SummaryBackgroundBullous pemphigoid is a blistering skin disorder with increased mortality. We tested whether a strategy of starting treatment with doxycycline gives acceptable short-term blister control while conferring long-term safety advantages over starting treatment with oral corticosteroids.MethodsWe did a pragmatic, multicentre, parallel-group randomised controlled trial of adults with bullous pemphigoid (three or more blisters at two or more sites and linear basement membrane IgG or C3). Participants were randomly assigned to doxycycline (200 mg per day) or prednisolone (0·5 mg/kg per day) using random permuted blocks of randomly varying size, and stratified by baseline severity (3–9, 10–30, and >30 blisters for mild, moderate, and severe disease, respectively). Localised adjuvant potent topical corticosteroids (<30 g per week) were permitted during weeks 1–3. The non-inferiority primary effectiveness outcome was the proportion of participants with three or fewer blisters at 6 weeks. We assumed that doxycycline would be 25% less effective than corticosteroids with a 37% acceptable margin of non-inferiority. The primary safety outcome was the proportion with severe, life-threatening, or fatal (grade 3–5) treatment-related adverse events by 52 weeks. Analysis (modified intention to treat [mITT] for the superiority safety analysis and mITT and per protocol for non-inferiority effectiveness analysis) used a regression model adjusting for baseline disease severity, age, and Karnofsky score, with missing data imputed. The trial is registered at ISRCTN, number ISRCTN13704604.FindingsBetween March 1, 2009, and Oct 31, 2013, 132 patients were randomly assigned to doxycycline and 121 to prednisolone from 54 UK and seven German dermatology centres. Mean age was 77·7 years (SD 9·7) and 173 (68%) of 253 patients had moderate-to-severe baseline disease. For those starting doxycycline, 83 (74%) of 112 patients had three or fewer blisters at 6 weeks compared with 92 (91%) of 101 patients on prednisolone, an adjusted difference of 18·6% (90% CI 11·1–26·1) favouring prednisolone (upper limit of 90% CI, 26·1%, within the predefined 37% margin). Related severe, life-threatening, and fatal events at 52 weeks were 18% (22 of 121) for those starting doxycycline and 36% (41 of 113) for prednisolone (mITT), an adjusted difference of 19·0% (95% CI 7·9–30·1), p=0·001.InterpretationStarting patients on doxycycline is non-inferior to standard treatment with oral prednisolone for short-term blister control in bullous pemphigoid and significantly safer in the long-term.FundingNIHR Health Technology Assessment Programme.

One hundred and fifty-two patients in whom a diagnosis of dermatitis herpetiformis was made at St John's Hospital for Diseases of the Skin, London, during 1950-85, were followed from the date of diagnosis to the end of 1989 for mortality, and from 1971, or the date of diagnosis if later, to 1986 for cancer incidence. Thirty-eight deaths occurred under the age of 85, slightly fewer than expected on the basis of national general population rates [standardized mortality ratio (SMR) = 87; 95% confidence interval (CI) 61-119]. All-cause mortality was somewhat lower in patients who had followed a gluten-free diet (SMR = 51; 17-120) than in those who had not (SMR = 97; 66-136), but the difference in SMRs was not significant (P = 0.3). Cancer mortality was non-significantly below expectations from national rates (SMR = 72; 31-142), but cancer incidence was significantly increased [standardized registration ratio (SRR) = 394; 180-749]. No particular cancer site accounted for the cancer incidence excess. One death occurred from cancer of the small intestine (SMR = 4953, P = 0.04), and one lymphoma was incident (SRR = 1555, P = 0.12). Increased risks of these malignancies have previously been found to be associated with coeliac disease (which is present in many patients with dermatitis herpetiformis), and with dermatitis herpetiformis, respectively. Mortality from ischaemic heart disease (IHD) was significantly below national rates (SMR = 37; 95% CI 12-86), and was similar in patients who had followed a gluten-free diet and those who had not.(ABSTRACT TRUNCATED AT 250 WORDS)

Summary We describe the clinico‐pathological findings of twelve cases of lichen aureus. The distinguishing features and differential diagnoses are discussed.

Analysis of the autoantibody profile is not only of diagnostic relevance but may also be helpful in predicting the course of the disease.