies for nail psoriasis.

INTRODUCTION: A discussion of safety of systemic treatments for nail psoriasis is lacking, particularly in reference to approval of new therapies assessed for nail outcomes. A review of safety profiles for agents commonly utilized for treatment of nail psoriasis is warranted to help inform treatment choices. The PubMed database was searched on 5 April 20235 April 2023, with articles discussing safety of nail psoriasis systemic therapies identified and reviewed. AREAS COVERED: Systemic treatments for nail psoriasis include biologic therapies (tumor necrosis factor-alpha inhibitors, interleukin-17 inhibitors, interleukin-23 inhibitors, interleukin-12/23 inhibitors), small molecule inhibitors (apremilast, tofacitinib), and oral systemic immunomodulators (methotrexate, cyclosporine, acitretin), each with unique safety profiles and considerations. Herein, we discuss adverse events, contraindications, drug-drug interactions, screening/monitoring guidelines, as well as utilization for special populations, including pregnant, older, and pediatric patients. EXPERT OPINION: The advent of targeted therapies, including biologic treatments and small molecule inhibitors, has revolutionized outcomes for nail psoriasis patients, but warrant review and monitoring for potential adverse events. Oral systemic immunomodulators have demonstrated moderate efficacy for nail psoriasis treatment, but are notable for frequent contraindications and drug-drug interactions. Further study of these agents and their use in special populations is needed to elucidate safety profiles for long-term use.

An update on therapeutic options for palmoplantar pustulosis: a narrative review and expert recommendations.

INTRODUCTION: Palmoplantar pustulosis (PPP) is a chronic inflammatory skin disease belonging to the localized form of pustular psoriasis. It is characterized by sterile pustule formation in palms and soles and a recurrent disease course. Although we have many treatments for PPP, there is no authoritative guidance. AREAS COVERED: A thorough search of PubMed was conducted to identify studies in PPP from 1973 onwards, with additional references to specific articles. Any treatment methods were outcomes of interest, including topical treatment, systemic treatment, biologics, other targeted treatments, phototherapy, and tonsillectomy. EXPERT OPINION: Topical corticosteroids are suggested as first-line therapy. Oral acitretin has become the most applied systemic retinoid recommended in PPP without joint involvement. For patients with arthritis, immunosuppressants like cyclosporin A and methotrexate are more recommended. UVA1, NB-UVB, and 308-nm excimer laser are effective phototherapy options. The combinations of topical or systemic agents and phototherapy may enhance the efficacy, particularly in recalcitrant cases. Secukinumab, ustekinumab, and apremilast are the most investigated targeted therapies. However, heterogeneous reported outcomes in clinical trials provided low-to-moderate quality evidence of their efficacy. Future studies are required to address these evidence gaps. We suggest managing PPP based on the acute phase, maintenance phase, and comorbidities.

A Meta-analysis of Xiaoyin Granules Combined with Acitretin Capsule in the Treatment of Psoriasis Vulgaris.

