例1 男, 12岁10个月, 全身反复红斑、脓疱9年, 加重3个月于2020年12月入院。9年前无明显诱因全身出现弥漫性红斑、脓疱, 伴高热, 结合皮损病理表现诊断为脓疱型银屑病, 先后使用阿维A、环孢素、甲氨蝶呤及重组人Ⅱ型肿瘤坏死因子受体-抗体融合蛋白等治疗, 病情可缓解, 但仍反复发作。3个月前皮损复发, 2周前皮损突然加重, 伴持续性高热, 最高39.9 ℃。无药物过敏史, 个人史无特殊, 无银屑病家族史。入院体检:体温39 ℃, 身高143 cm, 体重34 kg, 心肺腹部等检查未见异常。皮肤科检查:躯干、四肢泛发性水肿性红斑, 上覆米粒大小脓疱, 融合形成脓湖, 伴黄色云片状鳞屑(图1A);束发征、地图舌均阳性;关节无肿胀, 活动自如。实验室检查:C反应蛋白77 mg/L(参考值:< 8 mg/L, 下同), 白细胞计数17.11 × 109/L(4 × 109/L ～ 10 × 109/L), 中性粒细胞计数3.15 × 109/L(1.4 × 109/L ～ 6.5 × 109/L);白蛋白45.4 g/L(35 ～ 55 g/L)。肝肾功能、乙肝病毒抗原抗体、丙肝病毒抗体、结核感染T细胞检测(T-SPOT)、胸部X线片筛查均未见异常。脓疱皮损处分泌物培养阴性, 血培养阴性。自身炎症性疾病相关基因检测:患儿IL-36RN基因存在c.115+6T>C纯合突变及c.227C>T杂合突变, 其中c.115+6T>C纯合突变分别来自父亲及母亲, c.227C>T杂合突变来自父亲。

BACKGROUND:The scientific evidence of methotrexate (MTX) in children with severe plaque psoriasis is scarce.OBJECTIVES:To retrospectively evaluate the efficacy and safety of oral MTX in children with severe plaque psoriasis in a single center in China.METHODS:We enrolled 42 children with severe plaque psoriasis who were administrated MTX. Efficacy was evaluated by the psoriasis area and severity index (PASI) score, physician global assessment (PGA) score, and body surface area (BSA) score. The Children's Dermatology Life Quality Index (CDLQI) score and safety data were recorded.RESULTS:Among 42 children (22 males, 20 females), the mean age was 11.2 years old. The initial weight-based dosage of oral MTX ranged from 0.1 to 0.3 mg/kg weekly. Overall, 80.6 and 47.2% of patients achieved PASI75 (at least 75% improvement from baseline in PASI score) and PASI90 (at least 90% improvement from baseline in PASI score) at week 12, respectively. 72.2% of patients achieved PGA 0/1 at week 12. BSA and PGA scores significantly decreased from baseline from week 4, accompanied by CDLQI score improvement from week 8. The steady effect of MTX could be reached at week 16. Elevated liver enzymes (28.6%) and infections (28.6%) were the most common side effects. Relapse was recorded in 9 (30.0%) of 30 patients, and the mean posttherapy disease-free interval was 7.2 months.CONCLUSIONS:MTX is an effective and safe option for children with severe plaque psoriasis with adequate monitoring.

Objective:To evaluate the effect of oral acitretin on the height and bone development of children.Methods:Clinical and imaging data were collected from 106 children receiving oral acitretin for at least 1 month in Department of Dermatology, Beijing Children′s Hospital from March 2007 to January 2021, and retrospectively analyzed. The main outcome measures were height and near-adult height. Multivariate logistic regression analysis was carried out to investigate relevant factors for short stature in children, and non-inferiority test was used to analyze the proximity of the actual height to target height of children who had reached near-adult height. The secondary outcome measures were bone age and epiphyseal closure. Wilcoxon signed-rank test was used to analyze differences in the value of bone age minus chronological age between the baseline and last follow-up, and the premature closure of epiphysis was also evaluated.Results:Among the 106 children, 62 were males and 44 were females; 84 were diagnosed with pustular psoriasis, 10 with psoriasis vulgaris, 11 with pityriasis rubra pilaris, and 1 with lupus miliaris disseminatus faciei. These children received oral acitretin at doses of <1 mg·kg -1·d -1 for 1 - 90 months. Among the 96 children aged under 18 years, 91 (94.8%) were of normal stature, and 5 (5.2%) were short in stature; among the 83 children receiving acitretin monotherapy, 81 (97.6%) were of normal stature, and 2 (2.4%) of short stature. Binary logistic regression analysis showed that the risk of short stature caused by acitretin combined with glucocorticoid therapy was 76.57 times higher than that of acitretin monotherapy ( OR = 77.57, 95% CI: 2.20 - 2 738.82, P = 0.017) , while the type of disease, gender, age at onset, age at initial treatment with acitretin, course of treatment, and average daily dose of acitretin did not significantly affect the stature of children ( P = 0.988, 0.214, 0.087, 0.078, 0.066, 0.350, respectively) . At the last follow-up visit, 13 children who had reached near-adult height were of normal stature, and the non-inferiority test showed that their near-adult height was not inferior to the target height (Satterthwaite = 0.23, P = 0.030) . Bone age was evaluated in 45 children at baseline and last follow-up visit, there was no significant difference in the value of bone age minus chronological age between the baseline and last follow-up ( Z = -0.85, P = 0.250) , and no patients experienced premature closure of epiphysis before and after the treatment. Conclusion:This study preliminarily revealed that oral acitretin at doses of <1 mg·kg -1·d -1 for less than 90 months might not significantly affect the height and bone development of children.

Cutaneous sterile pustulosis is categorized as a non-infectious and non-follicular impetigo, with a low prevalence and difficulty in the treatment.Deficiency of interleukin(IL)-36 receptor antagonist (DITRA) is an auto inflammatory disease featured by the decreased interleukin-36 receptor antagonist (IL-36Ra) activity caused by IL36 RN mutation.Functional or structural defects of IL-36Ra increase the secretion of inflammatory and pro-inflammatory factors by keratinocytes, macrophages and dendritic cells.Upregulation of IL-36 receptor agonists induce type 17 helper T lymphocytes to secrete IL-17, which is essential for the onset of multiple subtypes of aseptic pustulosis.Research on the relationship between DITRA and cutaneous sterile pustulosis is important for developing targeted therapies.