Hospitalization and mortality in Asian autoimmune bullous dermatosis patients: A 17-year retrospective study.

Limited data exist on the factors associated with hospitalization and mortality in Asian inpatients with autoimmune bullous dermatoses (AIBDs). This study aimed to elucidate the risk factors affecting hospitalization and mortality rates in Asian patients with AIBDs. A retrospective analysis of patients with AIBDs treated at Siriraj Hospital during a 17-year period was performed using the International Classification of Diseases 10th revision codes. The characteristics of inpatients and outpatients were compared, and mortality rates and associated factors were identified. The study included 360 AIBD patients (180 inpatients, 180 outpatients). Inpatients were significantly younger than outpatients. The identified risk factors for hospitalization were malignancy (odds ratio [OR] 2.83, 95% confidence interval [CI] 1.13-8.04; p = 0.034), moderate to severe disease (OR 2.52, 95% CI 1.49-4.34; p < 0.001), systemic corticosteroid use ≥15 mg/day (OR 2.27, 95% CI 1.21-4.41; p = 0.013) and oral cyclophosphamide treatment (OR 9.88, 95% CI 3.82-33.7; p < 0.001). Kaplan-Meier analysis revealed mortality rates of 26%, 36% and 39% for inpatients with pemphigus at 1, 3 and 5 years, respectively. For inpatients with pemphigoid, the corresponding rates were 28%, 38% and 47%. Infections, particularly pneumonia, were the predominant cause of death in both conditions. This study confirmed that both Asian ethnicity and healthcare disparities may be correlated with adverse outcomes in patients with AIBDs. Pemphigus mortality rates were substantially greater in Asian patients than in Caucasian patients. Continuous monitoring of factors contributing to hospitalization and mortality is imperative to improve treatment outcomes.

Influence of pharmacogenomic polymorphisms on allopurinol-induced cutaneous adverse drug reactions in Thai patients.

BACKGROUND: Allopurinol has been causing substantial morbidity and mortality particularly in Asian population by producing cutaneous adverse drug reactions (cADRs). Nonetheless, there are no data describing whether other genetics are a valid marker for prediction of allopurinol-induced cADRs patients in addition to HLA-B*58:01 allele. The goal of this study was to identify suitable single nucleotide polymorphisms (SNPs) for allopurinol induced cADRs among Thai patients. METHODS: We conducted a case-control association study after enrolling 57 Thai patients with allopurinol induced cADRs and 101 allopurinol-tolerant controls. The genetic biomarkers and associated SNPs located on chromosome 6p21 were examined by TaqMan® SNP genotyping assays in both the cases and the controls. RESULTS: Out of fifteen SNPs in nine genes, we found four combined SNPs (rs3099844 of HCP5, rs9263726 of PSORS1C1, rs9263733 of POLR2LP, and rs9263745 of CCHCR1) were significantly associated with allopurinol-induced cADRs compared to the tolerant controls (OR 73.2; 95% CI 24.2-266.8; P = 1.9 × 10- 24). The overall sensitivity, specificity, positive predictive value and negative predictive value of these combinations were 84%, 94%, 9%, and 100%, respectively. However, the variant alleles of these SNP combinations were detected in 89.5% (51/57) of the cases. Moreover, the HLA-B*58:01 allele was observed in 86.0% of patients with allopurinol-induced cADRs, but only in 4.0% of tolerant controls (OR: 137.2; 95% CI: 38.3-670.5 and p-value = 1.7 × 10- 27). CONCLUSIONS: Thus, this research confirms the association between the specific HLA-B*58:01 allele and all phenotypes of allopurinol-induced cADRs in Thais. Furthermore, there was found the combined four SNPs (rs3099844, rs9263726, rs9263733, and rs9263745) could be used as alternative novel biomarkers for predicting cADRs in patients taking allopurinol.

A retrospective analysis of 65 patients with acrodermatitis continua of Hallopeau.

