Reliability and validity of a measure to assess the health-related quality of life of women with alopecia areata.

Alopecia areata (AA) has an impact on health-related quality of life (HRQoL). The Women's Androgenetic Alopecia Quality of Life (WAA-QoL) questionnaire is a reliable, validated HRQoL measure in women with androgenetic alopecia (AGA). There is no equivalent measure for female patients with AA. Data were collected as part of the Global Registry of Alopecia Areata Disease Severity and Treatment Safety (GRASS) Australia. The WAA-QoL, Dermatology Life Quality Index (DLQI) and Skindex-16 for AA, as well as the Severity of Alopecia Tool score, were extracted from GRASS for adult female patients. Cronbach's alpha and factor analysis were employed to determine the internal consistency of the measure, and nonparametric correlation testing assessed the validity of the questionnaire. Overall, 137 individuals completed the questionnaires. There was excellent internal consistency of the WAA-QoL among women with AA (Cronbach's α = 0.98). A moderate and high positive correlation was found between the WAA-QoL and the DLQI and the Skindex-16 for AA, respectively. The WAA-QoL is a reliable and valid assessment of HRQoL among women with AA.

Global Guidelines in Dermatology Mapping Project (GUIDEMAP): a systematic review of alopecia areata clinical practice guidelines.

INTRODUCTION: Alopecia areata (AA) is a nonscarring alopecia with an estimated global prevalence of 2% and limited data on the efficacy of current treatment. Clinical practice guidelines (CPGs) provide recommendations based on best available evidence. It is unclear how many AA CPGs are available globally. AIM: To systematically search for and identify CPGs on AA and to critically appraise their quality using validated tools. METHODS: We performed a literature search to identify CPGs published between October 2014 and April 2021, using the following databases: MEDLINE, Embase, National Institute for Health and Care Excellence (NICE), Guidelines International Network, Emergency Care Research Institute guidelines trust, Australian CPGs, Turning Research Into Practice database and DynaMed. The systematic review was conducted and reported in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses framework. Three critical appraisal tools were used: Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument, Lenzer's red flags and United States Institute of Medicine's (IOM) criteria of trustworthiness. RESULTS: In total, six AA CPGs from seven manuscripts (one CPG was in two parts published in separate papers) were included. The majority (four of six) of the CPGs focused on treatment. Four CPGs (total of five papers) were in English and two CPGs were only available in the original language (one Russian and one Japanese). All AA CPGs demonstrated low quality in several domains in the AGREE II appraisal, including stakeholder involvement and applicability, with the latter being deemed the worst domain for all CPGs, with an average of 29%. The mean (SD) number of Lenzer's red flags for the included CPGs was 3.4 (1.5) out of a total of 8 possible red flags, while the IOM criteria showed 1.6 (0.8) 'fully met' criteria and 3.1 (1.2) 'not met' out of a total of 9 criteria. CONCLUSION: We found a limited number of AA CPGs, all of which had significant methodological deficiencies. We encourage guideline development groups to use validated checklists/tools to develop reliable and trustworthy CPGs.

Effective treatment of refractory lichen planus pemphigoides with a Janus kinase-1/2 inhibitor.

Lichen planus pemphigoides is a rare autoimmune subepidermal blistering disease clinically and histopathologically characterized by features of lichen planus and bullous pemphigoid. We describe a case of refractory lichen planus pemphigoides successfully treated with the selective and reversible Janus kinase-1/2 inhibitor, baricitinib.

Epidermolysis Bullosa Acquisita (Brunsting-Perry Pemphigoid Variant) Localized to the Face and Diagnosed With Antigen Identification Using Skin Deficient in Type VII Collagen.

Brunsting-Perry pemphigoid is defined as an autoimmune vesiculobullous eruption typically localized on the head and neck region with minimal or no mucosal involvement. The disease tends to run a chronic and recurrent course with residual scarring. Histological features are characterized by subepidermal bullae and linear IgG deposits at the dermo-epidermal junction. We report a case of a 46-year-old lady who presented with typical features of Brunsting-Perry pemphigoid. Autoantibodies to type VII collagen were identified by using recessive dystrophic epidermolysis bullosa skin which lacks type VII collagen in an indirect immunofluorescence assay. As a result, we diagnosed our patient as having the Brunsting-Perry pemphigoid variant of epidermolysis bullosa acquisita (EBA). This finding led us to review the literature on target antigens in Brunsting-Perry pemphigoid. Only 11 out of the 58 cases reported to date had target antigens identified. Interestingly, type VII collagen was the second most common target antigen/autoantibody (4 cases) detected after BP180 (5 cases). However, 2 further cases of EBA localized to the face with typical features of Brunsting-Perry pemphigoid were found in the literature. Although the target antigens are heterogeneous in Brunsting-Perry pemphigoid, a significant number of cases represent a clinical presentation of localized EBA.