Telangiectasia as a potential clinical marker of microvascular lesions in systemic sclerosis patients from EUSTAR data in China.

OBJECTIVES: To investigate the prevalence and clinical relevance of telangiectasia in Chinese patients with systemic sclerosis (SSc). METHODS: Data from 230 SSc EUSTAR patients from Peking Union Medical College Hospital (2009-2011) that fulfilled the 1980 American College of Rheumatology SSc classification criteria were prospectively collected. Demographic, clinical, and laboratory data were calculated between groups with and without telangiectasia, and a six-minute walk test, pulmonary function test (PFT), transthoracic echocardiography (TTE), right heart catheterisation (RHC) and modified Rodnan skin score (mRSS) were performed. RESULTS: 96 patients (41.7%) were diagnosed with telangiectasia. There were no significant differences between patients with and without telangiectasia based on gender, age at onset, Raynaud's phenomenon (RP) duration, or SSc classification. Disease duration both from RP onset of patients and from first non-RP manifestation of patients with telangiectasia was significantly longer than patients without (p<0.05). RP (97.9% vs. 90.3%), finger/toe sclerosis (96.9% vs. 88.1%), facial sclerosis (68.8% vs. 53.7%), digital ulcers (DUs; 40.6% vs. 23.1%), digital pitting (49.0% vs. 33.8%), joint contracture (20.8% vs. 10.4%) and erythrocyte sedimentation rate elevation (26.7% vs. 14.8%) were significantly greater in telangiectasia patients (p<0.05). There were no differences in autoantibody development between patients with and without telangiectasia (p>0.05). PFT showed that forced vital capacity (77.0±17.26 vs. 83.05±16.53, p=0.005) and diffusion capacity for CO of the lung (58.9±19.4 vs. 65.7±19.7, p=0.030) were lower, while forced expiratory volume ratio (87.02±7.8 vs. 84.33±7.1, p=0.029) was higher in SSc with telangiectasia. Pulmonary artery hypertension (PAH) prevalence (25.0% vs. 14.2%) was significantly greater in patients with telangiectasia. CONCLUSIONS: Telangiectasia are common in Chinese SSc patients and usually associated with DUs, RP, and PAH. Telangiectasia could be a clinical marker of microvascular disease in SSc.

Characteristic dermoscopic features of melasma.

OBJECTIVE: To discribe the characteristic dermoscopic features of melasma. METHODS: Totally 20 patients with typical clinical presentations of melasma were included in this study. The melasma lesion and its surrounding normal skin were evaluated using dermatoscope. RESULTS: Melasma lesions manifested light yellow brown uniform patches in all patients, among whom dark brown patches were found in 19 patients, capillary network in 14 patients,and coarse hair in 6 patients. In contrast, no light yellow brown uniform patch was found in the normal skin in all patients (P<0.001), among whom dark brown patches were found in 10 patients (P<0.001), capillary network in 12 patients (P>0.05), and coarse hair in 2 patients (P>0.05). CONCLUSION: Light yellow brown uniform patches and dark brown patches are two major dermoscopic features of melasma.

Application of cloud database in the management of clinical data of patients with skin diseases.

OBJECTIVE: To evaluate the needs and applications of using cloud database in the daily practice of dermatology department. METHODS: The cloud database was established for systemic scleroderma and localized scleroderma. Paper forms were used to record the original data including personal information, pictures, specimens, blood biochemical indicators, skin lesions,and scores of self-rating scales. The results were input into the cloud database. The applications of the cloud database in the dermatology department were summarized and analyzed. RESULTS: The personal and clinical information of 215 systemic scleroderma patients and 522 localized scleroderma patients were included and analyzed using the cloud database. The disease status,quality of life, and prognosis were obtained by statistical calculations. CONCLUSIONS: The cloud database can efficiently and rapidly store and manage the data of patients with skin diseases. As a simple, prompt, safe, and convenient tool, it can be used in patients information management, clinical decision-making, and scientific research.

[Establishment of enzyme-linked immunosorbent assay in the detection of BP180NC16A-specific IgE and its significance in bullous pemphigoid].

