Cathy Y. Zhao

Report from the fourth international consensus meeting to harmonize core outcome measures for atopic eczema/dermatitis clinical trials (HOME initiative)

This article is a report of the fourth meeting of the Harmonising Outcome Measures for Eczema (HOME) initiative held in Malmö, Sweden on 23–24 April 2015 (HOME IV). The aim of the meeting was to achieve consensus over the preferred outcome instruments for measuring patient‐reported symptoms and quality of life for the HOME core outcome set for atopic eczema (AE). Following presentations, which included data from systematic reviews, consensus discussions were held in a mixture of whole group and small group discussions. Small groups were allocated a priori to ensure representation of different stakeholders and countries. Decisions were voted on using electronic keypads. For the patient‐reported symptoms, the group agreed by vote that itch, sleep loss, dryness, redness/inflamed skin and irritated skin were all considered essential aspects of AE symptoms. Many instruments for capturing patient‐reported symptoms were discussed [including the Patient‐Oriented SCOring Atopic Dermatitis index, Patient‐Oriented Eczema Measure (POEM), Self‐Administered Eczema Area and Severity Index, Itch Severity Scale, Atopic Dermatitis Quickscore and the Nottingham Eczema Severity Score] and, by consensus, POEM was selected as the preferred instrument to measure patient‐reported symptoms. Further work is needed to determine the reliability and measurement error of POEM. Further work is also required to establish the importance of pain/soreness and the importance of collecting information regarding the intensity of symptoms in addition to their frequency. Much of the discussion on quality of life concerned the Dermatology Life Quality Index and Quality of Life Index for Atopic Dermatitis; however, consensus on a preferred instrument for measuring this domain could not be reached. In summary, POEM is recommended as the HOME core outcome instrument for measuring AE symptoms.

A pilot comparison study of four clinician-rated atopic dermatitis severity scales

There are multiple severity outcome measures for atopic dermatitis (AD). There is a need to compare the reliability of these measures.

Outcome measures for autoimmune blistering diseases

Outcome measures are crucial in assessing an autoimmune blistering diseases (AIBD) severity as well as its impact on the patients quality of life (QOL). The standardization of AIBD outcome measures is pivotal to accurately monitor the patient and to pool results from randomized controlled trials for meta‐analysis, and thereby provide knowledge of the optimal AIBD therapies. In the past decade, several AIBD severity outcome measures have been developed and validated. For pemphigus severity, the Pemphigus Disease Area Index (PDAI) developed by the International Pemphigus Definitions Group was shown to be the most superior, followed by the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) by the German group. For bullous pemphigoid severity, the Bullous Pemphigoid Disease Area Index (BPDAI) was shown to be an accurate and valid measure. To quantify the burden of AIBD and its treatments on QOL, the Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) were also developed, validated, and are now being validated in multiple languages and cultures.

Autoimmune blistering diseases in females: a review☆ , ☆☆

The autoimmune blistering diseases (AIBDs) are a group of heterogeneous skin diseases with autoantibodies directed against structural proteins in the skin. A new interest in the female bias towards autoimmune diseases in general has led to our attention to focus on how and why this female bias manifests in AIBD. The authors aim to review and explore the various aspects of AIBD affecting females more than males, including the higher prevalence, worse quality of life, and complex management issues such as pregnancy and lactation.

A review of case-control studies on the risk factors for the development of autoimmune blistering diseases.

Autoimmune blistering diseases (AIBD) are a group of rare but potentially fatal diseases characterized by the production of autoantibodies directed against the structural proteins of the skin. Much has been published on the clinical manifestation and interventional options for AIBD, especially on the more common subtypes such as bullous pemphigoid, pemphigus vulgaris (PV) and pemphigus foliaceus (PF). However, the aetiology of AIBD remains unknown. We aim to provide an overview of published case–control studies focussing on the non‐genetic aetiological factors of bullous pemphigoid, PV and/or PF. The relevant studies were appraised for their validity and results. Our results showed that a large proportion of the studies had inconclusive results due to compromised study methodologies. Moreover, there were no identified case–control studies that investigated the possible associations between bullous pemphigoid and patient environment, or the potential links between pemphigus and drugs. Hence, a case–control study with a higher quality design addressing these shortcomings would contribute greatly to our knowledge of AIBD.

