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Erythrokeratodermia variabilis et progressiva (EKVP) is a rare genodermatosis of clinical and genetic heterogeneity, characterized by the manifestations of localized or disseminated persistent hyperkeratotic plagues and stationary to migratory transient erythematous patches. The majority of EKVP cases display an autosomal dominant mode of inheritance with incomplete penetrance, although recessive transmission has also been described. Mutations associated with EKVP have been primarily detected in connexin (Cx) genes. We herein reported a Chinese sporadic case of late-onset EKVP with a novel heterozygous missense mutation c.109G>A (p.V37M) in GJB4 (Cx30.3) gene, which resulted in a significant reduction of GJB4 expression in the epidermis of the patient. In accordance, while wild-type GJB4 localized at the cell membrane of HeLa cells forming intercellular junctions and intracellular puncta, V37M mutant variant was diffusely expressed within HeLa cells at a considerably lower level. Our findings reveal an essential role of GJB4 in the pathogenesis of EKVP and provides insights into the therapeutic potential of the disease.

Background Atopic dermatitis is a chronic, inflammatory condition causing a substantial burden to patients and caregivers. SHR0302 is an oral, highly selective, Janus kinase 1 inhibitor under investigation for inflammatory skin diseases. Objective The aim of this study was to investigate the efficacy and safety of SHR0302 in Chinese patients with moderate to severe atopic dermatitis. Design and Setting A randomized, double-blind, placebo-controlled, multicenter, phase II trial was conducted in China between October 2019 and August 2020. Participants Patients (n = 105) aged 18–75 years with moderate to severe dermatitis and nonresponsive or intolerant to topical or conventional systemic treatments were included. Interventions Patients were randomly assigned in a ratio of 1:1:1 to receive SHR0302 4 mg once daily, SHR0302 8 mg once daily, or placebo for 12 weeks. Main Outcome Measures The primary efficacy endpoint was the proportion of patients achieving Investigator’s Global Assessment (IGA) response (IGA of 0 [clear] or 1 [almost clear] with improvement of ≥2 grades) at week 12. Secondary efficacy assessments included Eczema Area and Severity Index (EASI) and pruritus Numerical Rating Scale (NRS) scores. Results At week 12, IGA response was achieved in nine patients (25.7%; 90% confidence interval [CI] 13.6–37.9%; p = 0.022) in the SHR0302 4 mg group, 19 patients (54.3%; 90% CI 40.4–68.1%; p

Little real-work data regarding the efficacy and safety of dupilumab in the treatment of atopic dermatitis (AD) is available at present. To assess the efficacy and safety of dupilumab at 12 weeks in the treatment of AD in clinical routine clinical practice. A retrospective, single-centre study of adult patients with moderate to severe AD treated with dupilumab for 12 weeks in China. In total, 60 patients (48 male, 12 female; mean age: 53.2 ± 15.6) were enrolled in this retrospective study. These patients exhibited a mean AD disease course of 10.6 years (6.0), 30% exhibited a family history of allergies, and 31 (51.7%) had one or more allergic comorbidities. Following dupilumab treatment for 12 weeks, 83.3% and 42% of patients had achieved EASI-50 and EASI-75, respectively. Overall, adverse events (AEs) were reported by 15% of patients, with the most common being conjunctivitis, injection site reactions, and herpes simplex virus infections. Laboratory testing after 12 weeks revealed pronounced decreases in both circulating eosinophil counts (from 0.6 (0.1-2.8) to 0.3 (0.1-9.7) 10

The objective of this study was to evaluate optimal treatment regimen of 308-nm excimer laser for palmoplantar pustulosis (PPP). 77 patients with PPP were randomly assigned to receive low dose (2-fold of MED as initial dose), medium dose (4-fold of MED as initial dose) and high dose group (6-fold of MED as initial dose) and the MED of each patient depended on the ultraviolet light sensitivity of individual's skin which ranged from 0.1 to 0.25 J/cm2 . All group received 308-nm excimer laser treatment three times weekly for 8 weeks. Clinical evaluation based on the Palmoplantar Pustular Psoriasis Area and Severity Index (PP-PASI) and Dermatology Life Quality Index (DLQI) score. All treatment groups achieved satisfied efficacy at the end of the treatment period with more obvious reduction of PP-PASI score in high dose group (16.05 ± 4.26) than low and medium dose group (23.67 ± 7.16, p < 0.01; 22.04 ± 5.74, p < 0.01). Improvement of DLQI score was greatest at week 4 for all patients in each group, while DLQI improved more quickly in high/medium dose group than low dose group. Adverse effects of erythema, blistering and erosions were more common with the higher dose regimen. High dose of 308-nm excimer laser could achieve a better efficacy in PPP treatment, reduce the severity of the disease in patients and improve the life quality of patients. Meantime, the incidence of adverse reactions should be aware of and it's necessary to evaluate the skin and lesion type before the dose selection.