OBJECTIVE: To establish a method of detecting circulating immunoglobulin E (IgE) autoantibodies for BP180NC16A and evaluate its significance in bullous pemphigoid (BP). METHODS: GST-NC16A fusion proteins were expressed in Escherichia coli using the pGEX-2T expression system and purified by glutathione affinity chromatography.For optimal working conditions of enzyme-linked immunoabsorbent assay (ELISA), checkerboard titrations were performed with serial dilutions of antigen. Also optimized dilution of secondary antibody was confirmed. Sera samples from 56 patients with BP, 24 healthy controls, 18 with pemphigus and 1 with Stevens-Johnson syndrome at our hospital during February 2011 to October 2012 were examined by the modified ELISA approach. The optimal cut-off point for a positive result was selected with receiver operating characteristic analysis. RESULTS: The optimized ELISA was performed with plates coated with 500 µg/ml GST-NC16A. And the optimal dilutions of sera samples and secondary antibody were 1: 10 and 1: 1000 respectively. According to the established cut-off value (0.549), 40 of 56 BP patients and none of controls had detectable levels of BP180NC16A IgE. CONCLUSION: The established ELISA provides a highly specific tool for the detection of IgE anti-BP180NC16A in BP patients.

[Correlation between blood eosinophil level and steroid doses in patients with bullous pemphigoid].

OBJECTIVE: To evaluate the correlation between blood eosinophil (EOS)level and steroid doses in patients of bullous pemphigoid (BP). METHODS: A total of 82 untreated BP inpatients (n=49) and outpatients (n=33) were enrolled in this study. The blood EOS level and the steroid doses before and after treatment were recorded. The correlation between EOS level and steroid doses was analyzed retrospectively. RESULTS: EOS increased in 69 BP patients (84.15%); on the contrary, only 10% of normal controls had increased EOS (t=1.99,P<0.001). In 44 inpatients, the blood EOS remained high before steroid treatment, and quickly returned to normal level after the disease became stable. There was a linear correlation between EOS and steroid doses (Spearman analysis,r=0.496,P<0.001). In 5 patients who were treated by non-steroid approach, EOS level also declined after the disease was resolved. CONCLUSION: EOS can be one of useful indicators for the application of steroids in the treatment of BP.

[Lupus erythematosus panniculitis: clinical and histopathological analysis of 22 cases].

OBJECTIVE: To explore the clinical and histopathological manifestations, therapy and prognosis of lupus erythematosus panniculitis (LEP). METHOD: We retrospectively reviewed the clinical data and histopathological features of 22 cases of LEP diagnosed at Peking Union Medical College Hospital from January 2008 to February 2010. RESULTS: The lesions appeared as atrophy, erythema, subcutaneous nodules, infiltrated erythema, and ulceration over the scalp, face, upper limbs, and buttock. Histopathological features were lobular panniculitis in the subcutaneous fat; sometimes septal panniculitis could be seen. Hydroxychloroquine sulfate and corticosteroid were effective treatment; most patients responded well to the treatment, while a few experienced recurrence when the treatment tapped or discontinued. CONCLUSIONS: LEP is a rare cutaneous variant of lupus erythematosus. The diagnosis of LEP is mainly based on clinical findings and pathological features. Hydroxychloroquine and low- and middle-dose corticosteroid are effective for the treatment of LEP.

Quantitative evaluation of rejuvenation treatment of nasolabial fold wrinkles by regression model and 3D photography.

OBJECTIVE: With the application of 3D photography, our study aimed to quantify parameters of static nasolabial fold wrinkles and establish mathematic regression model between parameters of wrinkles and age, further to quantitatively evaluate the effect of rejuvenation treatment in terms of age. METHODS: From October 2016 to May 2018, 433 Chinese female volunteers, aged 25-60 years old, were enrolled in this study. Antera 3D camera was used to collect four parameters of static nasolabial fold wrinkles on the left and right sides of the volunteers, including overall size, average depth (mm), average width (mm), and maximum depth (mm). For those presented a linear relationship with age, univariate linear regression fitting was performed, followed by residual analysis, goodness of fit test, and significance test. RESULTS: The results of univariate linear regression fitting showed there was a clear linear relationship between the maximum depth, average depth, overall size of nasolabial fold wrinkles and age, and the regression equations were established. The significance test of regression coefficients showed P values were less than .0001. CONCLUSIONS: With application of the regression model between parameters of nasolabial fold wrinkles and age, the effect of rejuvenation treatment can be quantitatively evaluated in terms of age, which has certain reference and promotion value.

