Case report: Interleukin-17 targeted biological therapy in netherton syndrome.

Netherton syndrome (NS) is rare and multisystemic congenital skin disorder classically distinguied as a triad of congenital ichthyosiform erythroderma, trichorrhexis invaginata (TI), and an atopic diathesis. Recent advances in pathogenesis have explored the role of IL-23/Th17 pathway in NS. Herein, we present a 17 years old girl harbouring homozygous four base pair deletion in exon 26 of the SPINK5 gene, presented with pruritus, scaling, dry skin and generalized eczematous lesions. She was administered anti IL17A (subcutaneous secukinumab) therapy. The treatment was well tolerated and resulted in a favourable clinical response, reduction of the IL17A gene expression and CD4 + Th17 cell population after 6 months which revealed an abrogation of Th17-skewing during therapy.

Chronic spontaneous urticaria and metabolic syndrome: a relationship conundrum.

Chronic spontaneous urticaria (CSU) and metabolic syndrome (MetS) are associated with low-grade inflammation. Despite intensive research and various hypothesis, there is lack of specific pathomechanisms of urticaria. Previous studies have suggested low-grade inflammation in obesity which may be linked to urticaria. However, there is limited literature on association of MetS and CSU. So, this study was conducted to evaluate the association of MetS and its components in patients with CSU. This was a hospital-based and cross-sectional cohort study in which 481 patients of CSU and 240 age- and gender-matched controls were recruited. MetS was defined using the revised National Cholesterol Education Program Adult Treatment Panel III. BMI, waist circumference, blood pressure, fasting blood sugar, plasma insulin, and lipid profile were measured after overnight fasting. Pearson's Chi-squared test was used to calculate significance. Logistic regression analysis was done to assess MetS as a predictor of CSU. All patients were started on antihistamines according to the severity of the disease. CSU patients comprised 220 men (45.7%) and 261 women (54.3%) with 97 patients (20.12%) qualifying criteria for metabolic syndrome compared with 73 (30.42%) controls without any significant difference (p = 0.177). CSU was significantly associated with a higher prevalence of central obesity (p = 0.003) but CSU patients with central obesity did not have a higher urticaria activity score (p = 0.727) or serum IgE levels (p = 0.359). In conclusion, our study found an increased association of central obesity with CSU that was not related to the severity of urticaria. This is of significance as obesity is the most prevalent and first component of MetS to be developed. There was no increase in the overall prevalence of MetS in patients with CSU. The increased association of obesity and urticaria in our study can be partially explained by the role of antihistamines in modulation of metabolic pathway and appetite. Future research into the same can give better insights and thus can aid in better management options in CSU patients.

Oral vitamin D versus acitretin in congenital non-syndromic ichthyosis: double blinded, randomized controlled trial.

BACKGROUND: Vitamin D has emerged as a promising treatment for congenital ichthyosis for which no comparative studies exist. METHODOLOGY: In this randomized, double-blinded study, patients with congenital ichthyosis received either Vitamin D 2000 IU/day (group A) or acitretin 0.5 mg/kg/day (group B) for 24 weeks. The primary outcome was improvement in the Visual Index for Ichthyosis Severity (VIIS) and Ichthyosis Area Severity Index (IASI). Secondary outcomes included Ichthyosis Quality of Life Index-32 (IQoL-32), mRNA expression of RORγt and IL-17 and adverse events. RESULTS: Twenty-four patients completed the study. Group A (n = 11) showed a significant decrease in VIIS (p = 0.023) and IASI (p = 0.026) at 12 but not 24 weeks. Group B (n = 13) showed a significant decrease in IASI at 24 weeks only (p = 0.016). The IQoL-32 improved over 24 weeks in both groups. A significant decrease in the mRNA expression of RORγt (p = 0.048) and IL-17 (p = 0.023) was seen only in group A. There was no significant difference between the two treatment arms in terms of VIIS, IASI and IQol-32 at 12 and 24 weeks. No serious adverse events were observed. CONCLUSION: Vitamin D maybe an alternative to acitretin in the treatment of congenital ichthyosis where it reduces the expression of RORγt and IL-17.