Generalized pustular psoriasis is an immune‐mediated dermatologic condition characterized by widespread, sterile, subcorneal pustules. However, limited information exists regarding the clinical course of generalized pustular psoriasis. This study aimed to examine the precipitating factors, clinical manifestations, laboratory data, relapse patterns, and prognosis of generalized pustular psoriasis at our hospital and to improve the diagnosis and treatment. A retrospective analysis was conducted for generalized pustular psoriasis in our department from 2014 to 2019. In total, 110 patients were included in our study (mean age 46.5 years). The female:male ratio was 1:2.7. Ninety‐four (85.5%) had a psoriasis vulgaris history, 12 (10.9%) had a psoriatic arthritis history, five (4.5%) had an erythrodermic psoriasis history, and 16 (14.5%) had a family history of psoriasis. Eleven (10.0%) cases were triggered by infections and 17 (15.5%) were caused by the sudden discontinuation of systemic drugs. During hospitalization, the proportion of patients with hyperlipidemia was higher after acitretin treatment than before acitretin treatment (P 0.05). The onset age of generalized pustular psoriasis was younger in patients without prior psoriasis (P 0.05). Moreover, among patients with fever, skin lesion clearance rates were highest in the biological agent group (81.8%). However, among patients without fever, skin lesion clearance rates were highest in the acitretin group (86.7%). No patients presented serious complications or died. Our study presents the detailed clinical course of generalized pustular psoriasis in Chinese patients. These results will help to better understand and treat generalized pustular psoriasis.

Introduction Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous drug reaction. Bullous pemphigoid (BP) is an acquired autoimmune disease that might be associated with drugs. There is currently no report of tripterygium glycosides (TG)-induced AGEP-like lesions combined with BP. Case presentation A 66-year-old male with a 20-year history of psoriasis was prescribed oral TG at 1 mg/kg, three times a day, due to aggravated psoriasis. Seven days later, erythemas and blisters appeared. After another 3 days, there were two types of blisters: (1) numerous small tension blisters with a lot of neutrophils on the top similar to AGEP combined with BP; (2) a BP. After intravenous injection of methylprednisolone and gamma globulin, the lesions were controlled. Conclusion This patient developed two types of lesions，including one similar to AGEP combined BP (AGEP-like) and a BP. It's a rare drug reaciton induced by TG.

Psoriasis is a chronic, systemic disease that requires long-term management. Biologic agents have been used widely against psoriasis, such as infliximab. We analyzed the survival and discontinuation rates of infliximab when treating psoriasis under real-world conditions in China. Patients with moderate-to-severe psoriasis treated with infliximab in Shanghai Skin Disease Hospital from January 2015 to April 2020 were included in our retrospective study. Information from their medical records (clinical characteristics, Psoriasis Area Severity Index [PASI] score, laboratory results, and time of discontinuation) was collected through the Shanghai Skin Disease Hospital database. The survival of infliximab was assessed with Kaplan-Meier plots and multivariate Cox regression. Forty-two patients who underwent treatment were assessed retrospectively (38.1% had been diagnosed with psoriatic arthritis [PsA]). The discontinuation rate was 57.1%, the mean survival time of discontinuation was 57 weeks for patients with PsA vs 69 weeks for those without PsA (P = .5993). The cholesterol level (P = .003) and lymphocyte percentage (P = .010) were associated with longer survival of infliximab according to Cox regression analysis. Our study revealed that infliximab had a similar drug survival as previous studies, the high cholesterol level and lymphocyte percentage might function as negative predictor for infliximab persistence.

is missing (Short Communication)

