Background:Kimura disease (KD) presents a diagnostic challenge to clinicians because of its rarity and atypical symptoms in its early stages, and it is difficult to treat and prone to recurrence or involvement of other organs. Aims and Objectives:This study aims to investigate the possible relevance of renal involvement and recurrence by analysing the clinical presentations, laboratory results, histopathological features, therapeutic data and follow-up results of KD. Materials and Methods:A total of 27 patients diagnosed as KD in two hospitals from January 1999 to December 2021 were analysed retrospectively in this study based on the diagnosis of histopathology. Results:KD mainly affected male more than female (8:1) with the onset age ranging from 3 to 58 years (median 29.8 years). The common initial symptoms included subcutaneous soft tissue or lymph node enlargement, non-specific skin lesions and proteinuria. One patient presented cough and expectoration as the first symptoms. KD patients often had high levels of serum immunoglobulin E (IgE) and basophils, which exhibited a significantly positive correlation with renal involvement and recurrence (p < 0.05). Early mass resection could prevent the development of nephritis and decrease the risk of relapse (p < 0.05). Conclusion:KD should be noted in patients presenting with intractable and relapsing atopic skin lesions and (or) subcutaneous mass. Patients with high levels of serum IgE and blood basophils may be prone to developing KD-associated nephritis and predict a high risk of recurrence. Early surgical removal of the mass may result in a better prognosis.

Psoriasis, characterized by erythematous plaques, desquamation, persistent pruritus, and discomfort, significantly compromises the quality of life for afflicted individuals. The intricate interplay of immunological, genetic, and environmental factors has left the molecular intricacies of psoriasis only partially elucidated, underscoring the imperative to unravel these intricate mechanisms. In contradistinction to conventional techniques such as nuclear magnetic resonance (NMR) and liquid chromatography-mass spectrometry (LC-MS), the burgeoning laser desorption/ionization (LDI) metabolic paradigm holds substantial promise in elucidating the molecular mechanism of psoriasis. Nonetheless, achieving heightened sensitivity and high-throughput metabolite detection within intricate clinical biosamples mandates stringent requisites, encompassing favourable ionization efficiency and high surface area. Henceforth, we proffered a dual-strategy entailing the utilization of transition metal carbides (MXene) with unique morphology and the integration of multi-walled carbon nanotubes (MWCNTs) to ameliorate these challenges for psoriasis. We have corroborated the morphological architecture, elemental composition, and efficacy of LDI when applied to MWCNTs-doped MXene. Egregiously, through the employment of both animal models and clinical specimens, we attained diagnostic efficiencies of 0.959 and 0.924 via receiver operating characteristic (ROC) curve analysis, respectively, for discrimination psoriasis from control individuals. Doped MXene evinced exceptional signal amplification and imperviousness to salt and protein interference. Furthermore, enrichment pathway analysis unveiled a significant escalation in leukotriene E4 and arachidonic acid levels within the psoriasis cohort. Harnessing LDI metabolic fingerprints, concomitant with MWCNTs-doped MXene, expedited the recognition of the distinct metabolic characteristics inherent to psoriasis, thereby laying bare the dysregulation in leukotriene metabolism. This epochal breakthrough holds immense potential to usher in new vistas for exploring the underlying mechanisms governing the onset and progression of psoriasis-related disorders, thereby significantly contributing to the refinement of psoriasis health management strategies.

A 19-year-old male patient presented with a slow-growing mass on his scalp over a 10-year history. After resecting the mass, he was diagnosed with pilonidal cyst based on the clinical and histological findings. An unusual pilonidal disease is observed on the scalp in this report.

