Prevalence estimates for pemphigoid in the United States: A sex-adjusted and age-adjusted population analysis.

BACKGROUND: The burden of the pemphigoid group of autoimmune blistering diseases is poorly understood. OBJECTIVE: To estimate standardized overall and sex-specific, age-specific, and race-specific prevalence estimates for pemphigoid among adults in the United States. METHODS: Cross-sectional analysis of electronic health records data for a demographically heterogeneous population-based sample of >55 million patients across all 4 census regions. RESULTS: Overall pemphigoid prevalence was 0.012%, or 12 pemphigoid patients/100,000 adults. Prevalence of pemphigoid among those aged ≥60 years was 0.038%, or 37.7 cases/100,000 adults. Prevalence increased ∼2-fold within each successive age group and was highest among patients aged ≥90 years (123.6 [95% CI 115.2-132.5] cases/100,000 adults). Adjusted prevalence in women was 12.7 (95% CI 12.3-13.2) cases/100,000 adults, slightly more than that in men (11.0 [95% CI 10.5-11.6] cases/100,000 adults). Adjusted prevalences were similar for blacks (15.4 [95% CI 14.0-17.0] cases/100,000 adults) and whites (13.5 [95% CI 13.0-13.9] cases/100,000 adults). LIMITATIONS: Analysis of electronic health data might result in disease misclassification. CONCLUSION: Pemphigoid is rare in the United States. Patients aged ≥60 years comprise the majority of cases.

Bullous pemphigoid and neurological disorders among a Jewish population.

A retrospective case control study included data regarding 65 Jewish bullous pemphigoid (BP) patients diagnosed in a tertiary medical center and a control group consisting of 182 matched Jewish patients. The study indicates that Jewish patients with BP suffer from a higher prevalence of neurological diseases compared to patients with no BP, similarly to previous reports showing high prevalence of neurological diseases among BP patients from different ethnic groups.

Ethnic variations in the epidemiology of bullous pemphigoid in Israel.

BACKGROUND: No ethnic or geographic predisposition to bullous pemphigoid (BP) was reported so far. OBJECTIVE: To evaluate trends in the incidence of BP in northern Israel, shedding light on differences between two distinct ethnic populations who inhabit the same region, namely Jews and Arabs. METHODS: Bullous pemphigoid incidence was retrospectively estimated from January 2000-December 2015 in two Israeli regions with a total population of 1.56 million inhabitants. RESULTS: A total of 287 new-onset BP patients (mean age: 77.6 ± 12.1) were identified. The incidence rate was 11.4 per million inhabitants per year (95% CI, 10.2-12.9). The crude incidence rate in Jews was 4.8-fold higher than that in Arabs (16.4 vs. 3.4 cases per million per year, respectively [P < 0.0001]). After adjusting for age, the discrepancy between the two populations diminished (11.7 vs. 8.9 cases per million per year, respectively) but remained statistically significant (P = 0.032). The incidence increased consistently from 7.6 cases per million per year in the calendar period 2000-2005 to 12.6 and 14.3 cases in 2006-2010 and 2011-2015, respectively (P < 0.0001). Bullous pemphigoid patients of Arab ancestry presented with the disease significantly earlier than Jews (69.5 ± 15.3 vs. 78.7 ± 11.1 years, respectively, P = 0.002). CONCLUSIONS: The incidence of BP in northern Israel has increased in the last 16 years and is significantly higher among Jews compared to Arabs. The age of presentation is remarkably different between patients belonging to the two populations.

Anti-BP180 NC16A IgG Titres as an Indicator of Disease Activity and Outcome in Asian Patients with Bullous Pemphigoid.

