Tofacitinib Treatment for Pretibial Myxedema.

This case report describes tofacitinib treatment for 2 patients with pretibial myxedema.

Bimekizumab for the treatment of moderate-to-severe plaque psoriasis: a meta-analysis of randomized clinical trials.

Background: Bimekizumab is a monoclonal IgG1 antibody that selectively inhibits both interleukin (IL)-17A and IL-17F, and is a promising drug for patients with moderate-to-severe plaque psoriasis. Objectives: This study aimed to assess the efficacy and safety of bimekizumab in treating patients with psoriasis and to determine the optimal maintenance dosing schedules of bimekizumab. Methods and design: Eligible trials were identified from PubMed, Cochrane Controlled Register of Trials, Embase, ClinicalTrials.gov, and Chinese medical databases. Only double-blind, randomized, active comparator, or placebo-controlled trials of bimekizumab treatment on patients with psoriasis were included in this study. Results: Five studies were identified, which included 2473 patients with moderate-to-severe plaque psoriasis. The results indicated that bimekizumab had better efficacy than placebo or active comparator for Psoriasis Area and Severity Index (PASI) 90 [risk ratio (RR) = 29.29, 1.52; 95% confidence interval (CI) = 10.30-83.30, 1.06-2.19], PASI 100 (RR = 59.87, 2.06; 95% CI = 15.06-237.99, 1.12-3.79), and Investigator's Global Assessment scores of 0 or 1 (IGA 0/1) (RR = 21.55, 1.36; 95% CI = 9.25-50.19, 1.02-1.81). Faster onset of clinically meaningful responses was observed with bimekizumab compared with both active comparators (RR = 2.59; 95% CI = 1.32-5.10) and placebo (RR = 40.46; 95% CI = 13.19-124.13), with PASI 75 response observed at week 4 after one dose. Subgroup analysis showed no significant difference in the reduction of PASI scores between 320 mg q4w dosage and q8w dosage (RR = 1.00; 95% CI = 0.96-1.03). Rates of patients with adverse events (AEs) were comparable in the bimekizumab and active comparator groups (RR = 1.13; 95% CI = 1.01-1.26), and oral candidiasis was one of the most common treatment-emergent AEs. Conclusion: The results of this meta-analysis suggest that bimekizumab is more efficacious and has a rapid onset of action than active comparators and placebo in the treatment of moderate-to-severe plaque psoriasis. After 16 weeks of initial maintenance treatment, both bimekizumab maintenance dosing schedules (320 mg every 4 and 8 weeks) had similar efficacy.

Risk factors of cardiovascular involvement in patients with Behcet's disease.

Objectives: Behcet's disease (BD) is a multi-systemic inflammatory vasculitis which may be life-threatening if combined with cardiovascular problems. The aim of the study was to identify potential risk factors associated with cardiovascular involvement in BD. Methods: We reviewed the medical databases of a single center. All BD patients identified as fulfilling the 1990 International Study Group criteria or the International Criteria for Behcet's Disease criteria. Cardiovascular involvement, clinical manifestations, laboratory features, and treatments were recorded. The relationship between parameters and cardiovascular involvement was analyzed. Results: 111 BD patients were included: 21 (18.9%) had documented cardiovascular involvement (CV BD group) and 99 (81.1%) had no cardiovascular involvement (non-CV BD group). Compared with non-CV BD, the proportion of males and smokers were significantly increased in CV BD (p = 0.024 and p < 0.001, respectively). Levels of activated partial thromboplastin time (APTT), cardiac troponin I and C-reactive protein were significantly higher (p = 0.001, p = 0.031, and p = 0.034, respectively) in the CV BD group. Cardiovascular involvement was associated with smoking state, the presence of papulopustular lesions, and higher APTT in multivariate analyzed (p = 0.029, p = 0.021, and p = 0.006, respectively). The ROC curve showed that APTT predicts the risk of cardiovascular involvement (p < 0.01) at a cut-off value of 33.15 s with a sensitivity of 57.1% and specificity of 82.2%. Conclusion: Cardiovascular involvement in BD patients was associated with gender, smoking state, the presence of papulopustular lesions, and higher APTT. All patients newly diagnosed with BD should be systematically screened for cardiovascular involvement.

Clinical characteristics and outcome of elderly onset adult-onset Still's disease: A 10-year retrospective study.