There are limited data on acrodermatitis continua of Hallopeau (ACH), particularly among Asian populations. The primary aim was to evaluate the clinical features of ACH and treatment approaches in a sizeable multicentre Asian cohort. We analysed data from adult patients diagnosed with ACH. Of 65 patients with ACH, seven patients had ACH with GPP. Females were more frequently affected in both conditions. Five (71.4%) developed GPP 5-33 years after ACH onset, while two (28.6%) developed GPP concurrently with ACH. The onset age for ACH with GPP (27.9 ± 13.6 years) was earlier than that of isolated ACH (39.8 ± 17.3 years). Metabolic comorbidities were common. ACH exhibited a chronic persistent course. Among systemic non-biologics, acitretin was the most frequently prescribed, followed by ciclosporin and methotrexate. Acitretin and ciclosporin demonstrated similar marked response rates, which surpassed that of methotrexate. Regarding biologics, a marked response was more commonly observed with interleukin-17 inhibitors than with tumour necrosis factor inhibitors. Females are predominant in both conditions. The onset age for ACH among Asian patients is earlier (late 30s) than that for Caucasian patients (late 40s). Interleukin-17 inhibitors may be more effective than tumour necrosis factor inhibitors in managing ACH.

The Steatosis-Associated Fibrosis Estimator (SAFE) score for assessing significant liver fibrosis in patients with psoriasis.

BACKGROUND: There is an urgent need for noninvasive tests to identify patients with psoriasis at risk of significant liver fibrosis. OBJECTIVES: To externally validate the ability of the Steatosis-Associated Fibrosis Estimator (SAFE) score to detect significant liver fibrosis in patients with psoriasis using transient elastography (TE) as a reference. METHODS: We analysed data from 75 patients with psoriasis, including TE, SAFE score, Fibrosis-4 Index (FIB-4) and Nonalcoholic Fatty Liver Disease Fibrosis Score (NFS). Significant liver fibrosis was defined as TE values ≥ 7.1 kPa. Diagnostic accuracy was assessed using the area under the receiver operating characteristic curve (AUROC). RESULTS: Fifteen patients (20%) exhibited significant liver fibrosis. The AUROCs for the SAFE and FIB-4 scores were 0.82 [95% confidence interval (CI) 0.67-0.97] and 0.62 (95% CI 0.45-0.79), respectively. The SAFE score outperformed the FIB-4 Index (P = 0.01) but was comparable with the NFS (P = 0.05) in predicting significant fibrosis. Using thresholds of < 0, 0 to < 100 and ≥ 100, the SAFE score categorized 36, 24 and 15 patients into low, intermediate and high-risk groups for significant fibrosis, respectively. The negative predictive value for excluding significant fibrosis with a SAFE score of < 0 was 94.4%, and the positive predictive value for diagnosing significant fibrosis with a SAFE score of > 100 was 53.3%. The duration of psoriasis, joint involvement and methotrexate treatment did not affect the diagnostic ability of the SAFE score whereas age of the patient did. CONCLUSIONS: The SAFE score demonstrated good accuracy in assessing clinically significant fibrosis among patients with psoriasis. This score should prove valuable for risk stratification and patient management in dermatology practice.

Unveiling the role of neutrophils in chronic spontaneous urticaria: Beyond mast cells.

Mast cells and eosinophils are considered pivotal contributors to the pathogenesis of chronic spontaneous urticaria (CSU). However, emerging evidence suggests that neutrophils also play a central role. Cutaneous mast cells and macrophages orchestrate the recruitment of neutrophils through the regulation and activation of diverse processes, including heightened local vascular permeability and chemokine release. Studies have demonstrated increased activation and elevated levels of neutrophil-related cytokines in CSU patients. Moreover, neutrophils have been proposed as antigen-presenting cells during the late-phase reaction of immunoglobulin E-mediated allergy and have been associated with the expression of calcitonin gene-related protein and vascular endothelial growth factor in CSU. Histopathological analysis of lesional skin in CSU patients revealed significantly higher eosinophil and neutrophil counts than unaffected skin. However, the extent of neutrophil infiltration in the skin does not appear to correlate with the number of neutrophils in peripheral blood. The utility of the neutrophil-lymphocyte ratio as a marker for disease activity or remission in CSU remains inconclusive. Neutrophil-targeted therapy may confer benefits for CSU patients who exhibit resistance to antihistamines. Omalizumab has demonstrated its ability to reduce neutrophil counts, the neutrophil-lymphocyte ratio, and the neutrophil-monocyte ratio in peripheral blood. While dapsone and colchicine are recommended as alternative treatment options for CSU, their evidential support from published studies remains limited. Inhibitors targeting interleukin-1 and neutrophil-related cytokines have been proposed as potential therapeutic interventions for patients exhibiting neutrophil predominance. Further research is warranted to gain deeper insights into the involvement of neutrophils in CSU and to explore potential therapeutic interventions.

