Progression and flaring of focal dermal hypoplasia during an acute illness

Focal dermal hypoplasia (FDH), or Goltz syndrome, is a rare genodermatosis affecting tissues of mesodermal and ectodermal origin. The characteristic skin changes have been reported to symptomatically flare in response to certain triggers as well as to progress over time in some cases. We present the case of a 5-year-old girl with cutaneous flaring and progression of FDH in the setting of septic shock. This case adds to the growing body of literature on both flaring and progression of the cutaneous manifestations of FDH.     

Case report of severe refractory inflammatory dermatoses in a young female diagnosed with hereditary alpha tryptasemia

Hereditary alpha tryptasemia (HaT), an autosomal dominant condition first described in 2014, has previously been associated with multiple dermatologic, allergic, gastrointestinal, neuropsychiatric, autonomic, and connective tissue abnormalities. We describe a pediatric patient with predominantly mixed cutaneous inflammatory manifestations and atopic manifestations resistant to treatment who was found to have HaT. HaT should be considered in individuals with refractory inflammatory dermatologic disease and signs and/or symptoms concerning for mast cell activation.     

Survey of factors related to diabetic foot pruritus in the elderly in Shanghai

The objective of the study is to explore the status quo of foot pruritus and related factors in elderly diabetics and provide a reference for targeted preventive measures. The study involved a survey using a self-designed foot pruritus assessment scale to understand the status quo of foot pruritus among 411 cases of elderly diabetics from 5 communities in Shanghai. The morbidity rate of foot pruritus in elderly diabetics in the community was 20.1%. Good self-management behaviour was the protective factor, while diabetic peripheral neuropathy, hyperlipidemia, and dry skin were risk factors (all P < 0.05). The incidence of foot pruritus in elderly diabetics was high and influenced by several factors. We recommend that self-management behaviour of patients be improved. Additionally, screening and interventions to address hyperlipidemia, diabetic peripheral neuropathy, and dry skin should be conducted regularly to prevent diabetic foot ulcers.     

Long-term dupilumab therapy in Netherton syndrome with severe atopic manifestations: Case report and review of the literature

We herein present a unique patient of Netherton syndrome (NS) with ichthyosis linearis circumflexa (ILC) lesions associated with severe atopic manifestations since infancy, showing different responses of atopic and ILC lesions to a 2-year dupilumab therapy. The atopic eczematous lesions and pruritus healed remarkably, dramatically improving the patient's quality of life, whilst the scalp hair showed a clinical and light microscopic improvement. The additional recovery in axillary/pubic/extremity hair growth, sweating and nail growth in the presented case was not previously reported in NS patients treated with dupilumab. However, dupilumab had no therapeutic effect on ILC lesions which were not pruritic and showed a treatment-independent wax and waned course.     

中药口服结合熏洗治疗老年皮肤瘙痒症的临床效果

目的 探究中药口服结合熏洗治疗老年皮肤瘙痒症的效果。方法 选取2022年4月-2023年3月我院 收治的87例老年皮肤瘙痒症患者作为研究对象，随机分成常规组43例和联合组44例。常规组采取常规治 疗，联合组采取常规治疗+中药口服结合熏洗治疗，比较两组瘙痒程度与继发皮损严重程度、睡眠质 量与生活质量、不良反应发生率、皮肤瘙痒复发率以及临床疗效。结果 两组治疗后瘙痒程度与继发 皮损严重程度的评分均低于治疗前，且联合组低于常规组（P＜0.05）；两组治疗后睡眠质量与生 活质量评分均低于治疗前，且联合组低于常规组（P ＜0.05）；两组不良反应发生率比较，差异 无统计学意义（P＞0.05）；联合组皮肤瘙痒复发率低于常规组（P＜0.05）；联合组治疗总有效率高于 常规组（P＜0.05）。结论 中药口服结合熏洗治疗老年皮肤瘙痒症的效果确切，可减轻患者的瘙痒程度与 继发皮损严重程度，有助提高睡眠质量与生活质量，降低不良反应发生几率，治疗有效率较高。     

肺肠同治指导下中医药灌肠干预持续性血液透析尿毒症皮肤瘙痒研究

目的 从“炎症-PTH”循环角度探索“肺肠同治”的科学性及“肠-肺-皮毛”关系内涵。方法 240例持续性血液透析（MHD）尿毒症皮肤瘙痒（UP）的热毒证患者随机分为6组：肺肠同治组（凉膈散）、治肠组（调胃承气汤）、治肺组（凉膈散与调胃承气汤差异药物）、乳果糖组、生理盐水组及氯雷他定组，在慢性肾脏病一体化治疗的基础上，前5组采用封闭式灌肠治疗，氯雷他定组加用氯雷他定口服，持续治疗60天，比较6组治疗前后的临床疗效。并观察其瘙痒可视模拟评分（VAS）、甲状旁腺素（PTH）、血钙、磷、及相关炎症指标（hs-CRP、TNF-α、IL-6）的水平。结果 各组总有效率由高到低依次为：肺肠同治组（92.11%） > 治肠组（84.21%） > 治肺组（76.92%） > 氯雷他定组（66.23%）、乳果糖组（67.57%） > 生理盐水组（58.97%），其中氯雷他定组与乳果糖组无统计学差异。较治疗前，各组治疗后VAS评分、PTH、钙、磷、相关炎症指标（hs-CRP、TNF-α、IL-6）皆有所改善，而治疗后各组间上述指标比较，与有效率指标的比较结果相一致。结论 肺肠同治MHD UP具有科学性，“肠-肺-皮毛”存在一定关系。     

Japanese subgroup analysis of GLIMMER: A global Phase IIb study of linerixibat for the treatment of cholestatic pruritus in patients with primary biliary cholangitis

Aim

To compare patient characteristics and outcomes between the overall and Japanese populations of GLIMMER.

Methods

GLIMMER was a multicenter, double-blind, randomized, placebo-controlled, Phase IIb study evaluating linerixibat for the treatment of pruritus in patients with primary biliary cholangitis.

Results

In total, 147 patients were randomized in the GLIMMER overall population with 38 patients comprising the Japanese population. Demographics and baseline clinical characteristics were similar across treatment groups and between both populations. A reduction in mean worst daily itch score from baseline to week 16 (primary endpoint) was seen in all groups, with the largest reduction observed with linerixibat 40 mg twice daily (BID; −2.92 [95% confidence interval: −5.07, −0.76] and −2.86 [95% confidence interval: −3.76, −1.95] for Japanese and overall populations, respectively). The highest proportion of responders was generally in the 40 mg BID group in both populations regardless of the responder definition applied. Improvements in health-related quality of life were generally consistent in both populations. In the Japanese and overall populations, on-treatment drug-related adverse events were reported in 25% and 19% of patients in the placebo group and 0%–86% and 31%–78% of patients in the linerixibat groups, respectively. Consistent with the mechanism of action, the most common events were gastrointestinal in nature. The effects of linerixibat on pharmacodynamic biomarkers favored BID dosing.

Conclusions

Therapeutic responses and safety of linerixibat were consistent between the Japanese and overall populations of GLIMMER. Linerixibat may provide an effective treatment option for cholestatic pruritus in patients with primary biliary cholangitis.

Clinical Trial Registration

NCT02966834.