Objective: Our objective was to retrospectively analyze the clinical characteristics and outcome of adult-onset Still's disease (AOSD) patients with elderly onset. Methods: Retrospective data of patients diagnosed with AOSD in our institute during 2013-2021 were analyzed. The diagnoses were based on the Yamaguchi criteria for AOSD. All long-term follow-up data were collected from medical records and phone calls. Results: In total, 281 AOSD patients were enrolled in this study, with the median follow-up interval of 47 months. Thirty-two (11.4%, ≥65 years) AOSD patients were classified into the elderly onset groups. Compared to the younger onset group, the percentage of patients with skin rash (p = 0.047), sore throat (p = 0.001), myalgia (p = 0.001), splenomegaly (p = 0.039), hepatosplenomegaly (p = 0.002) and the Pouchot's score (p = 0.002) were significantly lower in the elderly onset group. The death rate (p = 0.014) of elderly onset group is higher than younger onset group, and the independent risk factors of mortality in all AOSD patients were age at onset (HR: 1.115, p = 0.044), disseminated intravascular coagulation (HR: 391.576, p = 0.001) and pleuritis (HR: 23.162, p = 0.033). The probability of relapse was significantly increased in the patients with macrophage activation syndrome (MAS) compared with the patients without MAS (p < 0.001), though the different age groups of AOSD patients with MAS showed no difference in the probability of relapse (p = 0.737). Conclusion: Elderly onset AOSD patients were distinguished by several distinct clinical features compared to younger onset AOSD patients. The frequency of relapse and complications were similar to that of AOSD patients with elderly or younger onset. A higher mortality rate was observed in elderly onset AOSD patients, and the mortality of AOSD patients was related to age at onset, DIC and pleuritis.

Case Report: Cutaneous Squamous Cell Carcinoma Arising From the Ulcer of the Lesions of Dupuytren's Disease on the Palm.

Dupuytren's disease is a benign fibromatosis that mainly involves the fascia of the palm and digits. The relationship between Dupuytren's disease and the evolution of cutaneous squamous cell carcinoma is still unclear. Here we report the case of a 52-year-old female with squamous cell carcinoma arising from the ulcer of the lesions of Dupuytren's disease on the left palm. To our knowledge, this is the first reported case in the English literature of squamous cell carcinoma on the palm of someone with Dupuytren's disease.

Immune-Mediated Necrotizing Myopathy Initially Presenting as Erythema Nodosum.

Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by severe diffuse proximal myofiber necrosis in the context of inflammatory myopathy. Autoantibodies of anti-signal recognition particle and anti-hydroxy-3-methylglutaryl-CoA reductase are two antibodies specific to IMNM. Erythema nodosum (EN) is often accompanied by various systemic diseases, such as autoimmune diseases. Herein, we report a female patient with signal recognition particle-associated IMNM, with EN as the first presentation. She showed significant clinical improvement after the initiation of glucocorticoids, intravenous immunoglobulin, rituximab, and mycophenolate mofetil. This case indicates that IMNM can initially present as EN. IMNM and EN might have overlapping pathogeneses.

Histopathological diagnosis of persistent pruritic eruptions associated with adult-onset Still's disease.

AIMS: Persistent pruritic eruptions (PPEs), presenting with dyskeratotic keratinocytes histologically, are characteristic skin rash in patients with adult-onset Still's disease (AOSD). The lesions may be histologically similar to other entities that present with dyskeratosis. In the present study, we compared the histopathological features between PPEs and other entities presenting with dyskeratosis. METHODS AND RESULTS: To investigate whether histopathological findings can be used to discriminate among PPEs and other entities presenting with dyskeratotic keratinocytes, cutaneous histopathological changes of PPEs associated with AOSD (n = 26) were compared with those of systemic lupus erythematosus (SLE) (n = 16), dermatomyositis (n = 19), and drug eruption (n = 16). Dyskeratosis was observed in the upper one-third of the epidermal layer in all 26 PPEs. The rate of dyskeratosis for PPEs was higher than that for SLE (18.8%) and dermatomyositis (15.8%). In drug eruptions, the dyskeratotic cells were distributed in all levels of the epidermis. Variable densities of neutrophils were found in the dermis in all PPEs. CONCLUSIONS: Although this was a retrospective study conducted at a single centre, presentation of dyskeratotic keratinocytes in the upper one-third of the epidermal layer is a distinctive histopathological reactive pattern of PPEs. This pattern may be a useful histopathological marker for early diagnosis of AOSD.

[Sporadic cutaneous infections due to nontuberculous mycobacteria: a retrospective study of 37 cases].