Prevalence and clinical characteristics of pruritus, and the factors significantly associated with high pruritic intensity in patients with psoriasis: a cross-sectional study.

Despite the significant prevalence of pruritus in psoriasis, its pathogenesis remains unknown, and research on pruritus in Thai psoriasis patients is limited. Objectives: The objective was to investigate the prevalence and clinical characteristics of pruritus, and the factors significantly associated with high pruritic intensity in Thai psoriasis patients. Material and methods: In a cross-sectional study design, pruritus data were collected from the medical records of patients who attended an outpatient psoriasis clinic in Thailand between 2020 and 2021. Results: The overall prevalence of pruritus was 81.2% among 314 psoriasis patients. Psoriasis patients with pruritus had higher Psoriasis Area Severity Index and Dermatology Life Quality Index scores than those without pruritus. The legs, back, arms, and scalp were the most common areas for pruritus. Pruritus was relieved with topical emollients, topical corticosteroids, and oral antihistamines in 66.3, 63.1, and 52.9% of patients, respectively. Female sex, psoriasis body surface area greater than or equal to 10%, and genital psoriasis were factors that independently predicted high pruritus intensity. Conclusion: Psoriasis patients should be screened and treated for pruritus to improve both psoriasis treatment outcomes and patient quality of life. Further studies are needed to clarify the most effective medications for pruritus in patients with severe psoriasis.

Disease activity of patients with chronic urticaria receiving COVID-19 vaccines.

BACKGROUND: Cutaneous adverse events after receiving a COVID-19 vaccine were identified. The disease activity of urticaria after a COVID-19 vaccine has never been explored in chronic urticaria patients. OBJECTIVE: To evaluate disease activity of chronic urticaria after receiving a COVID-19 vaccine. METHODS: A prospective cross-sectional study was conducted in chronic urticaria patients aged 18 or above who visited Siriraj Hospital between July and September 2021, and received the first and second dose of COVID-19 vaccine. The status prior to vaccination, including disease activity, disease control and disease severity was assessed by a urticaria activity score over seven days, urticaria control test, and modified medication score. The disease activity after vaccination was recorded. RESULTS: A total of 130 patients with a mean age of 45.9 ± 14.7 were enrolled in this study. Adenoviral and inactivated vaccines were administered to 85 (65.4%) and 45 patients (34.6%), respectively. Exacerbation was reported in 20 cases (15.4%) after the first dose and 17 cases (13.1%) after the second dose. Nine patients (45%) reported exacerbation after both the first and second dose. The majority of patients only had wheal, while three patients reported wheal with angioedema. No anaphylaxis was reported. Factor predicting exacerbation was concurrent thyroid disease (aRR 2.78, p < 0.01). CONCLUSIONS: Approximately 15% of chronic urticaria patients reported exacerbation after receiving a COVID-19 vaccination. No serious events were observed. Chronic urticaria patients should be vaccinated against COVID-19 after a discussion of the risk of disease flare-up.

Adult female acne: Clinical characteristics and factors significantly associated with polycystic ovary syndrome.

BACKGROUND: Adult female acne (AFA) may be different from adolescent acne, and may be a sign of polycystic ovary syndrome (PCOS). The objective of the study was to investigate the clinical characteristics of AFA, and the factors significantly associated with PCOS in AFA. METHODS: AFA patients aged 25 years or older were enrolled. History taking and dermatologic examinations were performed by dermatologists. PCOS was diagnosed by gynaecologists. Perimenopausal acne (aged 45 years or older) and the Dermatology Life Quality Index (DLQI) were also evaluated. RESULTS: Among 208 patients, mean age was 31.8 ± 7.1 years and 47.1%, 26.9%, and 26% had persistent, late-onset, and recurrent acne, respectively. The common aggravating factors included pre-menstruation (72.6%) and stress (53.8%). Recurrent acne was significantly aggravated by cosmetic products. Higher body mass index (BMI) was positively correlated with acne severity. Acne lesions were predominately located on both cheeks (87.0%) and at the perioral area (81.7%). PCOS was identified in 48.1%. Younger age (≥25 to <33 years), premenstrual flare, and irregular menstruation, but not hirsutism or androgenetic alopecia, were associated with PCOS in univariate and multivariate analysis. Perimenopausal acne was identified in 6.7%. The total mean DLQI score was 8.0 ± 5.4 (range from 0 to 23). CONCLUSIONS: Persistent acne with moderate severity was common in AFA patients and higher BMI was associated with acne severity. PCOS should be screened in AFA patients with younger age, premenstrual flare, and irregular menstruation.