Background: Psoriasis is a chronic noncommunicable dermatological condition, and psoriasis vulgaris is the most common phenotype. Acitretin is the most widely used systemic retinoid in the treatment of psoriasis. This review evaluates the clinical therapeutic effects of Xiaoyin granule, a Chinese herbal medicine, combined with acitretin capsule in the treatment of psoriasis vulgaris. Methods: Six databases including PubMed, Cochrane Library, EMBASE, China National Knowledge Infrastructure (CNKI), Wan Fang, and China Biology Medicine disc (CBM) were searched for published studies on Xiaoyin granule and/or acitretin capsule in psoriasis vulgaris. The Cochrane Collaboration risk-of-bias instrument was used to assess the quality of the included RCTs. STATA 14.0 was used to conduct the statistical analysis. Results: Twenty-eight trials with 3281 patients were included in this meta-analysis. The results of this study show that the combined treatment of Xiaoyin granule and acitretin capsule could improve the total effective rate (TER) and cure rate (CR) when compared with acitretin capsule (TER: RR = 1.15, 95% CI (1.10, 1.21); CR: RR = 1.8, 95% CI (1.62, 2.00)) or Xiaoyin granule (TER: RR = 1.24, 95% CI (1.11, 1.39); CR: RR = 1.75, 95% CI (1.54, 1.98)) alone. The combined therapy could decrease the PASI score (mean difference = -1.45, 95% CI (-2.09, -0.80)) and inhibit inflammation (IL-10: mean difference = 1.16, 95% CI (0.94, 1.38); IL-17: mean difference = -2.06, 95% CI (-2.60, -1.51)) in psoriasis vulgaris patients. Conclusions: The combination of Xiaoyin granule and acitretin capsules could be a novel therapeutic strategy in the treatment of psoriasis vulgaris. However, the quality of trials in this study limited the conclusion, and more high-quality RCTs are needed for further evaluation.

Efficacy and safety of external application of Chinese herbal medicine for psoriasis vulgaris: a systematic review of randomized controlled trials.

OBJECTIVE: To evaluate the clinical efficacy and safety of external application of Chinese herbal medicine (ex-CHM) for psoriasis vulgaris (PV). METHODS: Different search portals, including the China National Knowledge Infrastructure Database, China Science and Technology Journal Database, Wanfang, SinoMed, clinicaltrials, PubMed, Cochrane Library, and Cochrane Central Register of Controlled Trials, were searched for randomized controlled trials (RCTs) that compared the ex-CHM intervention with other treatment protocols for PV, with available data as of November 25, 2020. The Review Manager 5.3 software was used for analysis. RESULTS: Nineteen RCTs involving 1988 participants were included, of which twelve RCTs qualified for the Meta-analysis. The results showed that the addition of CHM bath to narrow-band ultraviolet B (NB-UVB) had a higher response rate [RR = 1.29; 95% CI (1.19, 1.40); P < 0.000 01]; lower psoriasis area severity index (PASI) [MD = -3.15; 95% CI (-4.79, -1.52); P = 0.000 2)], adverse reactions rate [RR = 0.32; 95% CI (0.15, 0.66); P = 0.002], and recurrence rate [RR = 0.48; 95% CI (0.30, 0.79); P=0.004] than NB-UVB alone. The addition of CHM fumigation to NB-UVB also showed a higher response rate [RR = 1.29; 95% CI (1.11, 1.49); P = 0.000 7] and lower adverse reactions rate [RR = 0.44; 95% CI (0.24, 0.79); P=0.006]. In addition, CHM bath could reduce the adverse reactions induced by 308 nm excimer laser and improve patients' quality of life better than phototherapy. CHM fumigation could improve the efficacy of calcipotriol ointment and reduce the adverse reactions. CHM fumigation combined with external washing plus acitretin showed better results than using acitretin alone. No statistical difference was observed between CHM external washing and calcipotriol ointment or CHM ointment and retinoic acids. CONCLUSIONS: Current evidence showed that CHM bath and fumigation appeared to be efficient and safe for PV treatment. However, no definite conclusion could be drawn due to the low quality of included studies and thus more well-designed studies are needed for further assessment.

Anti-IL23for nail psoriasis in real life: Results of efficacy and safety during a 52-week period.