OBJECTIVE: To study the clinical and pathological characteristics of sporadic cutaneous infections due to nontuberculous mycobacteria (NTM), and investigate the diagnostic criteria and therapeutic principal. METHODS: Totally 37 cases of sporadic cutaneous infections due to NTM were confirmed in the Department of Dermatology, Peking University People's Hospital from January 2000 to March 2014. The microbiologic and clinical data were reviewed, and their skin biopsy specimens were reassessed. RESULTS: Of all the 37 patients, 30 cases were Mycobacterium marinum infection, 6 were Mycobacterium abscessus infection, and one was Mycobacterium chelonea and Mycobacterium fortuitum infection. Identification of mycobacterial species by analysis of hsp65 gene in tissue DNA was more sensitive than traditional bacterial culture. The most common risk factors were traumatic injuries (21 of 37) and aquarium or fish-related job (21 of 37). One case of Mycobacterium abscessus infection occurred after autologous fat filling. Nodule and plaque were most common lesions in Mycobacterium marinum infection. Twenty-four of the 30 cases of Mycobacterium marinum infection presented with multiple lesions or sporotrichoid spread lesions. Ulceration, papules, abscess, and purulent discharge were observed in cases of Mycobacterium abscessus infection. Infective granuloma was most common histopathological appearance. For the treatment of Mycobacterium marinum infection, rifampin, ethambutol, and clarithromycin were commonly used (combination of two antibiotics, or three antibiotics), with the cure rate 90.00%. Four of the six Mycobacterium abscessus infections cases were cured, and one patient died. CONCLUSION: The most common species of sporadic cutaneous infections due to NTM is Mycobacterium marinum. Traumatic injuries, aquarium or fish-related job, and cosmetic surgeries are common risk factors. Mycobacterium marinum infection often presents with nodules, plaques, and sometimes sporotrichoid spread lesions. Lesions of Mycobacterium abscessus infection may vary. Pathological changes were not species specific, final diagnosis must be made depending on the identification of the microorganism. For the treatment of Mycobacterium marinum infection, excellent outcomes can be achieved by the combination of rifampin and ethambutol, and the combination of clarithromycin and rifampin or ethambutoland. Treatment regimens of Mycobacterium abscessus infection should be decided according to the results of antibiotic susceptibility testing.

[Analysis of serum specific IgE in aeroallergen and food allergen in patients with allergic skin diseases].

OBJECTIVE: To investigate the role of aeroallergen and food allergen IgE in the pathogenesis of allergen diseases and the relationship between environments, diet, exposed factors and prevalence rate in allergic skin diseases. METHODS: Food allergen specific IgE (sIgE) antibody quantitative detection kit was used to detect 142 allergen cases, including artemisia, mixture of epithelia, mixed molds, mixed botany, mixed pollens, willow, poplar, egg, milk, shrimp, mutton, beef, fish, crab, staple foods, etc. RESULTS: Mixed molds and mixed botany were the most common allergens in the allergy dermatitis group (positive rate 60%), eczema group (43%), urticaria group (46%), and allergic purpuria group (71%). The milk revealed that it was the most common allergen in the allerge dermatitis group and eczema group (42% and 56%, respectively). While seafood was the most common allergen in the urticaria group (34%). There was no significant relationship between the exposure and prevalence rate. CONCLUSION: The detection of allergen IgE provides valuable basis for analysis of the cause in allergic disease.

Expression of glycoprotein non-metastatic melanoma protein B in cutaneous malignant and benign lesions: a tissue microarray study.

BACKGROUND: Glycoprotein non-metastatic melanoma protein B (GPNMB) plays an important role in the pathogenesis of inflammatory and malignant diseases. We investigated the expression of GPNMB in benign and malignant skin diseases. METHODS: Tissue microarray was performed in the skin tissues of 102 cases including malignant melanoma (MM), squamous cell carcinoma (SCC), basal cell carcinoma (BCC), and benign dermatosis. The expression of GPNMB in the tissues was detected by immunohistochemistry. Twenty cases of normal skin and adjacent neoplastic normal skin tissues were selected as controls. RESULTS: GPNMB was positively stained in skin malignancies (38/50, 76%), which was significantly higher than that in the control and the benign skin tissues (P = 0.001 and < 0.001 respectively). GPNMB was positively stained in MM (13/15, 87%) and SCC (16/20, 80%) (P < 0.001). Significant higher expression of GPNMB was observed in patients aged ≥ 65 years than those less than 65 years (n = 11 and n = 9 respectively, P = 0.027). No significant difference of the expression rates was observed between normal control and BCC; however, stronger intensity was detected in the latter. Negative or weak expression was observed in the controls. CONCLUSION: Over-expression of GPNMB correlated strongly and might play an important role in the pathogenesis of MM and SCC.

Hereditary benign telangiectasia without family history in China.

A case of hereditary benign telangiectasia without family history was reported. A 39-year-old woman presented with small and tiny telangiectases on the face, neck, upper trunk and forearms at birth. The numbers and sizes of the lesions increased gradually and she had no hemorrhagic diathesis and systemic diseases. No similar patients were found in her family. Upon physical examination, telangiectases were found on the face, neck, upper trunk and forearms; and a telangiectatic erythema was found on the right forearm 25 mm × 40 mm in size. Histopathology examination showed a normal epidermis and dilation of the capillaries at upper dermis. Hereditary benign telangiectasia without family history was diagnosed.