How do we treat psoriasis patients with hepatitis C infections in real-world situations? A retrospective analysis of 34 patients.

BACKGROUND: There is still relatively limited data on psoriasis and hepatitis C virus (HCV) infections. OBJECTIVE: This study investigated the clinical characteristics and treatment of psoriasis patients with HCV infections in real-world practice. METHODS: Medical records of all psoriasis patients with HCV infections who attended the outpatient clinic at Siriraj Hospital over a 10-year period were retrospectively reviewed. RESULTS: Of 34 patients, 26 and 8 patients were men and women, respectively with a mean age of 57.0 ± 8.7 (range, 42.2-77.2) years. The median age of psoriasis onset was 42.7 ± 12.7 (range, 8-67.25) years. With a median follow-up period of 13.6 years, cirrhosis and hepatocellular carcinoma were found in 67.6% and 29.4% of the patients, respectively. The interferon used for HCV treatment exacerbated the psoriasis in 20% of those patients. Conventional treatments and anti-tumor necrosis factors (anti-TNFs) were used in strict collaboration with hepatologists. No patients experienced a worsening of their HCV infection. CONCLUSION: Despite a limited number of patients, a male predominance and late-onset psoriasis were frequently observed. Although, interferon therapy for HCV can exacerbate psoriasis, it is not contraindicated. All conventional treatments and anti-TNFs can be used, provided that there is strict collaboration with hepatologists.

Impact of long-term systemic treatment for psoriasis on liver disease in psoriasis patients with coexisting hepatitis B virus infection.

Continuously updated information is helpful for evaluating the safety of long-term systemic drug use in psoriasis patients with concomitant hepatitis B virus (HBV) infection. To investigate the impact of long-term systemic treatment for psoriasis on liver disease in psoriasis patients with HBV infection. Data of patients during 10-year period were recorded and analyzed. Sixty-six patients (46 males and 20 females) with a mean age of 58.5 ± 13.1 years were recruited. Our study estimated that the 5-year cumulative risks of developing cirrhosis and HCC were 30% and 5%, respectively, in patients receiving systemic treatments for psoriasis. Risks of cirrhosis and HCC were not significantly different between systemic and topical treatment groups. Thirty patients were prescribed systemic treatments (acitretin, methotrexate, ciclosporin, and anti-tumor necrosis factors). Three HBsAg+ patients developed viral reactivation (two patients with methotrexate and one patient with ciclosporin). The effects of systemic treatments for psoriasis on liver outcome in patients with coexisting HBV infection are needed to be determined. HBsAg+ patients are more likely to develop viral reactivation during systemic treatment for psoriasis than HBsAg- patients. Monitoring of liver enzymes and HBV DNA every 3 months is recommended during treatment and for 6 to 12 months after drug discontinuation.

The efficacy of UVA1 phototherapy in psoriasis: Clinical and histological aspects.

BACKGROUND: Although ultraviolet A1 (UVA1) phototherapy is available for nearly 30 years, only few studies have been conducted for plaque-type psoriasis. OBJECTIVES: To determine the efficacy and safety of UVA1 phototherapy in psoriasis by assessing the clinical and histological outcomes. METHODS: This open study enrolled 15 patients with moderate to severe plaque-type psoriasis. All of the patients had skin type IV. A whole-body UVA1 device consisting of 24 lamps, was irradiated at a medium dose of 50 J/cm2 three-times weekly for 30 sessions. Topical and systemic psoriasis treatments were discontinued before and during treatment; patients could only use emollients and antihistamines until 1-month post-completion. Psoriasis Area and Severity Index (PASI) scores were determined at baseline; at sessions 10th, 20th and 30th; and 1 month after treatment. Four-millimetre punch biopsies were obtained from the same psoriasis lesion at baseline and session 30th. Changes in histopathological gradings and polymorphonuclear, lymphocyte and Langerhans cell numbers were monitored. RESULTS: Twelve patients completed the study. The mean age was 41.3 years (range: 25-71). The median PASI scores at baseline, session 30th and 1-month post-treatment were 16 (8.2, 43.3), 11 (4.4, 43.3) and 9.2 (2.7, 36.4), respectively. Although the PASI scores had improved significantly by 1-month post-treatment (P = .006), the histological parameters demonstrated minimal changes. All patients tolerated the phototherapy well and the most common side effect was skin tanning. CONCLUSIONS: While medium-dose UVA1 phototherapy demonstrated some efficacy in moderate to severe plaque-type psoriasis. However, it might not be an excellent choice.