Nail psoriasis (NP) is often considered disfiguring for patients with a relevant impact on quality of life (QoL). It is also difficult to treat for dermatologists who are often frustrated by the scarcity of effective therapeutic alternatives in this particular location. Topical therapies are often used as the first-line treatment for mild NP, but efficacy is the modest. Conventional disease-modifying antirheumatic drugs (cDMARDs) (e.g., cyclosporine, methotrexate, acitretin, and dimethyl fumarate) are generally avoided in NP without general cutaneous involvement. Biologics represent, to date, a concrete possibility for the management of these patients. The data from the clinical trials are encouraging, although there are still few data in real-life. Here, we report a study conducted at Siena University Hospital on 20 patients with NP on both hands and feet treated with anti-IL23 for 52 weeks. No differences were evaluated from baseline to week 4 of anti-IL-23 treatment. NAPSI greatly improved at week 24 with almost 60% of patients reaching NAPSI75 and 40% NAPSI50. At week 52, almost 75% of patients reached NAPSI90. No adverse effects were reported in the patients in the study. The clinical response observed in these patients suggests that treatments that target interleukin-23 may be an effective option for NP, especially when refractory to conventional therapies.

CITY-PSO: Prescribing behaviour of French private-practice dermatologists in psoriasis management: An observational, multicentre, cross-sectional study.

BACKGROUND: Information regarding the prescribing behaviour of French private-practice dermatologists (PPDs) is scarce. OBJECTIVES: First, to describe the population of PPDs involved in psoriasis management. Second, to describe the population of adult patients treated for psoriasis and their management. METHODS: We published a call for participation targeting PPDs; we first asked respondents to complete a form regarding their prescribing behaviour, and then to include consecutive patients consulting for psoriasis during a one-month study period and to collect patient data. RESULTS: The 94 participating PPDs included 1022 patients of mean age 52.9±17.9 years. The average body mass index was 28, and 25% had vascular comorbidities. Two thirds of patients had chronic psoriasis, for which 45% had consulted at least 5 times. Psoriasis was mostly with plaques (70.8%) and 11.4% of patients had psoriatic arthritis. The average body surface area (BSA) affected was 10.1%. Among the 679 patients without initial systemic treatment, 159 were started on systemic treatment. The main agents initiated were phototherapy (n=63), methotrexate (n=40), acitretin (n=30) and apremilast (n=20). In multivariate analysis, a higher BSA [Odds Ratio (OR) 1.10, 95% Confidence Interval (CI): 1.07-1.13; P<10-4] and Dermatology Life Quality Index (DLQI) [OR 1.09, 95% CI: 1.03-1.15; P=0.04] were associated with prescription of systemic therapy at the end of the consultation. CONCLUSION: The main limitation of our study was that participating PPDs were strongly involved in psoriasis management, which accounts for the high proportion of moderate-to-severe psoriasis and prescription of systemic treatments. Such committed PPDs and the development of psoriasis networks are key factors for improving the quality of care provided to psoriasis patients.

The potential impact of systemic anti-inflammatory therapies in psoriasis on major adverse cardiovascular events: a Korean nationwide cohort study.

This nationwide population-based cohort study aimed to investigate the impact of systemic anti-inflammatory treatment on the major adverse cardiovascular events (MACE) risk in patients with psoriasis from January 2006 to December 2018, using a database provided by the Korean National Health Insurance Service. Patients were grouped based on the following treatment modalities: biologics, phototherapy, methotrexate, cyclosporine, and mixed conventional systemic agents. Patients who had not received any systemic treatment were assigned to the control cohort. The incidence of MACE per 1000 person-year was 3.5, 9.3, 12.1, 28.4, 39.5, and 14.5 in the biologic, phototherapy, methotrexate, cyclosporine, mixed conventional systemic agents, and control cohorts, respectively. During the 36-month follow-up, the cumulative incidence of MACE in the phototherapy and biologic cohorts remained lower than that of other treatment modalities. Cyclosporine (hazard ratio (HR) = 2.11, 95% confidence interval (CI) = 1.64-2.71) and mixed conventional systemic agents (HR = 2.57, 95% CI = 2.05-3.22) treatments were associated with increased MACE risk. Methotrexate treatment was not associated with MACE. Our finding demonstrates that treatment modalities may affect cardiovascular comorbidities in patients with psoriasis. Thus, an appropriate combination of anti-psoriatic therapies should be considered to manage patients with high cardiovascular risk.IRB approval status: Waiver decision was obtained by the institutional review board, Konkuk University Hospital, Seoul, Republic of Korea (KUH1120107).