[Hypopigmented mycosis fungoides in children: a clinicopathological study of 6 cases].

OBJECTIVE: To explore the clinical and histopathological manifestations, therapy and prognosis of hypopigmented mycosis fungoides (HMF) in children. METHODS: Six pediatric cases of HMF from February 2008 to October 2009 were reviewed and analyzed at our hospital. RESULTS: There were 5 males and 1 female with a average age of 11 years old (range: 9-14). The lesions appeared poorly-demarcated hypopigmented macula and patches disseminated over the trunk and extremities, scales and infiltrated papules over the hypopigmented lesions or invasive erythemas arose and enlarged gradually. Histologically, there was an epidermal infiltration of atypical lymphocytes. The immunohistochemical staining revealed a pattern of T lymphoma and a predominance of CD8+ T cell. All patients were of T2N0M0/IB except for one at the stage of T1N0M0/IA. Two patients received narrow band ultraviolet B (NB-UVB), one NB-UVB plus alpha interferon, another NB-UVB plus alpha interferon and topical steroid, one Chinese traditional herbal regimen while the last one none. And NB-UVB alone or plus alpha interferon achieved a clinical complete response (CR). The herbal regimen failed to cure the lesions but achieved no response. The non-treated patient progressed gradually. The average follow-up was 16 months (range: 9-28). The progression-free rate was 83.3% and the overall survival rate 100%. CONCLUSIONS: Visceral and lymph note involvements are rare in HMF of children. The prognosis of early-stage treatment is satisfactory. NB-UVB alone or plus alpha interferon achieves a clinical CR in most early-stage patients.

Mycobacterium abscessus infections following injection of botulinum toxin.

BACKGROUND: The incidence of Mycobacterium abscessus infections has increased in recent years. Some of these infections are caused by invasive cosmetic procedures. AIMS: Raising the awareness of cosmetic procedure related Mycobacterium abscessus infection for clinicians. PATIENTS/METHODS: We presented a 28-year-old woman who developed multiple erythema and painful nodules in her lower extremities after injections of botulinum toxin. RESULTS: Mycobacterium culture and strain identification of the tissue confirmed Mycobacterium abscessus. Combination antibiotics therapy was given and the lesion healed with scar and pigmentation. CONCLUSION: Mycobacterium abscessus infections following injection of botulinum toxin are rare and easily misdiagnosed as common suppurative infections. Early microbiologic tests are necessary for diagnose. Standardized operation should be performed to avoid this particular infection.

A double-blind, randomized, placebo- and positive-controlled phase III trial of 1% benvitimod cream in mild-to-moderate plaque psoriasis.

BACKGROUND: Benvitimod cream, a novel synthetic small molecule, was effective in treating mild-to-moderate plaque psoriasis. We conducted a phase III clinical trial to assess the efficacy and safety of benvitimod cream in patients with mild-to-moderate plaque psoriasis. METHODS: We randomly assigned 686 patients (2:1:1) to receive 1% benvitimod cream, 0.005% calcipotriol ointment or placebo twice a day for 12 weeks. The primary efficacy end points were the percentage of patients with a 75% or greater reduction from baseline in the psoriasis area and severity index (PASI 75) score and with a score of 0 or 1 in static physician's global assessment (sPGA) at week 12. RESULTS: The results showed that 50.4% of patients in the benvitimod group achieved PASI 75, which was significantly higher than that in the calcipotriol (38.5%, P < 0.05) and placebo (13.9%, P < 0.05) groups. The proportion of patients achieving an sPGA score 0 or 1 was 66.3% in the benvitimod group and 63.9% in the calcipotriol group, which were both significantly higher than that in the placebo group (34%, P < 0.05). In the long-term follow-up study, 50.8% of patients experienced recurrence. After retreatment with 1% benvitimod, 73.3% of patients achieved an sPGA score of 0 or 1 again at week 52. Adverse events included application site irritation, follicular papules, and contact dermatitis. No systemic adverse reactions were reported. CONCLUSION: During this 12-week study, benvitimod cream was demonstrated with high effectiveness and safety in patients with mild-to-moderate plaque psoriasis. TRIAL REGISTRATION: Chinese Clinical Trial Registry (ChiCTR), ChiCTR-TRC-13003259; http://www.chictr.org.cn/showprojen.aspx?proj=6300.