Abstract Background NLRP12 has been authenticated as an important negative regulator in multiple metabolic and inflammatory diseases. Psoriasis is an important inflammatory disease and often suffer from comorbidities such as inflammatory bowel disease, obese, etc. However, the role of NLRP12 in psoriasis remains unexplored. Methods SNP mutation sites of NLRP12 gene were screened in psoriasis and control groups, followed by genotyping and correlation analysis. The expression of NLRP12 gene and protein in blood and tissue samples of psoriasis patients were determined by Quantitative Real-time polymerase chain reaction (qPCR), immunohistochemistry and Western Blot. Results We found a significant increase in the mutation frequency of NLRP12 rs12460528 co-dominant model, particularly in the dominant model. The dominant model GG + GA of NLRP12 rs12460528 exhibited an OR value of 4.167 (1.847–9.402). Furthermore, the qPCR results a significant upregulation of NLRP12 mRNA expression in psoriatic lesions and peripheral blood. The expression of NLRP12 protein was observed to be significantly elevated in psoriatic tissue by immunohistochemistry and Western Blot. Conclusions The results indicated SNP rs12460528 is a potential locus related to NLRP12 in psoriasis. And allele G had a protective effect on psoriasis. NLRP12 was significantly associated with psoriasis and may play a positively regulating role in psoriasis.

患者女, 72岁, 因双下肢水肿性红斑1月余, 新发水疱10 d, 于2020年7月就诊。患者于2020年6月初出现双下肢水肿, 臀部及双股部屈侧、双膝关节对称性暗红斑片, 在当地医院住院治疗, 住院期间完善检查, 诊断为"低蛋白血症, 轻度贫血, 湿疹", 给予人血白蛋白、脂肪乳等营养支持, 抗组胺药物口服, 糖皮质激素药膏外涂, 皮疹变暗并留色素沉着斑后出院。6月底双下肢及足背再次出现凹陷性水肿, 双足底、足趾出现疼痛性水疱伴渗液, 双小腿内侧对称性暗红斑片。既往史:2010年行胃癌根治术(毕Ⅱ式吻合)。体检:贫血貌, 双侧腋下及腹股沟未触及浅表淋巴结肿大。皮肤科检查:双足底、足趾弥漫性大疱伴渗液, 足趾水疱尼氏征阴性;双足背、踝关节及双下肢屈侧对称分布淡红斑片, 表面散在水疱, 部分斑片中央陈旧呈暗褐色, 表面鳞痂(图1A);双下肢凹陷性水肿;双股部屈侧及臀部褐色色素沉着斑。实验室检查:红细胞2.59 × 1012/L、血红蛋白84 g/L(参考值115 ～ 150 g/L, 下同)、红细胞比容0.258 L/L(0.35 ～ 0.45 L/L);生化检查示钾3.4 mmol/L(3.5 ～ 5.3 mmol/L)、钙1.83 mmol/L(2.11 ～ 2.52 mmol/L)、总蛋白42.1 g/L(65 ～ 85 g/L)、白蛋白23.0 g/L(40 ～ 55 g/L)、球蛋白19.1 g/L(20 ～ 40 g/L)、葡萄糖3.4 mmol/L(3.9 ～ 6.1 mmol/L)、磷0.82 mmol/L(0.85 ～ 0.51 mmol/L)、铜0.5 μmol/L(12.6 ～ 24.4 μmol/L)、锌3.6 μmol/L(10.7 ～ 17.5 μmol/L)、未饱和铁结合力1.4 μmol/L(34.7 ～ 48 μmol/L)、总铁结合力11.6 μmol/L(46.5 ～ 65.7 μmol/L);尿粪常规、肝肾功能、自身抗体谱、甲型肝炎、乙型肝炎、梅毒、艾滋病相关检查均未见异常。左下肢皮损组织病理:表皮角化不全, 角层下水疱, 表皮角质形成细胞坏死, 表皮上部淡染, 真皮浅层水肿, 真皮乳头毛细血管扩张伴红细胞外溢, 血管周围淋巴细胞浸润(图1B)。直接免疫荧光显示IgG、IgM、IgA、补体C3均阴性, 间接免疫荧光显示BP180、Dsg1、Dsg3均阴性。腹部CT示:胃癌术后化疗后改变, 肝、肾、胰腺、脾脏及双肾CT平扫未见明显异常。

