Critical dermatological emergencies generally refer to a group of dermatoses characterized by urgent onset, rapid progression, and potential life-threatening complications. This commentary elucidates the clinical characteristics of, diagnostic procedures, and therapeutic strategies for critical dermatological emergencies, including severe cutaneous adverse reactions, autoimmune bullous diseases, and psoriasis, emphasizing the importance of early recognition, prompt diagnosis, standardized treatment, and multidisciplinary collaboration in their management. Furthermore, this commentary provides an in-depth analysis of the current challenges and risks in managing these conditions, and proposes specific improvement measures, such as strengthening primary prevention, optimizing referral systems, and standardizing diagnostic and therapeutic procedures, aiming to provide practical clinical guidance for the management of critical dermatological emergencies.

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.

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

Critical dermatological emergencies generally refer to a group of dermatoses characterized by urgent onset, rapid progression, and potential life-threatening complications. This commentary elucidates the clinical characteristics of, diagnostic procedures, and therapeutic strategies for critical dermatological emergencies, including severe cutaneous adverse reactions, autoimmune bullous diseases, and psoriasis, emphasizing the importance of early recognition, prompt diagnosis, standardized treatment, and multidisciplinary collaboration in their management. Furthermore, this commentary provides an in-depth analysis of the current challenges and risks in managing these conditions, and proposes specific improvement measures, such as strengthening primary prevention, optimizing referral systems, and standardizing diagnostic and therapeutic procedures, aiming to provide practical clinical guidance for the management of critical dermatological emergencies.

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.

In recent years, great progress has been made in the research on the pathogenesis of pruritus, and many new targeted therapeutic drugs have been developed, mainly including small-molecule drugs or biological agents targeting signal transduction pathways involved in the pathogenesis of pruritus, such as neurokinin-1 receptor, tropomyosin-related kinase A, transient receptor potential vanilloid 1, transient receptor potential melastatin 8 channels, interleukin 31, etc. Some of these drugs have shown good efficacy and safety in the treatment of pruritus in a variety of skin diseases, providing more options for patients with pruritus, especially those who are resistant to traditional drug treatment. This review summarizes research progress in the above small-molecule drugs and biological agents targeting signaling pathways involved in pruritus, in order to provide more options for pruritus treatment.

Objective:To investigate the effect of narrow-band ultraviolet B (NB-UVB) phototherapy on the incidence of cutaneous carcinoma, herpes zoster and cataracts in patients with psoriasis vulgaris.Methods:A telephone follow-up was conducted among patients with psoriasis vulgaris receiving NB-UVB phototherapy at the Department of Dermatology, Peking Union Medical College Hospital from January 1, 2003 to December 31, 2019. The incidence of cutaneous carcinoma, herpes zoster and cataracts was investigated, and their incidence density and 95% confidence intervals ( CIs) were calculated. Subgroup analyses were performed according to the cumulative number of NB-UVB radiation (≤ 100 times, 101 - 300 times, > 300 times), cumulative radiation energy (≤ 100 J/cm 2, > 100 - 500 J/cm 2, > 500 J/cm 2), and average radiation frequency (≤ 1.5 times/week, > 1.5 times/week). Univariate Cox′s proportional hazards regression analysis was conducted to analyze differences in the incidence density of cutaneous carcinoma, herpes zoster, and cataracts between the groups, and the results were expressed as hazard ratios ( HRs) and their corresponding 95% CIs. Results:A total of 160 patients with psoriasis vulgaris completed the follow-up, including 99 males (61.9%) and 61 females (38.1%). Their ages (median [ Q1, Q3]) were 39 (28.3, 54.8) years, the cumulative numbers of NB-UVB radiation were 129.5 (52, 291) times, the cumulative radiation energy was 226.3 (71.1, 688.9) J/cm 2, and the average radiation frequencies were 1.57 (1.37, 1.83) times/week. No cutaneous carcinoma occurred during the follow-up of 1 288.87 person-years (7.77 [5.11, 9.92] years) ; herpes zoster occurred in 4 cases during the follow-up of 1 273.85 person-years (7.68 [5.11, 9.88] years), and the estimated incidence density of herpes zoster was 31.4/10 000 person-years (95% CI: [11.8 - 83.5]/10 000 person-years) ; cataracts occurred in 8 cases during the follow-up of 1 264.67 person-years (7.72 [4.84, 9.83] years), and the estimated incidence density of cataracts was 63.3/10 000 person-years (95% CI: [31.7 - 126.2]/10 000 person-years). No significant differences were observed in the incidence density of herpes zoster or cataracts among subgroups with different cumulative numbers of NB-UVB radiation, cumulative radiation energy and average radiation frequencies (all P > 0.05) . Conclusion:The incidence of herpes zoster or cataracts may not be affected by the cumulative number of NB-UVB radiation, cumulative radiation energy or average radiation frequency in patients with psoriasis vulgaris.

Psoriasis is a chronic inflammatory skin disease with significant physical and psychological burdens. The interplay between the innate and adaptive immune systems is thought to contribute to the pathogenesis; however, the details of the pathogenesis remain unclear. In addition, reliable biomarkers for diagnosis, assessment of disease activity, and monitoring of therapeutic response are limited. Metabolomics is an emerging science that can be used to identify and analyze low molecular weight molecules in biological systems. During the past decade, metabolomics has been widely used in psoriasis research, and substantial progress has been made. This review summarizes and discusses studies that applied metabolomics to psoriatic disease. These studies have identified dysregulation of amino acids, carnitines, fatty acids, lipids, and carbohydrates in psoriasis. The results from these studies have advanced our understanding of: (1) the molecular mechanisms of psoriasis pathogenesis; (2) diagnosis of psoriasis and assessment of disease activity; (3) the mechanism of treatment and how to monitor treatment response; and (4) the link between psoriasis and comorbid diseases. We discuss common research strategies and progress in the application of metabolomics to psoriasis, as well as emerging trends and future directions.
Humans ; Psoriasis/drug therapy* ; Skin/metabolism* ; Biomarkers/metabolism* ; Metabolomics/methods*

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.

Rare skin diseases are various in kinds facing many challenges and difficulties in diagnosis and treatment. Recently, the diagnosis of rare skin diseases has improved continuously. Molecular diagnostic tool, a representation of the next generation sequencing technology, can effectively assist the accuracy of clinical diagnosis for rare diseases. The research and development of orphan drugs and gene therapy have made continuous breakthroughs and progress, bringing hope to patients with rare skin diseases. It is crucial to establish scientific disease management system of the disease, layered referral networks, and the standardized clinical pathways to improve the diagnosis and treatment level of rare skin diseases.This article provides a brief overview of the progress in the diagnosis and treatment of rare skin diseases and explore the future possibilities.