The autoimmune blistering diseases in Australia: status and services

The exact epidemiology of autoimmune bullous disorders in Australia is unknown. This article describes the establishment of a registry and support group to serve patients with these disorders. The registry aids research in Australia by providing epidemiologic data and maintaining an up-to-date database with patient contact details. The support group caters to the needs of patients and/or caregivers. Teledermatology, diagnosis, and management of autoimmune bullous disease are briefly reviewed.     

Pathogenesis of bullous pemphigoid

Bullous pemphigoid, the most common autoimmune blistering disease, is induced by autoantibodies against type XVII collagen. Passive transfer of IgG or IgE antibodies against type XVII collagen into animals has revealed not only the pathogenicity of these antibodies but also the subsequent immune responses, including complement activation, mast cell degranulation, and infiltration of neutrophils and/or eosinophils. In vitro studies on ectodomain shedding of type XVII collagen have also provided basic knowledge on the development of bullous pemphigoid. The pathogenic role of autoreactive CD4+ T lymphocytes in the development of the pathogenic autoantibodies to type XVII collagen should also be noted.     

糖尿病小鼠角膜损害的形态观察

目的　观察糖尿病小鼠角膜形态损害的特点,为后期进行的糖尿病角膜病变保护因子研究提供研究基础。方法　ＳＴＺ诱导的糖尿病Ｃ５７ＢＬ／Ｊ小鼠,分别于糖尿病成功造模后３个月、６个月时,选取糖尿病小鼠和正常对照组小鼠角膜,石蜡包埋后行ＨＥ染色观察形态学变化。结果　糖尿病成功造模后３个月、６个月,与正常对照组相比,糖尿病小鼠角膜水肿、基质增厚,角膜溃疡区上皮缺损,溃疡区基质增厚、角膜水肿明显高于非溃疡区。结论　糖尿病小鼠造模后３个月即可发生角膜基质病变,６个月时角膜基质改变更加严重。     

A comparative study of risk factors for corneal infection in diabetic and non-diabetic patients

AIM: To compare the clinical characteristics of infectious keratopathy in type 2 diabetes mellitus (T2DM) and non-diabetes mellitus (NDM) and to investigate risk factors for infectious keratopathy in T2DM patients. METHODS: Totally 230 patients with T2DM and 168 with NDM diagnosed as infectious keratopathy were hospitalized at Qingdao Eye Hospital from 2001 to 2015. Data including sex, age, occupation, season, smoking and alcohol consumption habits, duration between onset and treatments, duration of hospitalization were collected. Initially identified indicators were analyzed with a multivariate logistic regression. Glycosylated hemoglobin A1c (HbA1c) in patients with T2DM was analyzed. The infectious keratopathies in the two groups were categorized and compared. RESULTS: The diabetic group consisted of 146 (63.5%) males and 84 (36.5%) females. The NDM group consisted of 111 (66.1%) males and 57 (33.9%) females. There was no signigicantly difference in sex distribution between the two groups (P>0.05). There were significant differences in age, occupation of patients, season of the onset of diseases, duration between onset and treatment, and durations of hospitalization between the two groups (P<0.05). In most of the patients in the diabetic group, the duration between onset and treatment was ≤3mo, and most was ≥3mo in the NDM group. Multivariate logistic regression analysis revealed that age and season were related to the development of corneal infection in the T2DM group (OR=1.709, 1.706). In the T2DM group, HbA1c was 9.09%±2.12%. There were statistically significant differences in the incidences of bacterial keratitis and herpes simplex keratitis in the two groups (P<0.05), but no significant statistical difference was found between fungal keratitis and amoebic keratitis (P>0.05). CONCLUSION: Advanced age and the summer and winter seasons are identified as risk factors for infectious keratopathy in T2DM patients, and T2DM patients are more prone to bacterial keratitis.     

Congenital erythropoietic porphyria: two case reports

Porphyrias form a group of disorders caused due to defects in the haem synthetic pathway. Congenital erythropoietic porphyia (CEP) is the rarest of the bullous porphyrias (less than 200 cases have been reported till recent times) and a clinician may not see a case during his professional life. We present two cases of CEP. One child with CEP presented with typical infancy-onset blistering, photosensitivity, red urine, and erythrodontia, with hypertrichosis of the upper arms and back. The other child of CEP presented with childhood-onset blistering, mutilation, and hypertrichosis on the face.     

Staphylococcal bullous impetigo in a neonate

An otherwise healthy, full-term neonate presented at day 15 of life to the pediatric emergency with generalized papulo-pustular rash for 2 d. This was finally diagnosed as bullous impetigo caused by Staphylococcus aureus (S. aureus). The skin lesions decreased significantly after starting antibiotic therapy and drainage of blister fluid. There was no recurrence of the lesions on follow-up. This case of generalized pustular eruption due to S. aureus in a neonate is reported, as it poses a diagnostic dilemma and can have serious consequences if left untreated.     

大疱性类天疱疮临床评估指标的研究进展

大疱性类天疱疮是一类自身免疫性疱病。针对自身免疫性大疱病,有专门的评分指标用以评估病情的严重程度和对治疗的反应。常用的评价指标有自身免疫性疱病严重程度评分,专门针对大疱性类天疱疮的大疱性类天疱疮疾病面积指数评分,以及其他的一些评分方式。自身免疫性大疱病严重程度评分已经经过了效度检验,广泛地应用在寻常型天疱疮和其他一些自身免疫性疱病的评估中。大疱性类天疱疮疾病面积指数评分尚处于检验的过程中,应用该评分的临床试验较少。上述两种评分方式均对全身的皮肤、黏膜皮损情况,主观瘙痒程度进行了评分。     

Fraktionierung und Klassifizierung humaner Immunglobuline und ihre Reaktion mit sessilen Antigenen beim Alterspemphigoid

Zusammenfassung Uns ist eineFraktionierung und eineAnreicherung der Basalmembran-Antikörper im Serum von am Alterspemphigoid Erkrankten gelungen. Wie der Vergleich des IgG-Gehaltes verschiedener Fraktionen ergibt, reagieren innerhalb der Familie der IgG-Proteine nur distinkte Antikörper mit der Basalmembran. Die Verteilung des aktiven Eiweißes auf verschiedene Fraktionen ist dabei höchstwahrscheinlich einpH-Effekt, so daß offenbar nur identische Molekeln hier eine Antigen-Antikörper-Reaktion bewirken.

---

     

A simple technique for immunoelectronmicroscopic localization of Ig on tissue sections with special reference to cutaneous pathology

Summary A simple technique using immunoperoxidase-labelled antibodies is reported for the ultrastructural localization of fixed immunoglobulins in tissue lesions. This technique allows the immunolabeling of frozen sections of 15 µ mounted on glass slides. These sections are then closed over for ultrastructural examination, using an Epon-filled gelatin capsule, and then returned to the glass slide. After polymerization, the capsule containing the tissue section is separated from the glass by immersion in liquid nitrogen. This technique is applied to the study of fixed Ig in the skin lesions of lupus erythematosus, pemphigus, bullous pemphigoid and dermatitis herpetiformis. The results obtained show a fine localization of fixed Ig, good tissue conservation and easy orientation of the biological material. It can be applied to the study of tissue antigens.Chargé de recherche INSERM. This study was supported by U.E.R. Biologie Humaine, Lyon Offprint requests to: D. Schmitt