Psoriasis is a common chronic skin disease characterized by epidermal proliferation and inflammation. Pustular psoriasis (PP) is one of the most serious and refractory. The number, differentiation, and function of B10 cells in patients with PP were analyzed, and the relationship between B10 cells and PP, an autoimmune disease, was explored. We established an Imiquimod psoriasis mouse model and subcutaneously injected B10 cells as treatment. We found that the proportion of B10 cells in the peripheral blood of patients with PP was lower than that of the normal controls. However, the number of B10 precursor cells increased. B10 cells in the peripheral blood may be mobilized to effector sites, such as the skin. In patients with PP, B10 cells do not display evident developmental disorders under the CD40 and TLR9 pathways. Normal human B10 cells were found to inhibit the secretion of IFN-γ and TNF-α by lymphocytes significantly. Whereas the function of B10 cells in patients with PP is impaired, and the inhibition is not apparent. Treatment with a B10 cells injection displays a certain therapeutic effect on PP. This study enriched the etiology and pathogenesis of PP. It provides a foundation for cell therapy for the treatment of autoimmune diseases.

Background The Dangguikushen (DGKS) pill is a proprietary traditional Chinese medicine that has shown superior efficacy in the treatment of acne vulgaris for many years. Methods A network pharmacology-based analysis was performed to explore the potential anti-acne compounds, core therapeutic targets, and the main pathways, involved in the DGKS pill bioactivity. The matching results between the predicted targets of the DGKS pill and the well-known targets of acne vulgaris were collected, followed by network establishment using protein-protein interaction (PPI) data. Cytoscape was utilized to analyze the network and screen the core targets. Furthermore, the Database for Annotation, Visualization and Integrated Discovery (DAVID), and ClueGO were used for the enrichment analysis of the Kyoto Encyclopedia of Genes and Genomics (KEGG) pathways and Gene Ontology biological processes (GO-BP). Finally, the "compound-target-pathway" network was constructed. Results This approach identified 19 active compounds, 46 therapeutic targets and 12 core therapeutic targets of the DGKS pill. The biological processes were primarily related to reactive oxygen species (ROS) metabolic process, gland morphogenesis, and female gonad development. The DGKS pill was significantly associated with eight pathways including the PI3K-Akt, TNF, NF-kappa B, and p53 signaling pathways. Conclusion DGKS pill might have a synergistic effect on the inhibition of excessive sebaceous lipogenesis and sebocyte differentiation, and likewise, anti-inflammatory effects via the different signaling pathways (PI3K-Akt, TNF, NF-kappa B, and p53). This article is protected by copyright. All rights reserved.

Juvenile generalized pustular psoriasis is a rare, severe type of psoriasis that can be life-threatening. Thus far, treatment for juvenile generalized pustular psoriasis has been challenging, and no standardized guidelines are available. Here, we describe two Chinese boys with juvenile generalized pustular psoriasis who were successfully treated with infusions of infliximab. During 12 months of follow-up, no recurrence or exacerbation was observed in either patient. Herpes zoster was observed as an adverse effect in one patient, following the initial infusion of infliximab; the other patient did not experience any adverse reaction. Although infliximab is effective therapy for patients with juvenile generalized pustular psoriasis, there is a need for close monitoring of adverse effects in these patients.

The association of psoriasis with thyroid dysfunction has been investigated. However, it remains unclear; some papers indicate it, and others do not. In this study, we evaluate the prevalence of thyroid dysfunction in patients with psoriasis vulgaris (PsV), psoriatic arthritis (PsA), generalized pustular psoriasis (GPP) and erythrodermic psoriasis (EP), and the association of thyroid dysfunction with inflammation. Data on 201 psoriatic patients visiting our hospital from January 2014 to November 2017 (159 men and 42 women; 74 PsV, 42 PsA, 38 GPP and 47 EP) were retrospectively analyzed. Thirty-three percent of psoriatic patients had thyroid dysfunction. The percentage of patients with thyroid dysfunction was the highest in those with EP (60% EP, 42% GPP, 19% PsA, 19% PsV). The prevalence of thyroid dysfunction decreased significantly when patients switched from EP to PsV or PsA (58% vs 17%; median, 20.5; range, 4-65 months). Most of the patients with thyroid dysfunction had low thyroxine syndrome (serum levels of free thyroxine are low, but serum thyroid-stimulating hormone level is normal). Patients with thyroid dysfunction demonstrated significantly higher CD3+ and CD4+ T-cell absolute count levels than those without thyroid dysfunction. Meanwhile, patients with thyroid dysfunction demonstrated lower immunoglobulin (Ig)A and IgM levels than those without thyroid dysfunction. Finally, patients with thyroid dysfunction demonstrated higher elevated serum C-reactive protein levels than those without dysfunction in total, although there were no statistical differences. Our data indicate that thyroid dysfunction in patients with psoriasis may be associated with inflammation caused by psoriasis.