Background The frequent coexistence of obesity and metabolic syndrome in patients with Androgenetic alopecia (AGA), may indicate a common pathogenetic pathway with adipokines being a possible implicating cytokine. Objective This study was conducted to investigate the changes in serum levels of adipokines, insulin resistance, vitamin D status and their relationship with AGA, and the relationship between serum levels of adipokines and insulin resistance. Methods 80 male patients with AGA were selected as the experimental group and 60 healthy males served as the control group. Both the AGA group and healthy control group were divided into 2 groups according to the presence or absence of insulin resistance (IR): the IR group and the NIR group. Serum levels of leptin, adiponectin, resistin, visfatin, insulin and 25(OH)D were evaluated in all subjects. Results Compared with the control group, AGA patients showed higher serum levels of leptin and lower adiponectin/leptin (Adpn/Lep) ratio (P<0.05), and both were positively correlated with the severity of the disease. Compared with the AGA NIR group, serum leptin levels were increased in the AGA IR group (P<0.05). AGA IR group and AGA NIR group possessed lower Adpn/Lep ratio when compared with the healthy IR group and healthy NIR group respectively (P<0.05). The multi-factor logistic regression analysis results showed decreased Adpn/Lep level and increased leptin level as risk factors for AGA. AGA Patients had lower vitamin D levels than healthy controls (P<0.05). Conclusion Patients with AGA show an imbalance between pro- and anti-inflammatory adipokines, and probably be involved in AGA pathogenesis. Insulin resistance may influence levels of adipokines, but the present findings cannot indicate insulin resistance plays a role in the onset of AGA. The insufficiency and deficiency of vitamin D are common health concern in our subjects and may be involved in the dysfunction of adipocytes and the development of AGA.

Natural killer/T‐cell lymphoma (NKTCL) is an aggressive and malignant condition with a high mortality rate. Prognostic factors may assist to evaluate the outcome of the disease and may also be useful in selecting appropriate therapeutic strategies for patients. The study aims to describe NKTCL in terms of its clinical features, laboratory examinations, and immunophenotypes and to analyze relevance affecting patient survival outcomes. The patients diagnosed as NKTCL in Jinling Hospital from Jan. 2012 to Dec. 2022 were reviewed retrospectively in this study basing on histopathology. The analysis was performed to evaluate overall survival (OS). A total of 125 NKTCL patients were included, which mainly affected male more than female with the onset median age of 51.00 years old (range, 14 85 y). NKTCL commonly affects the nasopharynx and upper aerodigestive tract, intestines, and skin. The median overall survival was 13.00 months (range, 2–156 m), and the 5-year survival rate was 9.8

23岁男性患者,项部、胸部及臀部毛囊性炎性丘疹、结节、脓肿伴瘢痕5年.家族成员有类似皮损表现.皮肤科情况:项部多发炎性丘疹、结节、脓肿,可挤出脓性分泌物,胸部散在红色丘疹、结节,臀部可见脓肿贯通形成窦道,伴白色条索样或萎缩性瘢痕形成.项部皮损组织病理:毛囊周围及真皮上部可见以中性粒细胞为主的混合炎性细胞浸润.基因测序显示NCSTN基因第11外显子第1300位胞嘧啶突变为胸腺嘧啶(c.1300C＞T),导致第434位密码子由CGA(精氨酸)变为TGA(终止密码子).家族中其他患者有相同突变,未患病成员及50名正常对照均未发现上述突变.诊断:反常性痤疮/化脓性汗腺炎.治疗:多西环素100 mg每日2次口服.治疗4周后,患者炎性丘疹及分泌物明显改善,部分皮损留有瘢痕.随访3个月,项部、胸部及臀部皮损较稳定,背部有部分新发炎性丘疹及结节.