Concurrent development of HIV-negative Kaposi's sarcoma and mycosis fungoides in an elderly Inuit from Canada.

An 88-year-old Inuit man from Northern Canada presented with an extensive skin rash associated with numerous violaceous skin nodules on his palms and lower extremities. Biopsy of a skin nodule revealed Kaposi's sarcoma (KS), a human herpesvirus 8 (HHV8)-associated malignancy, whereas biopsy of the erythematous skin showed an atypical infiltrate of CD4-positive T-cells that, together with TCR gene rearrangement and presence of clonal T-cells in peripheral blood by flow cytometry, was consistent with a T-cell lymphoma, mycosis fungoides (MF) subtype. Serology was negative for HIV and HTLV-I/II and no immunodeficiency syndrome was identified. The patient was successfully treated with an oral retinoid for KS, and with topical hydrocortisone and ultraviolet B (UVB) phototherapy for MF. This case highlights the existence of HHV8-related lesions in native persons of Northern Canada, and also that MF-induced immunosuppression combined with immunosenescence may play a role in the development of non-HIV-related KS.

Erythrodermic pityriasis rubra pilaris treatment: Two case reports and literature review.

Pityriasis rubra pilaris (PRP) is a rare inflammatory dermatosis characterized by hyperkeratotic follicular papules and erythematous-desquamative plaques that tend to progressively evolve into erythroderma. Treatment is challenging given that international guidelines are not available and large-scale trials do not exist. Traditionally, many topical and systemic drugs had been used as consolidated agents; recently, biologicals are gaining increasing importance, promisingly dominating the therapeutic scenario ahead. Herein, we present a case series showing the "past" and the "future" therapeutic approaches to erythrodermic PRP, one case treated with acitretin and nb-UVB phototherapy combination, while the other with ustekinumab, performing also a throughout literature review.