To compare the efficacy and safety of UVA1 and narrowband UVB (NB-UVB) therapy in the treatment of scalp psoriasis. Patients with scalp psoriasis were randomly assigned to either UVA1 or NB-UVB therapy. Both treatments were performed three times weekly for 6 weeks. Clinical efficacy was evaluated by using Psoriasis Scalp Severity Index (PSSI), and patient-reported quality of life (QoL) was assessed by Dermatology Life Quality Index (DLQI). Totally 68 patients completed the study. Both UVA1 and NB-UVB phototherapy achieved a statistically significant reduction of PSSI and DLQI scores at the end of the treatment period. Compared with the NB-UVB group, the significantly greater improvements occurred in UVA1 treatment group at week 3, although differences declined thereafter through week 10. Both UVA1 and NB-UVB therapy were well-tolerated in this study, and the occurrence of adverse events (AEs) was uncommon. Both UVA1 and NB-UVB phototherapy could offer relief of scalp symptoms in patients with scalp involvement. Furthermore, UVA1 treatment could improve the clinical manifestations and QoL more quickly than NB-UVB therapy.

Acute generalized exanthematous pustulosis, characterized by subcorneal or superficial intraepidermal pustules, is induced by drugs in more than 90% of cases. Psoriasis is an autoimmune disease triggered by different conditions in genetically susceptible people. Generalized pustular psoriasis is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this report the authors have reported a 40-year-old male patient with primary syphilis who developed generalized pustular dermatosis after the use of ceftriaxone. On the third day after ceftriaxone treatment, complete regression of the syphilis lesions was reached. While on the sixth day, erythematous pustular lesions accompanied with fever were observed on the whole body. A personal history of psoriasis and histopathological findings with psoriasiform changes and subcorneal pustule favored the diagnosis. After discontinuation of ceftriaxone, the patient’s condition slowly improved until he had plaque-type psoriasis 3 weeks later. A heterozygous c.115 + 6T>C missense substitution of IL36RN related to the pathogenesis of acute generalized exanthematous pustulosis/generalized pustular psoriasis was identified.

Liver test abnormalities (LTA) are a frequent extracutaneous manifestation in generalized pustular psoriasis (GPP). Due to possible hepatotoxicity of systemic monotherapy, it is challenging to simultaneously achieve clinical remission and LTA normalization. However, evidence for therapy is lacking. The aim of this study was to assess the effectiveness of combination therapy of acitretin and glycyrrhizin in nine GPP patients with LTA. During the acute phase of GPP, a combination of acitretin (0.5 mg/kg/d PO) and glycyrrhizin (80 mg/d intravenous) was initiated. After 2 weeks, all the patients promptly achieved at least 77% improvement in the severity score of GPP, as well as a significant reduction of liver enzymes. The patients were continuously treated with tapered doses of acitretin (20-30 mg/d PO) and glycyrrhizin (150 mg/d PO), and presented stable conditions during the 12-month follow-up. In conclusion, we consider that the combination of acitretin plus glycyrrhizin is an effective and safe therapy in GPP patients with LTA.

Psoriasis and pemphigus are clinically well-characterized chronic, inflammatory skin diseases. Many case reports have described the coexistence of psoriasis and bullous pemphigoid. However, the present report is about a rare case of pemphigus vulgaris in a patient with psoriasis vulgaris. We had a 68-year-old male psoriatic patient who developed blisters lesions and erosions on the trunk and extremities. The histopathology of a blister lesion showed the intraepidermal blisters that contained serous fluid and inflammatory cells. Both of desmoglein core protein 1 antibody and desmoglein core protein 3 antibody were detected. Diagnoses of pemphigus vulgaris and psoriasis vulgaris were made. The patient was treated with methotrexate (12.5 mg/week) and methylprednisone (16 mg/day) after his admission. Two weeks after admission, the patient's lesions gradually subsided. This case reminds us that the therapeutic effect of pemphigus vulgaris may be related to the incidence of psoriasis.