[Clinical and pathologic characteristics of patients with scleredema].

OBJECTIVE: To investigate the clinical and pathologic characteristics of patients with scleredema. METHOD: The clinical and pathologic data of 67 outpatients with scleredema who were admitted to PUMC Hospital from 1982 to 2008 were retrospectively analyzed. RESULTS: Neck and upper back lesions were detected in all patients, but no hand or foot involvement was noted. Among 54 patients who received serum immunoglobulin examination, 19 (35.2%) showed abnormal serum immunoglobulin results. Among 67 patients, 22 (32.8%) had concomitant diabetes mellitus. Alcian blue staining was performed in 35 patients, among whom 23 (65.7%) had positive results and 12 (34.3%) had negative results. CONCLUSIONS: Scleredema may have systemic involvements in addition to skin lesions. Patients with scleredema also tends to have concomitant diabetes mellitus. Alcian blue staining is not sufficient to differentiate scleredema and scleroderma.

[Genetic basis of systemic sclerosis].

Systemic sclerosis (SSc) is a connective tissue disease characterized by extensive fibrosis, vasculopathy, and activation of the immune system. Its pathogenesis and mechanisms have not been identified. Studies have shown that environmental and genetic factors are involved in the pathogenesis and development of SSc. Although the concordance for the disease among identical twins is low, concordance for antoantibodies associated with SSc and for fibroblast gene expression profiles is higher. However, the candidate-gene approach has not established clear associations between polymorphisms and SSc. Based on the involvement of SSc, the candidate gene can be screened from three groups: fibrosis, immune response, and vascular disease. This article summarizes the recent advances in these three aspects.

[Clinical characteristics of patients with juvenile localized scleroderma].

OBJECTIVE: To investigate the clinical characteristics of juvenile localized scleroderma (JLS). METHODS: The clinical data of 100 outpatients with JLS who were admitted to PUMC Hospital from 2000 to 2008 were retrospectively analyzed. RESULTS: Of a total of 100 cases, 51 (51%) were confirmed as linear scleroderma, 26 (26%) as plaque morphea, 26 (26%) as deep morphea, 12 (12%) as generalized morphea, and 15 (15%) as a mixed subtype. Nine patients (9%) had family histories of rheumatic or autoimmune diseases, while 16 (16%) might be triggered by unknown factors. Totally 84 patients underwent antinuclear antibody tests and 38 patients (45.2%) had positive results. CONCLUSIONS: Linear scleroderma are the most frequent subtype of JLS. Localized scleroderma may be associated with some autoimmune-related causes.

Analysis of the effect of different doses of oral tranexamic acid on melasma: a multicentre prospective study.

BACKGROUND: Melasma is pale brown to dark brown hyperpigmentation of the facial skin that commonly affects women of reproductive age. Treatment methods for melasma include oral and topical use of vitamin C, hydroquinone ointment, and laser treatment, with unsatisfactory results. Tranexamic acid (TA) has been shown to be effective against melasma, however, the optimal dose has not been investigated. OBJECTIVE: To analyse the therapeutic effect of different doses of oral TA on melasma. MATERIALS & METHODS: Patients with severe melasma were randomised to receive TA at a daily dose of 500 mg, 750 mg, 1,000 mg, or 1,500 mg. Clinical and VISIA photographs of the patients were taken at baseline, four weeks, eight weeks, six months, one year, and two years. The melasma area and severity index (MASI), as well as the melanin index, were measured. Routine blood and coagulation tests were performed at each time point. The photographs were divided into five groups according to level of improvement: apparent improvement, slight improvement, unchanged, and deterioration. RESULTS: Clinical photographs showed that all four doses of TA were effective in treating melasma, and the efficacy correlated with treatment time and dosage. However, there were no significant differences in the MASI or melanin index between the four doses. The treatment was generally safe for most patients and side effects included mild stomach upset and decreased menstruation. CONCLUSION: Oral TA was safe and effective for the treatment of melasma. Patient satisfaction was high and most patients could withstand long-term treatment.