Familial hemophagocytic lymphohistiocytosis (HLH) is a rare genetic and life-threatening immunodeficiency disease. Here, we present a 38-year-old male who initially developed multiple annular to irregular erythema accompanied by a fever after COVID-19 vaccination. He was diagnosed with HLH with evidence of leukocytopenia in a full blood test, elevations of ferritin and sCD25, decreased NK cell function, and hemophagocytosis of a bone marrow biopsy specimen. A genetic examination revealed two probable disease-causing heterozygous mutations on UNC13D associated with type 3 familial HLH. A review of the case reports relevant to HLH following COVID-19 vaccination and the cutaneous manifestations of HLH with genetic defects suggests the necessity that individuals with preexisting immune dysregulation or diseases not classified should be cautious about COVID-19 vaccination and reminds clinicians that various recalcitrant skin lesions may be a sign of HLH.

例1 女，71岁，2020年9月8日因全身红疹伴口唇黏膜破溃7 d于东部战区总医院就诊。2020年8月因右膝关节积液伴疼痛曾于外院静脉滴注地塞米松、口服头孢类药物等。9月2日因消化道出血外院予头孢尼西钠、奥美拉唑及输血等治疗，期间躯干、四肢新发红疹，口唇黏膜破溃，伴瘙痒、高热，予地塞米松、马来酸氯苯那敏、异丙嗪等治疗后热退，但皮损未见明显好转，遂至我院急诊科静脉滴注甲泼尼龙40 mg，以Stevens-Johnson综合征（Stevens-Johnson syndrome，SJS）转入皮肤科。高血压病史15年，口服苯磺酸氨氯地平片（1片/d）治疗，无过敏史。9月7日实验室检查：血红蛋白96 g/L（参考值：115 ~ 150 g/L，下同），C反应蛋白66.7 mg/L（0 ~ 8 mg/L），降钙素原0.109 μg/L（0 ~ 0.046 μg/L），脑利钠肽前体1 277 pmol/L（<106.2 pmol/L），总蛋白54.2 g/L（65 ~ 85 g/L），白蛋白28 g/L（40 ~ 55 g/L），尿素7.7 mmol/L（2 ~ 7.1 mmol/L），抗核抗体谱均阴性，补体C3、C4未见异常。生命体征平稳。皮肤科检查：口唇黏膜破溃结痂，面部、躯干、四肢弥漫暗红色丘疹、斑丘疹，部分表面黑痂（图1A、1B）。初步诊断为SJS。治疗：予单次皮下注射50 mg重组人Ⅱ型肿瘤坏死因子受体—抗体融合蛋白（益赛普）及对症支持治疗。用药24 h内未见新发皮损；8 d后面部、躯干四肢皮损明显消退，遗留部分暗红色结痂（图1E、1F）。治疗期间患者血中性粒细胞计数、淋巴细胞计数、总蛋白、白蛋白有回升趋势；C反应蛋白、降钙素原、脑利钠肽前体、尿素呈下降趋势。淋巴细胞计数、C反应蛋白、降钙素原、尿素治疗8 d后恢复正常。治疗期间无高热或其他并发症。治疗16 d，全身皮疹及结痂基本消退，随访至2021年5月未见复发。

Primary cutaneous amyloidosis is limited to the skin without involving any other tissue. Nodular amyloidosis is rare, and atrophic nodular cutaneous amyloidosis is even rarer. We describe the fourth case of atrophic nodular cutaneous amyloidosis by searching PubMed databases. A 52-year-old female presented to our hospital with a 2-year history of orange papules and nodules without subjective symptom on her right abdomen. Review of systems was negative. Atrophic nodular amyloidosis may progress to primary systemic disease in up to 7% of cases. Because our patient had no systemic involvement, she was diagnosed with atrophic nodular cutaneous amyloidosis based on characteristic symptoms and histopathologic examination. Routine follow-up for this patient is necessary to detect any potential disease progression.

Xanthoma disseminatum ( XD ) is a nonfamilial type of normolipidemic mucocutaneous xanthomatosis that belongs to the group of non–Langerhans cell histiocytoses. More than 100 cases of XD have been reported. In this study we report a case of XD in a 4‐year‐old boy with diabetes insipidus ( DI ). This boy is one of the youngest patients ever to present with XD combined with DI .