International Consensus Definition and Diagnostic Criteria for Generalized Pustular Psoriasis From the International Psoriasis Council.

Importance: Generalized pustular psoriasis (GPP) lacks internationally accepted definitions and diagnostic criteria, impeding timely diagnosis and treatment and hindering cross-regional clinical and epidemiological study comparisons. Objective: To develop an international consensus definition and diagnostic criteria for GPP using the modified Delphi method. Evidence Review: The rarity of GPP presents a challenge in acquiring comprehensive published clinical data necessary for developing standardized definition and criteria. Instead of relying on a literature search, 43 statements that comprehensively addressed the fundamental aspects of the definitions and diagnostic criteria for GPP were formulated based on expert reviews of 64 challenging GPP cases. These statements were presented to a panel of 33 global GPP experts for voting, discussion, and refinements in 2 virtual consensus meetings. Consensus during voting was defined as at least 80% agreement; the definition and diagnostic criteria were accepted by all panelists after voting and in-depth discussion. Findings: In the first and second modified Delphi round, 30 (91%) and 25 (76%) experts participated. In the initial Delphi round, consensus was achieved for 53% of the statements, leading to the approval of 23 statements that were utilized to develop the proposed definitions and diagnostic criteria for GPP. During the second Delphi round, the final definition established was, "Generalized Pustular Psoriasis is a systemic inflammatory disease characterized by cutaneous erythema and macroscopically visible sterile pustules." It can occur with or without systemic symptoms, other psoriasis types, and laboratory abnormalities. GPP may manifest as an acute form with widespread pustules or a subacute variant with an annular phenotype. The identified essential criterion was, "Macroscopically visible sterile pustules on erythematous base and not restricted to the acral region or within psoriatic plaques." Conclusions and Relevance: The achievement of international consensus on the definition and diagnostic criteria for GPP underscores the importance of collaboration, innovative methodology, and expert engagement to address rare diseases. Although further validation is needed, these criteria can serve as a reference point for clinicians, researchers, and patients, which may contribute to more accurate diagnosis and improved management of GPP.

Clinicopathological characteristics of cutaneous lupus erythematosus patients in Bangladesh.

Introduction: Nearly all epidemiologic studies have involved patients with systemic lupus erythematosus (SLE). Few authors have investigated the characteristics of patients with cutaneous lupus erythematosus (CLE). Aim: To describe the clinical and pathologic characteristics of a series of patients diagnosed with CLE. Material and methods: This is a descriptive retrospective cross-sectional study carried out using the consecutive registered records of 218 patients attending the 'Lupus Clinic' in Chittagong Medical College Hospital during the period between 2010 and 2020. The activity and damage of CLE were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Results: There were 187 (85.8%) females and 31 (14.2%) males, with the female:male ratio being 6 : 1. The mean age was 30.0 ±11.7 years. The chronic cutaneous lupus erythematosus (CCLE) patients numbered 154 (70.6%), followed by acute cutaneous lupus erythematosus (ACLE) n = 46 (21.1%), and subacute cutaneous lupus erythematosus (SCLE) n = 18 (8.3%). In LE-specific skin lesions, the most common manifestation was photosensitivity, 198 (90.8%), followed by discoid rash, 155 (71.1%) and maculo-papular lupus rash, 55 (25.2%). Among LE-nonspecific skin lesions, the most common manifestation was non-scarring alopecia, 123 (56.4%), followed by livedo reticularis, 18 (8.3%), Raynaud's phenomenon, 17 (7.8%), vasculitis, 15 (6.9%), periungual telangiectasia, 7 (3.2%), erythema multiforme, 6 (2.7%) and leg ulcers, 5 (2.3%). Antinuclear antibodies (ANA) were the most common type of autoantibody (n = 132, 60.5%) followed by anti-ds DNA (n = 91, 41.7%) and anti-phospholipid antibodies (n = 9, 4.1%). Conclusions: CCLE was the most common subtypes of CLE. Photosensitivity was the most common clinical manifestation, whereas ANA were the most frequent autoantibodies of the LE patients of this region. Patients with different subtypes of CLE have distinct clinical and pathological characteristics.

Clinical characteristics of itch in cutaneous lupus erythematosus: A prospective, multicenter, multinational, cross-sectional study.