INTRODUCTION: Anti-BP180 IgG titres were observed to parallel disease activity in case series of bullous pemphigoid (BP). This study aimed to examine whether anti-BP180 titres are an indicator of disease severity, clinical course and outcome in Asian patients with BP. MATERIALS AND METHODS: This was a prospective observational study conducted between March 2005 and March 2008 in the Immunodermatology Clinic at the National Skin Centre, Singapore. Disease activity and anti-BP180 IgG titres were measured 4-weekly for 12 weeks and during disease flares and clinical remission. Associations between anti-BP180 titres and disease activity, disease flare, clinical remission and cumulative prednisolone dose were examined. RESULTS: Thirty-four patients with newly diagnosed BP were recruited. Median follow-up duration was 3 years. Notable correlations between disease activity and anti-BP180 titres were at baseline (r = 0.51, P = 0.002), and disease flare (r = 0.85, P <0.001). Lower titres at Week 12 were associated with greater likelihood of clinical remission (P = 0.036). Post hoc, patients with anti-BP180 titres above 87.5 U/mL at time of diagnosis who reached remission within 2 years of diagnosis received significantly higher cumulative doses (mg/kg) of prednisolone (median, 72.8; range, 56.5 to 127.1) than those with titres <87.5 U/mL (median, 44.6; range, 32.5 to 80.8); P = 0.025). CONCLUSION: Anti-BP180 titres may be a useful indicator of disease activity at time of diagnosis and at disease flare. Lower titres at Week 12 may predict greater likelihood of clinical remission. Titres above 87.5 U/mL at time of diagnosis may suggest the need for higher cumulative doses of prednisolone to achieve remission within 2 years.

Bullous pemphigoid seen at the National Skin Centre: a 2-year retrospective review.

INTRODUCTION: Bullous pemphigoid (BP) is the most common immunobullous disorder in Western Europe, affecting mainly the elderly. Previous reports have suggested that it is rarer in the Far East. We report on the clinico-epidemiological features of BP seen at our centre. MATERIALS AND METHODS: This is a 2-year retrospective study of patients seen at the National Skin Centre who were diagnosed to have BP. RESULTS: Fifty-nine patients were newly diagnosed with BP over a 2-year period between January 1998 and December 1999. The minimum estimated incidence was 7.6 per million population per year, with mean age of onset of 77 years, and a male to female ratio of 1:2. There was a predilection for Chinese but not Indian. Localised disease was found in 34% and mucosal involvement in only 1.7%. Direct immunofluorescence was positive in almost all patients, and 94% of those tested had positive indirect immunofluorescence. 'Roof' pattern was present in 95.8% and 'roof and floor' pattern in 4.2%. Prednisolone was the treatment of choice in most patients (78%). Anti-inflammatory agents were more commonly used than a decade ago (13.5%). CONCLUSIONS: BP is the most common immunobullous disease locally, with an incidence at least equal to that in Western Europe. There was a predilection for ethnic Chinese rather than Indian. Apart from the rarity of mucosal involvement, the clinical features are similar to those of Western nations.

Polymorphisms of HLA-DR and -DQ genes in Japanese patients with bullous pemphigoid.

Bullous pemphigoid (BP), an autoimmune skin disease of the elderly, is mediated by autoantibodies that bind to hemidesmosomes of epidermal basal cells. This study investigated BP-associated HLA-DR and -DQ genes among Japanese patients. We analyzed HLA-DR and -DQ genes among 23 Japanese BP patients based on the polymerase chain reaction-restriction fragment length polymorphism. Eighteen of these 23 patients (78%) carried at least one allele of HLA-DRB1*04 or DRB1*1101, with significant increases in HLA-DRB1*04 (*0403, *0406)/DQA1*0301/DQB1*0302 and DRB1*1101/DQA1*0505/DQB1*0302 haplotypes as well as the individual alleles DRB1*1101 and DQB1*0302 (corrected p < 0.05 for each comparison), when compared to control subjects. These data differ from the accepted DQB1*0301 (DQ7) association with the same disease among Caucasians. These findings indicate that different HLA class II haplotypes genetically influence susceptibility to BP among different ethnic groups. Our findings, together with previous reports on Caucasian patients with the pemphigoid group of bullous diseases, suggest that HLA-DRB1 molecules might participate in the regulation of autoimmune responses to BP antigens.

Bullous pemphigoid in an Aborigine.

OBJECTIVE: To present the first case of bullous pemphigoid in an Australian Aborigine. CLINICAL FEATURES: A 47 year old female aborigine presented with a three week history of a generalised skin eruption consistent with bullous pemphigoid. Immunohistological examination confirmed the diagnosis. Therapy required high dose oral steroids, azathioprine and erythromycin as well as topical agents. Treatment was complicated by isolation and poor compliance but was ultimately successful in inducing and retaining remission. CONCLUSION: This is the first description of bullous pemphigoid in an Australian Aborigine. We recommend early biopsy to confirm diagnosis and plan therapy, and careful attention to patient education to encourage compliance.