Factors Predicting the Response to Cyclosporin Treatment in Patients With Chronic Spontaneous Urticaria: A Systematic Review.

PURPOSE: This study aimed to systemically review literature relating to factors that could potentially predict a favorable response to cyclosporine A (CsA) treatment for chronic spontaneous urticaria (CSU). METHODS: A systematic literature review was done according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations. RESULTS: A total of 13 studies (404 patients with CSU and 200 healthy patients) were included. There were only 1 randomized controlled trial (RCT) and 12 non-RCTs. Our systematic review showed that positive autologous serum skin test results, positive baseline basophil histamine release assays, positive baseline basophil activation test responses, elevated baseline plasma D-dimer levels, elevated baseline serum interleukin (IL)-2, IL-5, and tumor necrosis factor-alpha (TNF-α) levels, and low baseline serum IgE levels might assist in predicting favorable CsA responses in CSU patients. Decreased plasma D-dimer levels; and decreased serum IL-2, IL-5, and TNF-α levels were reported to be correlated with clinical improvement after CsA treatment. CONCLUSIONS: Since most positive results were from non-RCT articles and some data were still inconsistent, this systematic review identified no reliable practical biomarker for predicting CsA treatment response in patients with CSU. There were no positive predictors with good consistency and mechanical plausibility.

Proteomics in Psoriasis.

Psoriasis has been thought to be driven primarily by innate and adaptive immune systems that can be modified by genetic and environmental factors. Complex interplay between inflammatory cytokines and T-cells, especially Th1 and Th17 cells, leads to abnormal cell proliferation and psoriatic skin lesions. Nevertheless, such mechanisms do not entirely represent the pathogenesis of psoriasis. Moreover, earlier and better biomarkers in diagnostics, prognostics, and monitoring therapeutic outcomes of psoriasis are still needed. During the last two decades, proteomics (a systematic analysis of proteins for their identities, quantities, and functions) has been widely employed to psoriatic research. This review summarizes and discusses all of the previous studies that applied various modalities of proteomics technologies to psoriatic skin disease. The data obtained from such studies have led to (i) novel mechanisms and new hypotheses of the disease pathogenesis; (ii) biomarker discovery for diagnostics and prognostics; and (iii) proteome profiling for monitoring treatment efficacy and drug-induced toxicities.

Cutaneous toxicities of epidermal growth factor receptor inhibitors: A prospective study in 60 Asian patients.

BACKGROUND: Several prospective studies have been conducted in epidermal growth factor receptor (EGFR) inhibitor-related cutaneous reactions in Caucasian patients, but prospective studies in Asian populations are scarce. OBJECTIVE: To investigate the cutaneous side effects of EGFR inhibitors in Asian cancer patients and to assess tumor response to dermatologic manifestations. METHODS: Sixty patients with lung or colorectal cancer who were receiving EGFR inhibitors were prospectively followed for at least one year by oncologists and dermatologists. RESULTS: Of 60 patients (33 males, 27 females), 46 lung cancer patients received erlotinib (n=29) and gefitinib (n=17). Cetuximab was prescribed in 14 colorectal cancer patients. Fifty-eight patients (58/60, 96.7%) developed cutaneous reactions. The most common reactions were xerosis (82.8%), acne (79.3%), and skin desquamation (62.1%). Most reactions were mild and well-tolerated. Of 14 patients who had severe reactions, temporary treatment interruption was necessary in 3 patients and a decreasing dose was required in another 3 patients. There were no statistically significant differences in type, severity, or number of cutaneous reactions between responders (29/58) and non-responders (29/58) to EGFR inhibitors. At median follow-up time of 11.92±1.08 months, no patient died from cutaneous toxicities. Nine patients died from cancer and 11 patients lost to follow-up. CONCLUSION: In this Asian population, almost all patients (96.7%) developed cutaneous toxicities of EGFR inhibitors. Xerosis, acne, and desquamation were common in Asian cancer patients. Most reactions were mild and well tolerated. Due to limited number of patients, this study did not show significant associations between cutaneous toxicities and tumor response.

