BACKGROUND: Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited. METHODS: This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed. RESULTS: Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]-3rd quartile [Q3]: 11-44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset ( P  = 0.021) and transitioning to plaque psoriasis ( P  = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1-Q3: 2-41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1-Q3: 13.0-23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP. CONCLUSIONS The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.
Humans ; Male ; Female ; Psoriasis/pathology* ; Adult ; Cross-Sectional Studies ; Adolescent ; Child ; Young Adult ; Quality of Life ; Middle Aged ; China/epidemiology* ; Recurrence ; Risk Factors ; Surveys and Questionnaires ; East Asian People

Objective:To analyze the clinical characteristics of Pneumocystis pneumonia (PCP) in patients with drug-induced hypersensitivity syndrome (DIHS), and to identify factors influencing the development of PCP in patients with DIHS. Methods:Patients with DIHS accompanied by PCP who were hospitalized at Department of Dermatology, Peking Union Medical College Hospital from January 2019 to August 2024 were collected, and their demographic characteristics, clinical manifestations, laboratory tests, and treatment data were retrospectively analyzed. A matched control group of DIHS patients without PCP was selected based on sex, age (± 3 years), and disease severity at a ratio of 2∶1. Comparisons between groups were made using the independent samples t-test, Mann-Whitney U test, and Fisher's exact test. Results:Eight patients with DIHS accompanied by PCP were enrolled, aged 62.38 ± 20.74 years, including 5 males and 3 females. All DIHS cases were categorized as severe, and high-dose corticosteroids were applied in the treatment of DIHS, with 7 cases additionally receiving intravenous immunoglobulins, immunosuppressants, biologic agents, or Janus kinase inhibitors. During the corticosteroid tapering, 8 patients experienced recurrences of DIHS, and 6 patients exhibited reactivation of viruses, with cytomegalovirus (CMV) as the most common virus. All the patients presented with cough and dyspnea when PCP occurred, and the diagnosis of PCP was confirmed by the presence of Pneumocystis jirovecii DNA in sputum or bronchoalveolar lavage fluid. Seven patients improved with anti-PCP treatment, while one succumbed due to disease progression. Univariate analysis indicated that, compared to DIHS patients without PCP, DIHS patients with PCP received longer treatment with prednisone equivalent > 20 mg/d, had lower peripheral lymphocyte counts, CD4 + T lymphocyte counts, and albumin levels, and had a higher frequency of DIHS recurrence and CMV reactivation during the disease course (all P < 0.05) . Conclusions:Cough and dyspnea are common manufestations of PCP in patients with DIHS. Prolonged use of high-dose corticosteroids, decreased peripheral lymphocytes, decreased CD4 + T lymphocytes, DIHS recurrence, CMV reactivation, and decreased albumin levels may be the influencing factors for concurrent PCP in DIHS patients.

Objective:To analyze the clinical characteristics of Pneumocystis pneumonia (PCP) in patients with drug-induced hypersensitivity syndrome (DIHS), and to identify factors influencing the development of PCP in patients with DIHS. Methods:Patients with DIHS accompanied by PCP who were hospitalized at Department of Dermatology, Peking Union Medical College Hospital from January 2019 to August 2024 were collected, and their demographic characteristics, clinical manifestations, laboratory tests, and treatment data were retrospectively analyzed. A matched control group of DIHS patients without PCP was selected based on sex, age (± 3 years), and disease severity at a ratio of 2∶1. Comparisons between groups were made using the independent samples t-test, Mann-Whitney U test, and Fisher's exact test. Results:Eight patients with DIHS accompanied by PCP were enrolled, aged 62.38 ± 20.74 years, including 5 males and 3 females. All DIHS cases were categorized as severe, and high-dose corticosteroids were applied in the treatment of DIHS, with 7 cases additionally receiving intravenous immunoglobulins, immunosuppressants, biologic agents, or Janus kinase inhibitors. During the corticosteroid tapering, 8 patients experienced recurrences of DIHS, and 6 patients exhibited reactivation of viruses, with cytomegalovirus (CMV) as the most common virus. All the patients presented with cough and dyspnea when PCP occurred, and the diagnosis of PCP was confirmed by the presence of Pneumocystis jirovecii DNA in sputum or bronchoalveolar lavage fluid. Seven patients improved with anti-PCP treatment, while one succumbed due to disease progression. Univariate analysis indicated that, compared to DIHS patients without PCP, DIHS patients with PCP received longer treatment with prednisone equivalent > 20 mg/d, had lower peripheral lymphocyte counts, CD4 + T lymphocyte counts, and albumin levels, and had a higher frequency of DIHS recurrence and CMV reactivation during the disease course (all P < 0.05) . Conclusions:Cough and dyspnea are common manufestations of PCP in patients with DIHS. Prolonged use of high-dose corticosteroids, decreased peripheral lymphocytes, decreased CD4 + T lymphocytes, DIHS recurrence, CMV reactivation, and decreased albumin levels may be the influencing factors for concurrent PCP in DIHS patients.

Glucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body. A skin biopsy was performed and resulting features matched with NME, whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon. After the diagnosis was confirmed, the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days. Meanwhile, we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians, including clinical manifestations, laboratory and imaging examinations, diagnosis and treatments.

Authenticity verification is a very important aspect of medical device registration quality management system verification of medical device. How to verify the authenticity of samples is a problem worth discussing. This study analyzes the methods of authenticity verification from the aspects of product retention sample, registration inspection report, traceability of records, hardware facilities and equipment. In order to provide reference for relevant supervisors and inspectors in the verification of registration quality management system.

Growth hormone deficiency (GHD) has become a serious healthcare burden, and presents a huge impact on the physical and mental health of patients. Here, we developed an actively separated microneedle patch (PAA/NaHCO₃-Silk MN) based on silk protein for sustained release of recombinant human growth hormone (rhGH). Silk protein, as a friendly carrier material for proteins, could be constructed in mild full-water conditions and ensure the activity of rhGH. After manually pressing PAA/NaHCO₃-Silk MN patch to skin for 1 min, active separation is achieved by absorbing the interstitial fluid (ISF) to trigger HCO₃ ^‒ in the active backing layer to produce carbon dioxide gas (CO₂). In rats, the MN patch could maintain the sustained release of rhGH for more than 7 days, and produce similar effects as daily subcutaneous (S.C.) injections of rhGH in promoting height and weight with well tolerated. Moreover, the PAA/NaHCO₃-Silk MN patch with the potential of painless self-administration, does not require cold chain transportation and storage possess great economic benefits. Overall, the PAA/NaHCO₃-Silk MN patch can significantly improve patient compliance and increase the availability of drugs, meet current unmet clinical needs, improve clinical treatment effects of GHD patients.

Pyroptosis, a form of cell death associated with inflammation, is known to be involved in diabetic nephropathy (DN), and discoid domain receptor 1 (DDR1), an inflammatory regulatory protein, is reported to be associated with diabetes.However, the mechanism underlying DDR1 regulation and pyroptosis in DN remains unknown. We aimed to investigate the effect of DDR1 on renal tubular epithelial cell pyroptosis and the mechanism underlying DN. In this study, we used high glucose (HG)-treated HK-2 cells and rats with a single intraperitoneal injection of streptozotocin as DN models. Subsequently, the expression of pyroptosis-related proteins (cleaved caspase-1, GSDMD-N, Interleukin-1β [IL-1β], and interleukin-18 [IL-18]), DDR1, phosphorylated NF-κB (p-NF-κB), and NLR family pyrin domain-containing 3 (NLRP3) inflammasomes were determined through Western blotting. IL-1β and IL-18 levels were determined using ELISA. The rate of pyroptosis was assessed by propidium iodide (PI) staining. The results revealed upregulated expression of pyroptosisrelated proteins and increased concentration of IL-1β and IL-18, accompanied by DDR1, p-NF-κB, and NLRP3 upregulation in DN rat kidney tissues and HG-treated HK-2 cells. Moreover, DDR1 knockdown in the background of HG treatment resulted in inhibited expression of pyroptosis-related proteins and attenuation of IL-1β and IL-18 production and PI-positive cell frequency via the NF-κB/NLRP3 pathway in HK-2 cells. However, NLRP3 overexpression reversed the effect of DDR1 knockdown on pyroptosis. In conclusion, we demonstrated that DDR1 may be associated with pyroptosis, and DDR1 knockdown inhibited HG-induced renal tubular epithelial cell pyroptosis. The NF-κB/NLRP3 pathway is probably involved in the underlying mechanism of these findings.

