Evaluation of Adrenal Suppression of a Lipid Enhanced, Topical Emollient Cream Formulation of Hydrocortisone Butyrate 0.1% in Treating Children with Atopic Dermatitis

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/75102/1/j.1525-1470.2007.00342.x.pd

Definitions and outcome measures for mucous membrane pemphigoid: Recommendations of an international panel of experts

Mucous membrane pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of mucous membranes, skin, or both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for mucous membrane pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index

Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic non-inferiority randomised controlled trial

Background: Bullous pemphigoid (BP) is a blistering skin disorder with increased mortality. We tested whether a strategy of starting treatment with doxycycline conveys acceptable short-term blister control whilst conferring long-term safety advantages over starting treatment with oral corticosteroids. Methods: Pragmatic multi-centre parallel-group randomised controlled trial of adults with BP (≥3 blisters ≥2 sites and linear basement membrane IgG/C3) plus economic evaluation. Participants were randomised to doxycycline (200 mg/day) or prednisolone (0·5 mg/kg/day). Localised adjuvant potent topical corticosteroids (<30 g/week) was permitted weeks 1-3. The non-inferiority primary effectiveness outcome was the proportion of participants with ≤3 blisters at 6 weeks. We assumed that doxycycline would be 25% less effective than corticosteroids with a 37% acceptable margin of noninferiority. The primary safety outcome was the proportion with severe, life-threatening or fatal treatment-related adverse events by 52 weeks. Analysis used a regression model adjusting for baseline disease severity, age and Karnofsky score, with missing data imputed. Results: 132 patients were randomised to doxycycline and 121 to prednisolone from 54 UK and 7 German dermatology centres. Mean age was 77·7 years and 68.4% had moderate to severe baseline disease. For those starting doxycycline, 83/112 (74·1%) had ≤3 blisters at 6 weeks compared with 92/101 (91·1%) for prednisolone, a difference of 18·6% 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 18·5% for those starting doxycycline and 36·6% for prednisolone (mITT analysis), an adjusted difference of 19·0% (95% CI, 7·9%, 30·1%, p=0·001). Conclusions: A strategy of starting BP patients on doxycycline is non-inferior to standard treatment with oral prednisolone for short-term blister control and significantly safer long-term

Immunodiagnosis in cutaneous T cell lymphoma: how does gene expression of the variable region of the T cell receptor fit into the diagnostic and pathophysiological picture of T cell neoplasia

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/71456/1/j.1600-0560.1992.tb01552.x.pd

Anti-p200 pemphigoid (anti-laminin-γ1 pemphigoid) demonstrating pathergy

Anti-p200 pemphigoid, also called anti-laminin-γ1 pemphigoid, is a recently defined entity. First reported in 1996, the incidence is relatively rare, with approximately 70 reports in the literature. Clinical presentation is heterogeneous, but the disease most commonly mimics bullous pemphigoid with urticarial papules, plaques, or tense bullae on the trunk or extremities. Described here is a case with additional features of pathergy that have not yet been reported in the literature