OBJECTIVE: Pruritus is an important symptom frequently accompanying various inflammatory skin conditions and some recent data indicated that it may be associated with autoimmune connective tissue diseases. The aim of this study was to assess the frequency and clinical presentation of itch in CLE. METHODS: A multinational, prospective, cross-sectional study was performed to assess the prevalence, intensity and clinical characteristic of pruritus in various subtypes of CLE. A total of 153 patients with active CLE lesions were included. Their age ranged between 17 and 82 years (mean 49.8 ± 15.4 years), and 115 patients (75.2%) were women. The disease activity and damage were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Pruritus severity was assessed with Numeric Rating Scale (NRS) and the 12-Item Pruritus Severity Scale. Dermatology Life Quality Index and EQ-5D questionnaire were used to measure quality of life. RESULTS: Pruritus was present in 116 (76.8%) of patients of whom half had NRS scoring equal or above 4 points indicating moderate or severe pruritus. Most commonly itch was localized on the scalp, face (excluding ears and nose) and arms (40.5%, 36.2%, 31.9%, respectively). Sensations connected with pruritus were most frequently described as burning, tingling and like ants crawling feeling, but 31.9% patients described it as "pure itch". More than half of patients reported that pruritus was present every day, and it was most frequent during the evenings. The pruritus scoring and the CLASI activity score were significantly correlated (r = 0.42, p = 0.0001), while no correlation was found with the CLASI damage score (p = 0.16). Both the maximum and average itch intensity were correlated with systemic lupus erythematosus (SLE) activity measured with the Systemic Lupus Erythematosus Disease Activity Index. CONCLUSIONS: Pruritus is a common, but frequently overlooked symptom of CLE. Its intensity correlates with the activity of CLE, but not with the skin damage. In more than a half of patients it occurs on a daily basis. The correlation between the intensity of pruritus and the activity of the skin lesions and the systemic involvement indicate that pruritus could be an individual indicator of both SLE and CLE activity.

Comparison of effectiveness of topical tacrolimus 0.1% vs topical halobetasol propionate 0.05% as an add-on to oral hydroxychloroquine in discoid lupus erythematosus.

In recent years, calcineurin inhibitors have been used as the first line alternative to topical corticosteroids in the treatment of discoid lupus erythematosus (DLE). We aim to evaluate the efficacy and safety of topical tacrolimus 0.1% vs topical halobetasol propionate 0.05% in patients with DLE. This comparative study was carried out in the Department of Dermatology and Venereology, Chittagong Medical College Hospital (CMCH), Bangladesh between the period of July 2018 and June 2019. The change of DLE activity assessed with the cutaneous lupus erythematosus disease area and severity index was used as a primary outcome measure. The effective sample was 40 patients in each group. Both groups were similar in terms of baseline demographic and clinical characteristics. After 8 weeks of treatment, the mean total erythema score decreased significantly in both groups (in tacrolimus treated group [TTG] from 12.53 ± 8.05 to 8.03 ± 5.69, [P < .001] and in halobetasol propionate treated group [HTG] from 11.83 ± 7.17 to 7.30 ± 4.56 [P < .001]), as well as the mean total scale/hypertrophy score (in TTG from 8.08 ± 5.30 to 4.33 ± 3.21; [P < .001] and in HTG from 7.40 ± 4.73 to 3.68 ± 2.01, [P < .001]. The magnitude of reduction was significantly better in HTG [P = .032]). The mean total activity score decreased significantly in both groups (in TTG from 22.95 ± 13.40 to 14.33 ± 8.89, [P < .001] and in HTG from 22.15 ± 11.95 to 13.7 ± 7.22, [P < .001]). The present study demonstrated that tacrolimus 0.1% ointment and halobetasol propionate 0.05% ointment had a comparable efficacy in DLE patients; however, halobetasol showed significantly better improvement regarding scaly, hypertrophic lesions.

Efficacy of oral methotrexate (MTX) monotherapy vs oral MTX plus narrowband ultraviolet light B phototherapy in palmoplantar psoriasis.