A comparison study of clinician-rated atopic dermatitis outcome measures for intermediate to dark skin patients

Atopic dermatitis (AD) assessment is more difficult in patients with skin of colour (SOC).

The Reliability, Validity and Responsiveness of Two Disease Scores (BPDAI and ABSIS) for Bullous Pemphigoid: Which One to Use?

A significant obstacle in guiding evidence-based management of bullous pemphigoid (BP) is the lack of a standardised, validated scoring system for the condition. The aim of this study is to evaluate the suitability of the Bullous Pemphigoid Disease Area Index (BPDAI) and the Autoimmune Bullous Skin disorder Intensity Score (ABSIS) as outcome measures for BP in clinical trials. Thirty-two BP patients were repeatedly assessed over four years using Physician Global Assessment (PGA), anti-BP180 ELISA titres, BPDAI, ABSIS, BPDAI-Pruritus, Autoimmune Bullous Disease Quality of Life (ABQOL) and Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires. The reliability, validity, responsiveness, and minimal clinically important differences (MCIDs) were calculated. For inter-rater reliability, the intraclass correlation coefficients (95% CI) were: BPDAI 0.957 (0.901-0.982) and ABSIS 0.881 (0.736-0.949). Compared to ABSIS, BPDAI was better correlated with PGA(r = 0.875, p < 0.001), BPDAI-Pruritus (r=0.632, p = 0.004), ABQOL (r = 0.521, p = 0.011) and TABQOL (r=0.538, p = 0.008). MCIDs for BPDAI were 4-points for assessing clinical improvement and 3-points for deterioration. ABSIS demonstrated less responsiveness with MCIDs at 8.6-points for improvement and 4-points for deterioration. These results indicate that BPDAI demonstrated excellent reliability, validity and responsiveness, while ABSIS had moderate to good reliability, validity and responsiveness.

Advances in understanding and managing bullous pemphigoid

Bullous pemphigoid (BP) is the commonest subtype of autoimmune blistering disease in most countries of the world. It occurs most frequently in elderly patients and is characterised clinically by large, tense blisters in the skin preceded by urticarial plaques and pruritus. Immunopathologically, it is characterised by autoantibodies directed against the 180 kD antigen (BP180) and the 230 kD antigen (BP230). New knowledge regarding BP is being continually uncovered. This article reviews the recent advances in BP, including newer diagnostic tests, standardised outcome measures and emerging therapeutic options, as well as the evidence supporting their use.

Topical corticosteroid phobia in atopic dermatitis: International feasibility study of the TOPICOP score

Adherence to topical corticosteroids (TCS) is essential for the effective treatment of atopic dermatitis but can be limited by concerns about their use. This study examined the feasibility of applying the validated TOPICOP score for assessing TCS phobia across different countries.

Neonatal Autoimmune Blistering Disease: A Systematic Review

We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Exclusion criteria were age greater than 4 weeks and no confirmed AIBD diagnosis. We identified 51 cases of neonatal AIBDs: 34 cases of pemphigus (31 pemphigus vulgaris [PV], 3 pemphigus foliaceus [PF]) and 17 cases of pemphigoid diseases (9 bullous pemphigoid [BP], 5 linear immunoglobulin A bullous dermatosis [LABD], 1 BP and LABD, 1 epidermolysis bullosa acquisita, 1 bullous systemic lupus erythematosus). Pemphigoid diseases had a higher male predominance (male:female ratio 4.6:1) than pemphigus (male:female ratio 1:1.06) (p = 0.004). Pemphigus had a higher proportion presenting at birth (79.4%) than pemphigoid diseases (29.4%) (p = 0.008). The most common sites involved were the trunk (63.0%), followed by the head and neck (60.9%). The mucosal membranes were involved in 32.6% of cases (27.6% in pemphigus, 41.6% in pemphigoid diseases). Only 33.3% used systemic therapy, and 75.5% achieved control within 3 weeks. Most PV, PF, and BP cases, but not LABDs, reported maternal disease. In pemphigus cases, 75.0% of mothers had active disease and 25.0% were in control. Pregnant women with PV, PF, and PG of any severity can passively transfer autoantibodies leading to neonatal AIBD. Pemphigoid diseases are more likely to present after birth and may be more male predominant. The presentation of LABDs may be different from that of all other AIBDs.