Interventions for mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, a malignant, chronic disease initially affecting the skin. Several therapies are available, which may induce clinical remission for a time. This is an update of a Cochrane Review first published in 2012: we wanted to assess new trials, some of which investigated new interventions. OBJECTIVES: To assess the effects of interventions for MF in all stages of the disease. SEARCH METHODS: We updated our searches of the following databases to May 2019: the Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, and LILACS. We searched 2 trials registries for additional references. For adverse event outcomes, we undertook separate searches in MEDLINE in April, July and November 2017. SELECTION CRITERIA: Randomised controlled trials (RCTs) of local or systemic interventions for MF in adults with any stage of the disease compared with either another local or systemic intervention or with placebo. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. The primary outcomes were improvement in health-related quality of life as defined by participants, and common adverse effects of the treatments. Key secondary outcomes were complete response (CR), defined as complete disappearance of all clinical evidence of disease, and objective response rate (ORR), defined as proportion of patients with a partial or complete response. We used GRADE to assess the certainty of evidence and considered comparisons of psoralen plus ultraviolet A (PUVA) light treatment as most important because this is first-line treatment for MF in most guidelines. MAIN RESULTS: This review includes 20 RCTs (1369 participants) covering a wide range of interventions. The following were assessed as either treatments or comparators: imiquimod, peldesine, hypericin, mechlorethamine, nitrogen mustard and intralesional injections of interferon-α (IFN-α) (topical applications); PUVA, extracorporeal photopheresis (ECP: photochemotherapy), and visible light (light applications); acitretin, bexarotene, lenalidomide, methotrexate and vorinostat (oral agents); brentuximab vedotin; denileukin diftitox; mogamulizumab; chemotherapy with cyclophosphamide, doxorubicin, etoposide, and vincristine; a combination of chemotherapy with electron beam radiation; subcutaneous injection of IFN-α; and intramuscular injections of active transfer factor (parenteral systemics). Thirteen trials used an active comparator, five were placebo-controlled, and two compared an active operator to observation only. In 14 trials, participants had MF in clinical stages IA to IIB. All participants were treated in secondary and tertiary care settings, mainly in Europe, North America or Australia. Trials recruited both men and women, with more male participants overall. Trial duration varied from four weeks to 12 months, with one longer-term study lasting more than six years. We judged 16 trials as at high risk of bias in at least one domain, most commonly performance bias (blinding of participants and investigators), attrition bias and reporting bias. None of our key comparisons measured quality of life, and the two studies that did presented no usable data. Eighteen studies reported common adverse effects of the treatments. Adverse effects ranged from mild symptoms to lethal complications depending upon the treatment type. More aggressive treatments like systemic chemotherapy generally resulted in more severe adverse effects. In the included studies, CR rates ranged from 0% to 83% (median 31%), and ORR ranged from 0% to 88% (median 47%). Five trials assessed PUVA treatment, alone or combined, summarised below. There may be little to no difference between intralesional IFN-α and PUVA compared with PUVA alone for 24 to 52 weeks in CR (risk ratio (RR) 1.07, 95% confidence interval (CI) 0.87 to 1.31; 2 trials; 122 participants; low-certainty evidence). Common adverse events and ORR were not measured. One small cross-over trial found once-monthly ECP for six months may be less effective than twice-weekly PUVA for three months, reporting CR in two of eight participants and ORR in six of eight participants after PUVA, compared with no CR or ORR after ECP (very low-certainty evidence). Some participants reported mild nausea after PUVA but no numerical data were given. One participant in the ECP group withdrew due to hypotension. However, we are unsure of the results due to very low-certainty evidence. One trial comparing bexarotene plus PUVA versus PUVA alone for up to 16 weeks reported one case of photosensitivity in the bexarotene plus PUVA group compared to none in the PUVA-alone group (87 participants; low-certainty evidence). There may be little to no difference between bexarotene plus PUVA and PUVA alone in CR (RR 1.41, 95% CI 0.71 to 2.80) and ORR (RR 0.94, 95% CI 0.61 to 1.44) (93 participants; low-certainty evidence). One trial comparing subcutaneous IFN-α injections combined with either acitretin or PUVA for up to 48 weeks or until CR indicated there may be little to no difference in the common IFN-α adverse effect of flu-like symptoms (RR 1.32, 95% CI 0.92 to 1.88; 82 participants). There may be lower CR with IFN-α and acitretin compared with IFN-α and PUVA (RR 0.54, 95% CI 0.35 to 0.84; 82 participants) (both outcomes: low-certainty evidence). This trial did not measure ORR. One trial comparing PUVA maintenance treatment to no maintenance treatment, in participants who had already had CR, did report common adverse effects. However, the distribution was not evaluable. CR and OR were not assessable. The range of treatment options meant that rare adverse effects consequently occurred in a variety of organs. AUTHORS' CONCLUSIONS: ​​There is a lack of high-certainty evidence to support decision making in the treatment of MF. Because of substantial heterogeneity in design, missing data, small sample sizes, and low methodological quality, the comparative safety and efficacy of these interventions cannot be reliably established on the basis of the included RCTs. PUVA is commonly recommended as first-line treatment for MF, and we did not find evidence to challenge this recommendation. There was an absence of evidence to support the use of intralesional IFN-α or bexarotene in people receiving PUVA and an absence of evidence to support the use of acitretin or ECP for treating MF. Future trials should compare the safety and efficacy of treatments to PUVA, as the current standard of care, and should measure quality of life and common adverse effects.

Monotherapy and combination therapy with acitretin for mycosis fungoides: results of a retrospective, multicentre study.