Analysis of HLA-B Allelic Variation and IFN-γ ELISpot Responses in Patients with Severe Cutaneous Adverse Reactions Associated with Drugs.

BACKGROUND: The prevention and confirmation of drug-induced severe cutaneous adverse reactions (SCARs) are difficult. OBJECTIVE: To determine the benefit of HLA-B allele prescreening and the measurement of drug-specific IFN-γ-releasing cells in the prevention and identification of the culprit drug in patients with SCARs. METHODS: A total of 160 patients with SCARs were recruited from 6 university hospitals in Thailand over a 3-year period. HLA-B alleles were genotypically analyzed. The frequencies of drug-specific IFN-γ-releasing cells in patients with SCARs were also measured. RESULTS: The drugs commonly responsible for SCARs were anticonvulsants, allopurinol, beta-lactams, antituberculosis agents, and sulfonamides. If culprit drugs had been withheld in patients carrying known HLA-B alleles at risk, it would have prevented 21.2% of SCAR cases, mainly allopurinol- and carbamazepine-related SCARs. Culprit drug-specific IFN-γ-releasing cells could be identified in 45.7% (53 of 116) of patients with SCARs caused by 5 major drug groups, particularly in patients diagnosed with drug reactions with eosinophilia and systemic symptoms (DRESS) (50.0%), followed by Stevens-Johnson syndrome/toxic epidermal necrolysis (46.0%), and acute generalized exanthematous pustulosis (31.3%). According to our study, high frequencies of drug-specific IFN-γ-releasing cells were significantly demonstrated in patients who suffered from DRESS phenotype, having anticonvulsants or the drugs belonging to the "probable" category based on the Naranjo algorithm scale, as the culprit drugs. CONCLUSIONS: HLA-B prescreening would succeed in preventing only a minority of SCAR victims. Drug-specific IFN-γ-releasing cells are detectable in almost half of patients. Better strategies are required for better SCAR prevention and culprit drug confirmation.

Assessing the validity and response distribution of the simplified psoriasis index in patients receiving phototherapy.

BACKGROUND/OBJECTIVES: The simplified psoriasis index (SPI) is a three-part multidimensional tool incorporating disease severity, psychosocial impact and historical course completed by the health-care professional (professional SPI, [proSPI]) or the patient (self-assessment SPI, [saSPI]). We aimed to assess the validity and response distribution of proSPI and saSPI in patients with psoriasis undergoing phototherapy. METHODS: The validity and response distribution of SPI was assessed by recording saSPI and proSPI in patients with psoriasis before and after a course of phototherapy. Recruitment ended once 100 complete data sets were available for analysis. RESULTS: Altogether 52 of the 100 patients evaluated were male and most (93) underwent narrowband UVB phototherapy. There was a close correlation between the proSPI-current severity score (proSPI-s) with the psoriasis area and severity index (PASI) score (r = 0.76, r = 0.86) before and after treatment, respectively. Although pretreatment correlation between the saSPI-current severity score (saSPI-s) and PASI was weak (r = 0.39), a more close correlation was noted at the end of treatment (r = 0.50). A moderate correlation was observed between the SPI-psychosocial impact score (SPI-p) and the dermatology life quality index (DLQI), both before and after phototherapy (r = 0.64, r = 0.73). The SPI had wide response distributions in all three domains. CONCLUSIONS: Both versions of SPI demonstrated wide response distributions and the proSPI-s in particular was shown to have good validity with PASI.

Clinicopathological Significance among Patients with In vivo Epidermal Nuclear Staining by Direct Immunofluorescence Study.