While infliximab has been shown to be paradoxically associated with the development of pustular psoriasis in patients with rheumatoid arthritis, spondyloaripathies, juvenile idiopathic, and inflammatory bowel disease, there are few cases of pustular psoriasis induced by infliximab in patients with psoriasis. We here present a 55-year-old female patient with longstanding plaque psoriasis and psoriatic arthritis who developed generalized pustular psoriasis 1 month after the fifth infusion of infliximab. Given the lack of other side effects and the rapid initial response of the underlying psoriatic arthritis, we opted against discontinuing infliximab therapy, and the sixth infusion of infliximab was administered 10 days ahead of schedule. Topical corticosteroids were added for the management of pustular lesions on initial presentation. One week after the sixth infusion, the pustular psoriatic lesions almost completely disappeared. No recurrence of pustular psoriasis was observed during the 3-month follow-up. Our experience shows that pustular lesions associated with infliximab can be successfully managed with topical corticosteroids without discontinuing infliximab therapy or compromising therapeutic benefit seen upon the underlying condition.

Porphyria cutanea tarda (PCT) is a condition that affects liver and skin by reduction of hepatic uroporphyrinogen decarboxylase activity. It is characterized by blistering lesions, erosions and crusts on sun-exposed areas. We report a 51-year-old male presenting with recurrent episodes of bullae, erosions, and crust on his neck and dorsum of the hands for 3 months. Aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transferase, alkaline phosphatase, lactate dehydrogenase levels, as well as total plasma porphyrin and urinary uroporphyrin levels were elevated. Based on the clinical manifestations, the history and laboratory findings, a diagnosis of PCT was made. The cutaneous and biochemical abnormalities of the patient improved with therapy of glycyrrhizin.

Miliaria crystallina is a skin disorder that often erupts in the process of febrile diseases or under hot and humid climatic conditions. Toxic epidermal necrolysis (TEN) is a rare, acute, and life-threatening mucocutaneous disease with a mortality rate of 25-35%. There has been no inevitable connection between the two diseases among previously reported cases, but we observed a case of secondary miliaria crystallina a woman with herbal remedies-induced TEN during the therapeutic process.

Infliximab is a tumor necrosis factor-alpha (TNF-a) inhibitor widely used in the treatment of moderate to severe chronic plaque psoriasis. Here, we report a case of vitiligo following infliximab administration in a patient with chronic plaque psoriasis. The case serves as a reminder of vitiligo induced by TNF-a-antagonist therapy.

Palmoplantar pustulosis (PPP) is recalcitrant to traditional topical and systemic therapies. Ultraviolet A1 (UVA1) phototherapy, a new therapeutic approach, has recently been shown good efficacy in the treatment of PPP. The purpose of this study was to evaluate the efficacy and safety of UVA1 therapy for the treatment of PPP. Patients with PPP were treated with UVA1 irradiation three times a week for up to 30 sessions and had a 3-month follow-up visit. UVA1 therapy was conducted with a fixed dose (80 J/cm2). Clinical evaluation was based on the Palmoplantar Pustular Psoriasis Area and Severity Index (PPPASI) score. Totally, 62 patients completed the study. The mean PPPASI score decreased from a baseline value of 9.4 ± 2.8 to a value of 4.9 ± 2.4 at 15 sessions, 1.7 ± 1.9 at 30 sessions, and 2.0 ± 2.1 at follow-up visit. A reduction of 75 % in the PPPASI score was observed in 4 (6.5 %) patients at 15 sessions and 45 (72.6 %) patients at 30 sessions. The adverse effects were limited including burning sensation, pruritus, and hyperpigmentation. UVA1 is an effective therapy for PPP with mild side effects.

The present authors report a 12-year-old Chinese child with generalized pustular psoriasis who was responded well to infliximab, but an adverse effect of herpes zoster occurred after the first infusion soon. The antiviral treatment was effective and no recurrence or flaring was observed after half-year of follow-up. This case reminds us to highlight the risk of viral infection during biological treatment on patients with psoriasis or autoimmune disease.

UVA1 phototherapy, a new therapeutic approach, has recently been shown good efficacy in the treatment of palmoplantar pustulosis (PPP). The purpose of this study was to compare the efficacy of UVA1 and narrowband UVB (NB-UVB) therapy in the treatment of PPP. Patients with PPP were randomly assigned to either UVA1 or NB-UVB therapy according to a left-right randomization table. Both treatments were performed three times weekly for up to 30 sessions. Clinical evaluation was based on the Palmoplantar Pustular Psoriasis Area and Severity Index (PPPASI) score. Totally 64 patients completed the study. Both UVA1 and NB-UVB therapy showed a statistically significant reduction of PPPASI score compared with the baseline value at the end of the treatment period (P