BACKGROUND: Retinoids have long been used in the treatment of cutaneous T-cell lymphomas. However, data on acitretin use for mycosis fungoides (MF) are very limited. OBJECTIVES: To evaluate treatment outcomes of acitretin in patients with MF attending three academic referral centres in different regions of Greece. METHODS: Data on effectiveness, safety and drug survival of acitretin as monotherapy or as adjuvant regimen were collected in a multicentre, register-based, retrospective study. RESULTS: Overall, 128 patients (64.8% male; mean age at MF diagnosis 59.7 years) were included. Folliculotropic MF was present in 24 (18.8%) cases. Most patients (n = 118; 92.2%) had early-stage disease (≤IIA) at acitretin initiation. In all, 28 (21.9%) patients received acitretin monotherapy, while 100 (78.1%) subjects on acitretin concomitantly received phototherapy (n = 65; 50.8%) or topical steroids (n = 27; 21.1%). Acitretin was given as a first-line agent in 73 (57%) cases. A 77.3% overall response rate was noted: 44.5% and 32.8% for complete and partial responses, respectively. Acitretin was more effective as first-line than as a subsequent agent (P = 0.008). A trend towards better response was observed in the combination arm compared to patients receiving acitretin alone (P = 0.056). Median time to best response was 6.9 months (IQR 4.4-9.4); median duration of response was 23.7 months (IQR 11.9-35.4). Overall, the mean length of all treatment patterns was 569 days (SD 718.8). Therapy was discontinued in 5 (3.9%) cases due to drug intolerance. Adverse effects were recorded in 62 (48.4%) cases with dyslipidaemia (n = 31; 24.2%), xerosis (n = 24; 18.6%) and hair loss (n = 10; 7.8%) being the most commonly recorded. CONCLUSIONS: Acitretin, either alone or as adjuvant, showed a stable long-term effectiveness in this cohort, especially when used in the first-line setting. This RAR-selective agonist may serve as an attractive option for treatment of MF and should be further evaluated.

Research "recover from illness defense complex" helper T cell immune mechanisms based on the "Fuxie" theory clearing away heat evil thoroughly nourishing kidney treatment of recurrent blood-heat syndrome Psoriasis.

INTRODUCTION: Psoriasis vulgaris (PV) is a chronic, painful, disfiguring, and disabling dermatological disease, which affects the physical and mental health of patients and impacts their quality of life. Current conventional systemic therapies can be costly, present risks of side effects, have limited efficacy and commonly recur following treatment cessation. Some Chinese herbal medicine therapies have shown therapeutic benefits for psoriasis vulgaris, including relieving symptoms and improving quality of life, and a potential of reducing relapse rate. However, explicit evidence has not yet been obtained. METHODS AND ANALYSIS: This is a pilot randomized controlled trial with the objective of investigating the effect of Jia Wei Liang Xue Xiao Feng San granules on relapse rate of recurrent PV and the correlation between Psoriasis area severity index (PASI) and key psoriasis-related cytokine changes and the number of cells. A total of 102 participants were recruited for this study, including 72 patients with recurrent PV, 15 healthy volunteers and 15 patients with psoriasis vulgaris who have recovered for more than 1 year. A total of 72 patients, with recurrent PV, will be randomized (1:1) to receive the oral Chinese herbal medicine Jia Wei Liang Xue Xiao Feng San or the oral Acitretin Capsule treatments for a period of 8 weeks. After this period, participants whose PASI scores improvement reached more than 75%, will undergo a 52-week follow-up phase.The primary outcome measures are as follows:The secondary study outcomes will include:This trial may provide a novel regimen for recurrent PV patients if the granules decrease recurrence rate without further adverse effects. ETHICS AND DISSEMINATION: The ethics approval was provided by the Sichuan Traditional Chinese medicine regional ethics review committee. The ethics approval number is 2018KL-055. The design and the results of the study will be disseminated through peer-reviewed publications and conference presentations. TRIAL REGISTRATION NUMBER: Chinese Clinical Trial Registry (ChiCTR1900022766).