CONTEXT: In vivo epidermal nuclear staining (ENS) can be found in patients with autoimmune connective tissue diseases (CNTDs) and other diseases. AIMS: The aim of this study was to reveal the underlying diseases and direct immunofluorescence (DIF) characters of patients with in vivo ENS and association of in vivo ENS with circulating autoantibodies. SETTINGS AND DESIGN: A retrospective analysis was conducted involving skin biopsy specimens submitted for DIF study at the Dermatoimmunology Laboratory at Siriraj Hospital between 2002 and 2012. SUBJECTS AND METHODS: The findings of DIF study, clinical manifestations, and diagnosis of patients who had positive ENS were investigated. STATISTICAL ANALYSIS USED: The SPSS software version 18.0. Descriptive statistics were used to report demographic data, clinical characteristics, and laboratory investigation results. Moreover, Chi-squared test or Fisher's exact test were used to compare the categorical variables. RESULTS: One hundred and thirty-eight out of 3735 submitted specimens (3.7%) showed positive ENS. The most common diagnosis was CNTD (79%) followed by vasculitis (10.1%). Lupus erythematosus was the most common diagnosis among CNTD (78%). No association between levels of serum antinuclear antibodies (ANA) titer and intensity of ENS (P = 0.660). However, we found that patients with positive in vivo ANA had lower prevalence of systemic involvement. CONCLUSIONS: Although lupus erythematosus was the most common diagnosis among patients with in vivo ENS, the presence of ENS does not indicate any specific diagnosis. However, patients with ENS tend to have less systemic involvement.

Diagnostic performance of transient elastography for detection of methotrexate-induced liver injury using Roenigk classification in Asian patients with psoriasis: a retrospective study.

Liver biopsy, the gold standard for monitoring methotrexate-induced liver fibrosis in psoriasis patients, has potential morbidity and mortality. Transient elastography (TE) has been widely used as an alternative non-invasive method. Currently, psoriasis-specific data of TE comparing to Roenigk histopathology is lacking. This retrospective study assessed the diagnostic performance of TE in the detection of methotrexate-induced liver injury and liver fibrosis in Asian psoriasis patients. Risk factors that associated with liver injury by TE and histopathology were also determined. Psoriasis patients who had received methotrexate and undergone both TE and liver biopsy (gold standard) examinations between 2005 and 2016 were enrolled. Ten of 41 patients developed methotrexate-induced liver injury (Roenigk grade ≥3a) and two of them had significant liver fibrosis (Metavir fibrosis stage ≥2). Area under the receiver operating characteristic curve (AUROC = 0.78) indicated that TE was capable of identifying patients with and without liver injury. Using a cut-off TE value of 7.1 kilopascal (kPa), this ultrasound-based elastography yielded 50% sensitivity and 83.9% specificity for detecting methotrexate-induced liver injury and had 50% sensitivity and 76.9% specificity for identifying significant liver fibrosis. A total cumulative dosage of methotrexate, age, gender, metabolic syndrome, and metabolic components were not significantly associated with TE values ≥7.1 kPa and Roenigk grade ≥3a. Thus, using clinical context, laboratory information, and a cut-off TE value of 7.1, TE is an attractable non-invasive tool for identify psoriasis patients who have a low probability of methotrexate-induced liver injury and significant liver fibrosis. Liver biopsy can be reserved for selected patients.

Cardiac involvement in DRESS syndrome.

OBJECTIVE: Cardiac involvement in drug rash with eosinophilia and systemic symptoms (DRESS) syndrome varies considerably between 4% and 21%. Here we present our case and review literatures for its diagnosis and management. An algorithm for diagnosis of cardiac involvement in DRESS syndrome is proposed in this article. DATA SOURCES: Data regarding DRESS-associated myocarditis and eosinophilic myocarditis were gather primarily from MEDLINE database. RESULTS: DRESS syndrome is a hypersensitivity reaction which is due to massive T cell stimulation resulting in cytotoxicity and eosinophil activation and recruitment. It is characterized by fever, morbilliform rash, and various systemic symptoms, in particular hepatitis. Hypersensitivity myocarditis (acute eosinophilic myocarditis) which is typically related to a drug reaction can lead to acute necrotizing eosinophilic myocarditis, cardiac thrombosis and fibrotic stage. Cardiac symptoms range from no symptoms to cardiogenic shock. Diagnosis is based on history, clinical findings, cardiac biomarkers and cardiac imaging techniques. Endomyocardial biopsy is done in a minority of patients for definite diagnosis. If suspected, drug discontinuation and suppression of immune reactions are the first therapies. Corticosteroids are the cornerstone of systemic treatments and should be initiated at the time of diagnosis of DRESS syndrome. Additional therapy and ventricular assist devices could be considered in refractory cases. CONCLUSIONS: According to its high morbidity and mortality, patients with DRESS syndrome should be carefully monitored or screened for cardiac involvement. Multidisciplinary care is important for a successful treatment outcome.