Mucosal Pemphigus Vulgaris Anti-Dsg3 IgG Is Pathogenic to the Oral Mucosa of Humanized Dsg3 Mice

There are two major clinical subsets of pemphigus vulgaris (PV), mucosal PV (mPV) and mucocutaneous PV (mcPV). The mPV subset exhibits anti-human desmoglein (Dsg) 3 autoantibodies that fail to recognize murine Dsg3; thus, passive transfer experiments of mPV IgG into WT mice have been unsuccessful at inducing disease. We therefore generated a fully humanized Dsg3 (hDSG3) murine model utilizing a human Dsg3 transgenic animal crossed to the murine Dsg3 knockout line. Expression of hDsg3 in the mucosa rescues the murine Dsg3 knockout phenotype. Well characterized mPV sera bind mucosal epithelia from the hDsg3 mice, but not mucosal tissues from WT mice by as detected by indirect immunofluorescence. The majority of mPV sera preferentially recognize hDsg3 compared to mDsg3 by immunoprecipitation as well. Passive transfer of mPV IgG into adult hDsg3 mice, but not WT mice, induces suprabasilar acantholysis in mucosal tissues, thus confirming pathogenicity of mPV anti-hDsg3 IgG in vivo. Human anti-hDsg3 antibodies are detected in perilesional mucosa as well as in sera of recipient mice by immunofluorescence. These findings suggest that the Dsg3 epitopes targeted by pathogenic mPV IgG are human specific. This hDsg3 mouse model will be invaluable in studying the clinical transition from mPV to mcPV

'Detection of pemphigus desmoglein 1 and 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison to serum values'

Although there is much literature on the detection of pemphigus and pemphigoid autoantibodies by enzyme-linked immunosorbent assay (ELISA) in serum, nothing is known about their presence in saliva. The aim of this study was to evaluate the salivary levels of these autoantibodies in pemphigus and pemphigoid patients. Autoantibodies against desmoglein3, desmoglein1, and BP180 were assayed, by ELISA, in serum and saliva samples of patients and healthy controls. The titres of autoantibodies against Dsg1/3 found in both serum and saliva of pemphigus patients showed a statistically significant correlation, suggesting that saliva may be a useful biological material for diagnostic purposes, in monitoring disease activity, as well as for the early detection of relapses. By contrast, the titres of autoantibodies against BP180 in the serum and saliva of bullous pemphigoid patients were not statistically related, and further study of the usefulness of the BP180 ELISA for saliva in this disease is needed. In addition, based on our results, the BP180 ELISA with a recombinant NC16a epitope failed to detect the autoantibodies against BP180 in the serum and saliva of mucous membrane pemphigoid patients

Characteristics of psoriasis in greece: an epidemiological study of a population in a sunny mediterranean climate

Psoriasis is a chronic inflammatory skin disease with important socio-economic consequences. Data on psoriasis prevalence in Greece is scarce and circumstantially reported. The aim of this study was the recording of psoriatic patients' demographic data, clinical characteristics of the disease, and exacerbating factors. Seven hundred and eighty four patients were enrolled in 6 centres (4 in Athens and 2 in Thessaloniki) in a multicenter epidemiologic prospective study. The mean age of patients was 43.2 (standard deviation, SD 17.44) years (median 42 years), while the men: women ratio was 1.8:1. Additionally, 35% of patients reported a positive family history of psoriasis. The mean age of patients at the first episode of psoriasis was 31.3 (SD 16.39) years (median 28 years). Psoriasis vulgaris was the most common form of psoriasis in the population participating in this study. Flares of psoriasis occurred 2.6 times per year on average. The patients considered stress as the main cause for psoriasis exacerbation. Most frequent target points of psoriasis included elbows, legs, scalp and knees. The most common symptoms reported were scaling, and itching. On average, patients visited dermatologists 2.4 times per year for issues related to psoriasis. This study provides epidemiological information regarding psoriasis in Greece. Results of this survey could assist in delineation of patient profiles, and improve communication between doctors and patients

Catarata subcapsular posterior em pacientes com pênfigo e penfigóide tratados com corticoterapia oral Posterior subcapsular cataract in patients with pemphigus and pemphigoid using oral corticosteroid

FUNDAMENTO: O tratamento mais utilizado para pacientes com dermatoses bolhosas crônicas é a corticoterapia oral, muitas vezes em altas doses e por períodos prolongados. Como efeitos colaterais dessa terapêutica, freqüentemente ocorrem: hipertensão arterial, diabete, osteoporose, infecções e distúrbios hidroeletrolíticos. A catarata subcapsular posterior é raramente citada na literatura como efeito colateral da corticoterapia em pacientes com doenças bolhosas. OBJETIVO: Avaliar a prevalência de catarata subcapsular posterior como efeito colateral da corticoterapia oral. MÉTODOS: Trata-se de um estudo retrospectivo incluindo 49 pacientes com dermatoses bolhosas crônicas em uso de corticoterapia acompanhados entre janeiro de 1987 e dezembro de 1997 no ambulatório de Dermatoses Bolhosas do Departamento de Dermatologia da Unifesp/EPM e regularmente submetidos à avaliação oftalmológica em busca de catarata cortisônica. RESULTADOS: No período de 1987 a 1997, 49 pacientes foram avaliados, tendo apresentado prevalência de catarata subcapsular posterior como efeito colateral da corticoterapia oral de 28,57%. CONCLUSÕES: 1) a prevalência de catarata foi de 28,57%; 2) o tempo médio de tratamento até o surgimento de catarata foi de 45,71 meses; 3) a média da dose máxima de corticosteróide utilizada pelos pacientes foi de 78,57mg/dia.<br>BACKGROUND: Oral corticosteroid is the treatment most used for patients with chronic bullous dermatosis, often at high dosages and over long periods of time. Arterial hypertension, diabetes, osteoporosis, infections and hydro-electrolytic disturbances frequently occur as side effects of this therapy. Posterior subcapsular cataract is rarely mentioned in the literature as a side effect of corticosteroid in patients with bullous diseases. OBJECTIVES: To evaluate the incidence of posterior subcapsular cataract as a side effect of oral corticosteroid. METHODS: A retrospective study: 49 patients with chronic bullous diseases using a corticosteroid were followed-up from 1987 to 1997 at the Bullous Dermatosis Outpatient Care Unit of the Department of Dermatology, UNIFESP - EPM. The patients were regularly submitted to ophthalmologic evaluation in search for cortisone cataract. This evaluation was carried out at the beginning of the treatment and regularly during the use of the corticosteroid. RESULTS: From 1987 to 1997, the incidence of posterior subcapsular cataract as a side effect of oral corticosteroid was 28.57% in these patients. CONCLUSIONS: 1) among 49 patients, 14 (28.57%) presented with cortisone cataract. 2) the period of time of prednisone use up to the appearance of cataract varied from eight months to nine years and three months (average 45.71 months). 3) the maximum dose of oral corticosteroid required for the management of the clinical picture ranged from 60 to 120 mg daily (average of 78.57 mg daily)