Objective: To evaluate the application value of ultrasound and dermoscopy in the precise preoperative evaluation of basal cell carcinoma (BCC) , and to analyze the association of high-frequency ultrasound and dermoscopic findings with pathological recurrence risk of BCC. Methods: Clinical data were collected from 33 outpatients with confirmed BCC in the Department of Dermatology, Peking Union Medical College Hospital between April 2016 and December 2018, and high-frequency ultrasonographic and dermoscopic findings from 36 BCC lesions were analyzed. The lesions were classified into high-risk and low-risk groups based on pathological findings. Statistical differences in ultrasound and dermoscopic characteristics between high-risk and low-risk BCC groups were analyzed by using Fisher′s exact test, and the correspondence between high-frequency ultrasonographic and dermoscopic features of BCC was analyzed by calculating the simple matching coefficient. Results: Of the 36 BCC skin lesions, 4 were high-risk lesions and 32 were low-risk lesions. Ultrasonographic features of the high-risk and low-risk lesions overlapped markedly, and no significant differences were observed between the high-risk and low-risk lesions with regard to the shape, boundary, internal echo, hyperechoic spots, or posterior echo (all P > 0.05) . However, 24 (75.0%) low-risk lesions were confined to the dermis, whereas 4 high-risk lesions involved the subcutaneous tissue, and there was a significant difference between the high-risk and low-risk BCC groups with regard to the distribution of BCC (P = 0.008) . In 5 BCC lesions, ultrasound could identify small easy-to-ignore lesions or deep and invisible lesions besides obvious lesions. There were no significant differences in dermoscopic features between high-risk and low-risk groups. However, none of spoke-wheel area, milky-red structureless area, milia-like cysts, comedo-like openings and rainbow pattern was observed in 4 high-risk BCC lesions. The simple matching coefficient between enhanced hyperechoic spots in the lesion observed by ultrasound and milia-like cysts under a dermoscope was 36.1%, and the simple matching coefficient between discontinuous hyperechoic echo in the epidermis on ultrasonography and ulcer/erosion under a dermoscope was 75.0%. Conclusion High-frequency ultrasound and dermoscopy both provide important information for preoperative evaluation of risk of BCC lesions, and high-frequency ultrasound can identify easy-to-ignore hidden lesions in clinical practice.

Objective To analyze the disease constitution,accuracy of clinical and pathological diagnoses of skin biopsy samples in Peking Union Medical College Hospital.Methods A total of 29987 patients subjected to skin biopsy were collected from Department of Dermatology,Peking Union Medical College Hospital from June 2010 to November 2018,and clinical and histopathological diagnoses of these skin biopsy samples were analyzed retrospectively.Results According to the results of histopathological diagnosis,confirmed diagnoses of these patients could be classified into 33 categories and 242 kinds.Common disease categories included epidermal tumors (2931 cases,9.77％),connective tissue diseases (2809 cases,9.37％),melanocytic tumors (2078 cases,6.93％),erythematous scaly pustular dermatoses (1376 cases,4.59％),lichenoid dermatoses (1291cases,4.31％),allergic or eczematous skin diseases (1282 cases,4.28％)and infectious skin diseases (1156 cases,3.86％).Common skin diseases included scleroderma (1887 cases,6.29％),pigmented nevus (1755 cases,5.85％),seborrheic keratosis (1136 cases,3.79％),eczema (1089 cases,3.63％),psoriasis (881 cases,2.94％),lichen planus (867 cases,2.89％),lupus erythematosus (638 cases,2.13％),pemphigus (549 cases,1.83％),and basal cell carcinoma (501 cases,1.67％).Poor consistency was observed between clinical diagnosis and histopathological diagnosis of lichen planus,bullous pemphigoid,granuloma annulare and hypereosinophilic dermatitis.Conclusions Common disease categories of the skin biopsy samples in Peking Union Medical College Hospital were epidermal tumors,connective tissue diseases,melanocytic tumors,erythematous scaly pustular dermatoses,lichenoid dermatoses,and allergic or eczematous skin diseases.Poor consistency was observed between clinical and pathological diagnosis in some skin diseases,and understanding of these diseases should be improved.

The COVID-19 outbreak may have some impact on the use of biologics in psoriatic patients because immunosuppressive effects of biologics may potentially alter the susceptibility of patients to the virus, deteriorate the condition of infected patients or even change the prognosis of infection. According to currently available recommendations from international psoriasis academic organizations and specialists, as well as specific situation in China, the authors provide some guidance on the use of biologics for psoriatic patients undergoing or planning to undergo treatment with biologics, those with low or high risk of infection, and for those with or without COVID-19 infection, so as to provide references for clinical practice.