Acquired perforating dermatosis: clinicopathologic study of a 10-year period at a tertiary teaching hospital.

BACKGROUND: Acquired perforating dermatosis (APD) comprises an uncommon group of skin disorders that develop in adulthood in association with systemic diseases. The aim of this study was to characterize clinicopathologic features and treatment outcomes in a series of patients diagnosed with APD. METHODS: Retrospective study of all patients diagnosed with an APD over a 10-year period (2009-2018) at a tertiary teaching hospital in Lisbon, Portugal. RESULTS: Fifty-seven patients with APD were identified. Thirty-five patients presented lesions in multiple anatomic areas (61.4%), and the lower limbs were the most common location. Forty-six patients reported pruritus (80.7%), which was classified as severe in 21 of them (36.8%). An underlying systemic disease was identified in 53 patients (93.0%). Diabetes mellitus (DM) and chronic kidney disease (CKD) were the most common associated systemic diseases, but psychiatric disorders, malignancies, and chronic infections were present in a significant number of patients. The combination of topical steroids with antihistamines was the most prescribed initial treatment, but only 37.8% of the patients had a complete response. Acitretin, systemic steroids, and phototherapy were the treatments associated with the best outcome. CONCLUSION: Acquired perforating dermatosis can be associated with many systemic disorders that have pruritus as a common factor. Chronic viral infections and an occult malignancy should be sought, particularly in the absence of DM and CKD. The management of APD is challenging and is best achieved with the control of the underlying systemic diseases.

Plasma miR-126 expression correlates with risk and severity of psoriasis and its high level at baseline predicts worse response to Tripterygium wilfordii Hook F in combination with acitretin.

BACKGROUND: Treatment of psoriasis is always difficult, which requires intensive scientific research. OBJECTIVE: Tripterygium wilfordii Hook F (TwHF) with acitretin(TwHF + acitretin) is normally used in treating psoriasis. This study aimed to investigate the correlation of plasma miR-126 expression with risk and severity of psoriasis, and its predictive value of response to TwHF + acitretin treatment in psoriasis. METHODS: MiRNA-126(MiR-126) expression in plasma was analyzed in psoriasis patients at month 0 (M0), M1, M3 and M6 and in health controls (HCs) at enrollment by qPCR. Psoriasis-affected body surface area (BSA) and Psoriasis Area and Severity Index (PASI) score were used to assess severity and treatment response. RESULTS: Plasma miR-126 levels were decreased in psoriasis patients compared with HCs (P < 0.001), with area under the curve (AUC) of 0.771. MiR-126 expression was negatively correlated with PASI score (P = 0.001), and negatively associated with psoriasis-affected BSA (P = 0.825). At M6, 65.3% and 36.1% patients achieved PASI 50 and 75, respectively. MiR-126 increased at M1, M3 and M6 after TwHF + acitretin treatment when comparing with M0 (all P < 0.001). Meanwhile, miR-126 expression baseline in PASI 50 group declined when comparing with non-PASI 50 group (P < 0.001). Additionally, data revealed that the cause of high miR-126 baseline level was due to unsuccessfully achieving PASI 50 at M6 after TwHF + acitretin treatment (P < 0.001). However, miR-126 baseline expression was not a predictive factor for PASI 75 achievement (P > 0.05). CONCLUSION: Plasma miR-126 expression is negatively correlated with psoriasis risk and severity, and its high baseline level can be used as a biomarker to predict worse clinical response to TwHF + acitretin treatment in psoriasis.

Chronic hand eczema in Germany: 5-year follow-up data from the CARPE registry.