Palmoplantar psoriasis (PPP) is a chronic, inflammatory dermatosis of the palms and/or soles with significant morbidity. It is notoriously difficult to treat and unresponsive to traditional topical agents. We aim to compare the effect of oral methotrexate (MTX) monotherapy vs MTX plus narrowband ultraviolet light B (NB-UVB) in the treatment of recalcitrant PPP. This was a comparative clinical trial involving 90 patients of PPP. Eligible patients were randomly assigned to one of the two treatment groups. We aim patients in group A received 10 mg oral MTX weekly, and patients in group B received oral MTX 10 mg weekly and NB-UVB sessions twice weekly for 12 weeks. There was a statistically significant difference in reduction of modified PPP Area Severity Index (m-PPPASI) of patients in MTX plus NB-UVB at week 12. The mean m-PPPASI at week 12 was 3.66 ± 2.11 in MTX plus NB-UVB group and 6.51 ± 2.04 in MTX only group (P < .001). Marked improvement (m-PPPASI 75) was achieved in 20 (44.44%) patients in MTX plus NB-UVB group compared with 6 (13.3%) in MTX monotherapy group (P < .001). Combination of MTX and NB-UVB phototherapy helps to attain a better clinical response (reduction in m-PPPASI score) than MTX monotherapy in the treatment of recalcitrant PPP.

Prevalence of Pruritus in Cutaneous Lupus Erythematosus: Brief Report of a Multicenter, Multinational Cross-Sectional Study.

Pruritus is an important symptom frequently accompanying various inflammatory skin conditions. Some recent data have indicated that it may also be associated with autoimmune connective tissue diseases, including systemic sclerosis and dermatomyositis; however, studies on the prevalence and clinical characteristics of pruritus in CLE are limited. We have performed a multinational, prospective, cross-sectional study in order to assess the prevalence and intensity of pruritus in adult patients suffering from various subtypes of CLE. After developing a questionnaire assessing various aspects of pruritus, we have surveyed 567 patients with cutaneous involvement during the course of LE regarding the presence and intensity of pruritus. Pruritus was present in 425 of all patients (75.0%) and was most frequently reported by subjects with acute CLE (82.1%), followed by chronic CLE (78.8%), subacute CLE (65.9%), and intermittent CLE (55.6%) (p<0.001). Based on the Numerical Rating Scale, the severity of itch was mild, moderate, and severe in 264 (62.1%), 98 (23.1%), and 63 (14.8%) patients, respectively. The highest mean pruritus intensity was reported by subjects with hypertrophic LE (5.1 ± 3.0 points) followed by generalized discoid LE (3.6 ± 3.0 points), subacute CLE (3.0 ± 3.0 points), chilblain LE (3.0 ± 1.0 points), localized discoid LE (2.6 ± 2.0 points), intermittent CLE (2.6 ± 3.0 points), acute CLE (2.5 ± 1.2 points), and lupus erythematosus profundus (1.9 ± 2.7 points). In conclusion, pruritus is a frequent phenomenon in CLE; however, in most patients it is of mild severity. Further studies are needed to better characterize its clinical characteristics and influence on patients' well-being.

Leprosy profiles in post-elimination stage: a tertiary care hospital experience.

BACKGROUND: Most countries that were previously highly endemic for leprosy have achieved elimination at the national level. OBJECTIVES: To find out the pattern, prevalence, and trends of leprosy in the post-elimination stage. METHODS: A descriptive retrospective cross-sectional study was carried out using the registered records of patients attending the leprosy clinic in Chittagong Medical College Hospital between the periods 2001 and 2011. RESULTS: The new case detection rate was declining. The prevalence rate was 0.75 at the end of 2011. Of a total of 789 patients, males (74%) outnumbered the females (26%). The age of the patients in the study group ranged from 6 years to 87 years with mean age 35.58 years ± 0.05 SEM. Paucibacillary (PB) and multibacillary (MB) cases were 314 (39.80%) and 475 (60.20%), respectively. MB percentage was increasing more among new cases in the study period, and 119 (15.08%) patients presented with grade 2 deformities. Smear positive cases were 141 (17.87%). Leprosy reaction comprised of 193 (24.46%) type 1, 68 (8.62%) type 2, and 97 (12.29%) neuritis cases. LIMITATIONS: This is a retrospective study from a single tertiary health center. CONCLUSION: MB cases with grade 2 deformities are in an upward trend, and rates of children are declining. Developing reinforced new therapies to curb reactions, deformities are very important and contact tracing, especially of children, is essential. Domiciliary treatment needs to be made available to ensure early diagnosis.