Review of autoimmune blistering diseases: the Pemphigoid diseases.

Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. The classification and management is based on clinical, histological and direct and indirect immunofluorescence findings. There are, however, overlapping clinical and histological features between the conditions and clinical heterogeneity within each disease.

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

BACKGROUND: Atopic dermatitis (AD) assessment is more difficult in patients with skin of colour (SOC). OBJECTIVES: To compare the reliability of commonly used outcome measures for assessing AD in patients with SOC and to evaluate a novel greyscale in this population. METHODS: Twenty-five patients with AD each attended a 1-day scoring exercise based in either Sydney or Melbourne, Australia. Each patient was scored by the same five physicians using the Eczema Area and Severity Index (EASI), objective Scoring Atopic Dermatitis (oSCORAD), Investigator's Global Assessment (IGA) and a novel greyscale. Patients also completed the Patient-Oriented Eczema Measure and quality-of-life measures. A Mexameter was used to measure baseline melanin indices. Ten random patients were rescored to test intrarater reliability. RESULTS: We included 11 light-skinned patients (melanin index ≤ 200) and 14 patients with SOC (melanin index > 200) in the cohort. The inter-rater intraclass correlation coefficients (ICCs) were EASI 0·83 [95% confidence interval (CI) 0·66-0·94] for light skin and 0·77 (95% CI 0·60-0·91) for SOC; oSCORAD 0·68 (95% CI 0·44-0·88) for light skin and 0·74 (95% CI 0·54-0·89) for SOC; and IGA 0·80 (95% CI 0·62-0·93) for light skin and 0·70 (95% CI 0·49-0·87) for SOC. The greyscale had an ICC of 0·78 (95% CI 0·60-0·91) when replacing the EASI's erythema scale for patients with SOC. All scores showed excellent intrarater reliability for all skin types. Erythema component analysis showed that erythema did not contribute to variability. CONCLUSIONS: EASI showed excellent reliability for patients of all skin colours, and is recommended as the optimal core measure for patients with all skin colours.

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

BACKGROUND: There are multiple severity outcome measures for atopic dermatitis (AD). There is a need to compare the reliability of these measures. OBJECTIVES: To compare the inter-rater and intrarater reliability of the objective Scoring Atopic Dermatitis (oSCORAD), Eczema Area and Severity Index (EASI), Six Area, Six Sign Atopic Dermatitis (SASSAD) and Three Item Severity index (TIS); and to analyse the correlation between these outcome measures and the quality-of-life instruments Patient-Orientated Eczema Measurement, Dermatology Life Quality Index and Skindex-29. METHODS: Twelve patients with AD attended a 1-day scoring exercise by five trained dermatology clinicians. Inter-rater and intrarater reliability were assessed using the intraclass correlation coefficient (ICC). Correlation between clinician-rated and patient-reported measures was analysed using Spearman's rho. RESULTS: Regarding inter-rater reliability, EASI and SASSAD showed good reliabilities, with ICCs of 0·730 [95% confidence interval (CI) 0·500-0·900] and 0·680 (95% CI 0·440-0·880), respectively. However, the ICCs were poor for TIS and oSCORAD, with 0·497 (95% CI 0·233-0·785) and 0·498 (95% CI 0·234-0·785), respectively. Separate body surface area (BSA) component analyses showed that the oSCORAD BSA component contributed to its inter-rater variations. Regarding intrarater reliability, EASI and TIS showed excellent ICCs of 0·886 (95% CI 0·744-0·952) and 0·820 (0·614-0·923), respectively, while SASSAD showed a good reliability with an ICC of 0·720 (95% CI 0·424-0·878). However, the intrarater ICC was poor for oSCORAD, with 0·446 (95% CI 0·037-0·730). Regarding correlation with patient-reported measures, only SASSAD demonstrated moderate correlation with Skindex-29 (ρ = 0·611, P = 0·035). CONCLUSIONS: EASI demonstrated the highest inter-rater and intrarater reliability, supporting it as the optimal AD severity outcome measure.

The development and validation of the treatment of autoimmune bullous disease quality of life questionnaire, a tool to measure the quality of life impacts of treatments used in patients with autoimmune blistering disease.