BACKGROUND: The CARPE registry was set up in 2009 to prospectively investigate the management of patients with chronic hand eczema (CHE). OBJECTIVES: To report comprehensive follow-up data from the CARPE registry. PATIENTS AND METHODS: We investigated sociodemographic and clinical characteristics, provision of medical care, physician-assessed outcomes, and patient-reported outcomes (PROs). Data were collected between 2009 and 2016, with up to 5 years of follow-up, and are reported descriptively. RESULTS: Overall, 1281 patients were included in the registry (53.7% female). Mean age was 47.0 years. Of the patients, 793 and 231 completed the 2-year follow-up and 5-year follow-up, respectively. At baseline, 5.4% had changed or given up their job because of CHE, the average duration of CHE was 6.1 years, and, in 22.4%, the CHE was severe according to physician global assessment. Systemic treatment (alitretinoin, acitretin, and methotrexate) was prescribed at least once to 39.0% of the patients during the course of the follow-up. Disease severity, quality of life and treatment satisfaction improved over time, and the proportion of patients receiving systemic treatments decreased. CONCLUSIONS: Under continued dermatological care, substantial improvements in disease severity and PROs over time was achieved during the course of the CARPE registry, even in patients with long-standing and severe hand eczema.

Systemic Treatment of Moderate to Severe Psoriasis in Pediatric Patients in Galicia, Spain: A Descriptive Study. / Tratamiento sistémico de la psoriasis moderada-grave en edad pediátrica en Galicia: estudio descriptivo.

BACKGROUND AND OBJECTIVE: Studies on the use of systemic therapy for psoriasis in pediatric patients are scarce. The main aim of this study was to describe the systemic treatments used for moderate to severe psoriasis in pediatric clinical settings. The second aim was to describe the effectiveness and safety of these treatments. MATERIAL AND METHODS: Descriptive, cross-sectional, multicenter study of patients under 18 years of age with moderate to severe psoriasis who were being treated or had been treated with a systemic drug (conventional or biologic) or phototherapy. We recorded demographic and clinical information, treatments received, tolerance, adverse effects, and response to treatment. RESULTS: Data were collected for 40 patients (60% female; mean age, 13 years) who had received 63 treatments in total. The most common first treatment (n=40) was phototherapy (administered to 68% of patients), followed by acitretin (15%). The most common treatments overall (n=63) were phototherapy (57%) and methotrexate (16%). At week 12 (evaluation of systemic treatment and phototherapy), 66% of the patients were classified as good responders and 22% as partial responders. The respective rates for week 24 (evaluation of systemic treatment only) were 36% and 32%. The treatments were well tolerated (97%) and adverse effects were reported in just 11% of cases. There were no treatment discontinuations because of adverse effects. CONCLUSIONS: Phototherapy, followed by methotrexate, was the most common treatment for moderate to severe psoriasis in this series of patients under 18 years. The treatments showed a favorable safety profile and were associated with a good response rate of 66% at week 12 (systemic treatment and phototherapy) and 36% at week 24 (systemic treatment only).

Adjuvant high-dose intravenous immunoglobulins for recalcitrant erosive oral lichen planus: mixed clinical responses.

BACKGROUND: Erosive oral lichen planus (OLP) is, at times, extremely difficult to treat and has a major impact on patients' quality of life. There are only limited therapeutic options, such as topical and systemic glucocorticoids, retinoids, and immunosuppressants with considerable side effects and limited efficacy upon chronic use. OBJECTIVES: In the present individualised clinical trial, we assessed the efficacy of adjuvant intravenous immunoglobulins (IVIG; 2 g/kg/monthly cycle) in addition to the oral retinoid, acitretin, in three patients with refractory OLP over a period of two to six months. MATERIALS & METHODS: The efficacy of adjuvant IVIG treatment was evaluated using the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) which measures both extent of mucosal lesions and functional sequelae. RESULTS: The three OLP patients showed mixed responses to adjuvant IVIG treatment, ranging from therapeutic efficacy to a lack of response to IVIG. CONCLUSIONS: In light of the observed therapeutic responses and a lack of good therapeutic options, adjuvant IVIG, although costly, warrants further investigation as a treatment option for OLP.