Incidence of adverse cutaneous drug reactions in 22,866 Chinese inpatients: a prospective study.

Cutaneous adverse drug reactions (ADRs) are common. However, no prospective study assessing cutaneous ADRs is available for Chinese populations. This study aimed to assess the incidence, manifestations, causative drugs, and other factors related to cutaneous ADRs. A total of 22,866 inpatients were surveyed prospectively from January to April 2012 at the Peking Union Medical College Hospital. Only cutaneous ADRs induced by systemic drugs were considered. Fifty cases were confirmed as cutaneous ADRs, for an estimated incidence of 2.2 per 1000 during this period (95 % confidence interval 1.6-2.8). Cases of cutaneous ADRs comprised 69 % females, while 63 % of all inpatients were female (χ (2) = 0.641, P = 0.427). The department of infectious diseases was the most frequently involved department. Morbilliform exanthema (40 %) was the most frequent cutaneous ADR, followed by urticaria (23.1 %). Anti-infection drugs (36.9 %) caused most cases of cutaneous ADRs, followed by iodinated contrast media (ICM, 18.5 %) and non-steroidal anti-inflammatory drugs (NSAIDs, 18.5 %). The most frequently associated disorders were cancer (24 %), infection (22 %), cardiovascular and cerebrovascular diseases (20 %), and autoimmune diseases (18 %). In this first prospective study assessing the incidence of cutaneous ADRs in China, anti-infection drugs were the most commonly involved drugs, followed by ICM and NSAIDs. No evidence of increased cutaneous ADR incidence in AIDS or SLE patients was observed. Our findings indicate that cancer and its treatments were often related to cutaneous ADRs in China.

[Architecture, influencing factors, and sensitivity to antifungal agents of Candida biofilm].

OBJECTIVE: To establish a model of Candida biofilm and to explore its characteristics, ultrastructure, influences by saliva and serum, and sensitivity to antifungal agents. METHODS: Evaluations of the in vitro growth kinetics, influences by saliva and serum, and sensitivity to antifungal agents of Candida biofilm were performed with the abated tetrazolium salt XTT method on a 96-well microtire petri dish. The ultrastructure of Candida biofilm was observed under Confocal Laser Scanning Microscope (CLSM). RESULTS: The bioactivity of Candida biofilm increased with culturing time and serum could obviously increase the action of biofilm. The Candida biofilm was significantly resistant to routine antifungal agents. CONCLUSION: The Candida cells adhered in biofilms are significantly different in morphology from those in suspension and are resistant to routine antifungal agents such as Amphotericine B, Fluconazole and Itraconazole.

Application of a peripheral nerve block technique in laser treatment of the entire facial skin and evaluation of its analgesic effect.

BACKGROUND: The aim was to develop a technique for peripheral nerve block anesthesia (PNB) for the skin of the entire face and neck, considering the nerves anesthetized, injection sites, use of an injection method assisted by computer-controlled local anesthetic delivery (CCLAD), and to evaluate the analgesic effects of the PNB technique. METHODS: 50 patients who suffered from large nevus of Ota lesions or port-wine stains on their facial and neck skin and who required laser treatment were included. This study was designed as a non-randomized self-control trial. All the patients received the laser treatment under topical anesthesia in the first phase and three to six months later, they received the same treatment under the PNB using CCLAD. The differences in scores from the visual analogue scale of pain for the two phases were analyzed by the t-test. P values <0.05 were considered to be statistically significant. RESULTS: The peripheral nerve block technique was simple to execute and easy to learn, the anesthetic injection site was generally located at a subcutaneous depth of 0.5-1.0 cm. The analgesic effect of PNB was significant, the mean pain score (2.8 ± 2.2) was significantly lower than that with topical anesthesia (P<0.0001). Patients during the PNB phase did not experience injection pain following CCLAD. CONCLUSION: The peripheral nerve block technique can greatly ease the pain that occurs during laser treatment, especially for patients with larger lesions. CCLAD will allow PNB to be broadly applied in laser treatments.