BACKGROUND: Treatments for autoimmune blistering diseases have significant risk of medical complications and quality of life impacts during treatment, and it is difficult to differentiate these impacts from disease burden or the effects of treatment. OBJECTIVES: To develop a quality of life instrument specific to the effects of treatments used in patients with autoimmune bullous disease (AIBD). METHODS: A comprehensive item generation process was used to build a 45-item pilot Autoimmune Bullous Disease Quality of Life (ABQOL) questionnaire, distributed to 70 patients with AIBD. Experts in bullous disease refined the pilot ABQOL, selecting only those questions pertaining to the treatment effects. This pilot Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaire was administered to 70 patients, before factor analysis was performed to yield the final questionnaire of 17 questions. Validity and reliability were evaluated across a range of indices. RESULTS: Face and content validity were established through a comprehensive patient interview process, expert review and summaries of treatments used. The questionnaire was found to have appropriate correlation with the Dermatology Life Quality Index (r = 0.64) and the level of treatments used (P < 0.01), and was found to be responsive to overall variations in treatment burden. The TABQOL was also found to be a reliable instrument as evaluated by internal consistency (Cronbach α = 0.892) and test-retest reliability (r = 0.99). CONCLUSIONS: We have shown that the TABQOL questionnaire is a valid and reliable instrument that may to be used to measure treatment burden in AIBD and serve as an end point in clinical trials.

Squeezed light in an optical parametric oscillator network with coherent feedback quantum control.

We present squeezing and anti-squeezing spectra of the output from a degenerate optical parametric oscillator (OPO) network arranged in different coherent quantum feedback configurations. One OPO serves as a quantum plant, the other as a quantum controller. The addition of coherent feedback enables shaping of the output squeezing spectrum of the plant, and is found to be capable of pushing the frequency of maximum squeezing away from the optical driving frequency and broadening the spectrum over a wider frequency band. The experimental results are in excellent agreement with the developed theory, and illustrate the use of coherent quantum feedback to engineer the quantum-optical properties of the plant OPO output.

400 µJ 79 ns amplified pulses from a Q-switched fiber laser using an Yb(3+)-doped fiber saturable absorber.

We report a passively Q-switched all-fiber laser using a large mode area (LMA) Yb(3+)-doped fiber cladding-pumped at 915 nm and an unpumped single-mode Yb(3+)-doped fiber as the saturable absorber (SA). The saturable absorber fiber and gain fiber were coupled with a free-space telescope to optimize the coupling efficiency between the disparate fibers, preferentially bleaching the SA fiber before gain depletion in the pumped fiber. Using this scheme we first demonstrate a Q-switched oscillator with 40 µJ 79 ns pulses at 1026 nm, and show that pulses can be generated from 1020 nm to 1040 nm. The associated peak power of the oscillator alone is more than two orders of magnitude larger than that reported in previous experimental studies using an Yb(3+)-doped fiber as a saturable absorber. We further demonstrate an amplified pulse energy of 0.4 mJ using an Yb(3+)-doped cladding pumped fiber amplifier. Experimental studies in which the saturable absorber length, pump times, and wavelengths are independently varied reveal the impact of these parameters on laser performance.

Intravenous immunoglobulin: recent advances in the treatment of autoimmune bullous diseases.

Autoimmune bullous diseases describes a heterogenous group of diseases with skin and mucosal involvement with varying degrees of mortality. Intravenous immunoglobulin (IVIg) is a specialised treatment used in the management of these AIBD with variable success. This paper outlines the use of IVIg in these diseases.

High-power all-fiber passively Q-switched laser using a doped fiber as a saturable absorber: numerical simulations.

We report a design for a power-scalable all-fiber passively Q-switched laser that uses a large mode area Yb-doped fiber as a gain medium adiabatically tapered to an unpumped single-mode Yb-doped fiber, which serves as a saturable absorber. Through the use of a comprehensive numerical simulator, we demonstrate a passively Q-switched 1030 nm pulsed laser with 14 ns pulse duration and 0.5 mJ pulse energy operating at 200 kHz repetition rate. The proposed configuration has a potential for orders of magnitude of improvement in both the pulse energies and durations compared to the previously reported result. The key mechanism for this improvement relates to the ratio of the core areas between the pumped inverted large mode area gain fiber and the unpumped doped single-mode fiber.

The effect of dispersion on spectral broadening of incoherent continuous-wave light in optical fibers.

In addition to fiber nonlinearity, fiber dispersion plays a significant role in spectral broadening of incoherent continuous-wave light. In this paper we have performed a numerical analysis of spectral broadening of incoherent light based on a fully stochastic model. Under a wide range of operating conditions, these numerical simulations exhibit striking features such as damped oscillatory spectral broadening (during the initial stages of propagation), and eventual convergence to a stationary, steady state spectral distribution at sufficiently long propagation distances. In this study we analyze the important role of fiber dispersion in such phenomena. We also demonstrate an analytical rate equation expression for spectral broadening.

The actual management of pemphigus.

Pemphigus is usually treated with systemic prednisone in combination with adjuvant immunosuppressants. Though the mortality associated with pemphigus has been reduced with the use of systemic corticosteroids, the iatrogenic complications have warranted the development of newer therapies. This review discusses the different treatments, therapies and procedures.