Adult-onset Still's disease (AOSD) is a rare autoinflammatory systemic disorder classically characterised by inflammatory polyarthritis, daily fevers and a transient asymptomatic salmon-pink maculopapular rash that typically arises with the onset of fevers. We report a case of AOSD presenting with a severely pruritic urticarial eruption starting 6 weeks prior to the onset of fever and arthritis and complicated by macrophage activation syndrome. This case highlights the importance of early recognition of diverse cutaneous manifestations of AOSD to facilitate timely diagnosis and treatment to improve disease outcomes.
View Article and Find Full Text PDFJ Low Genit Tract Dis
December 2024
Objectives: This case series aims to evaluate the demographic features, disease characteristics, and treatment outcomes of 8 patients receiving subcutaneous (SC) adalimumab for severe, refractory vulval lichen sclerosus (VLS) and/or vulval lichen planus (VLP). Both conditions are chronic inflammatory dermatoses that significantly impair quality of life, and although first-line treatment typically involves potent to ultrapotent topical corticosteroids, managing severe cases is challenging due to a lack of FDA-approved systemic therapies. Adalimumab, a TNF-α inhibitor, may offer a promising alternative by targeting the inflammatory cytokine implicated in the pathogenesis of both conditions.
View Article and Find Full Text PDFPyoderma gangrenosum is a rare, autoinflammatory disorder characterized by rapidly progressive painful ulcers that are challenging to diagnose and treat. This qualitative study aimed to explore the experiences of patients living with pyoderma gangrenosum. Using an inductive qualitative approach, semi-structured interviews were completed with a purposive sample of 21 patients with pyoderma gangrenosum recruited from a public dermatology outpatient clinic in Melbourne, Australia.
View Article and Find Full Text PDFHidradenitis Suppurativa is a burdensome inflammatory skin disease with significant quality of life impact. These management guidelines were developed to direct appropriate clinical management in the Australasian context. A systematic review was used for the basis of the consensus guidelines.
View Article and Find Full Text PDFBackground: Hidradenitis suppurativa (HS) is a debilitating chronic inflammatory disease with significant impact upon quality of life. Generic quality-of-life measures suffer from decreased face validity and content validity, leading to the development of disease-specific quality-of-life measures such as the Hidradenitis Suppurativa Quality of Life (HiSQOL) outcome measure. The aim of this study was to validate the use of the HiSQOL in the Australian population and evaluate the quality-of-life impact in HS patients in Australia.
View Article and Find Full Text PDFObjectives: It is common practice to advise people with vulvar lichen sclerosus to perform vulvar self-examination (VSE) to optimize topical therapy and detect changes that may represent active disease, scarring, and/or squamous cell carcinoma development. The aim of this study was to better understand people's willingness and potential barriers to performing VSE.
Materials And Methods: A cross-sectional survey was undertaken of all patients with vulvar LS presenting to a tertiary referral vulvar dermatology clinic in Australia, from June 2022 to January 2023.
Australas J Dermatol
August 2023
Objectives: Plasma cell vulvitis (PCV) is a rare inflammatory vulvar condition. The aim of this study was to describe the natural history, treatment, impact on quality of life, and factors associated with poorer outcomes for PCV.
Methods: A mixed-methods approach was used combining a retrospective case note review with a cross-sectional telephone questionnaire.
This letter summarizes the outcomes from an anonymous survey conducted among Australian dermatologists. It provides a comparison of Australian isotretinoin prescribing practices against best available evidence and guidelines in the UK, Europe and USA.
View Article and Find Full Text PDFBackground/objectives: Vulval lichen sclerosus (VLS) is a chronic inflammatory skin condition predominantly affecting the anogenital region in women and children. To date, there is lack of agreement amongst experts on a severity scale to aid assessment, research and treatment stratification on VLS. Furthermore, literature on best practice for long-term management of VLS is lacking.
View Article and Find Full Text PDFObjectives: Plasma cell vulvitis (PCV) is a rare chronic inflammatory disorder, where the symptoms can be severe and may affect patient's quality of life. However, there are currently no evidence-based treatment guidelines. The aims of this systematic review were to evaluate efficacy of individual treatments and to inform future research.
View Article and Find Full Text PDFBackground/objectives: The Vulval disease Quality of Life Index (VQLI) is a new tool that assesses the burden of vulval disease on quality of life (QoL). Our objective was to assess the correlation between VQLI score and clinician-rated severity scores, overall patient itch/discomfort, disease duration, sexual activity, and age, in vulval lichen sclerosus (VLS) at a vulval disorders clinic.
Methods: A retrospective case note review, including consecutive women with VLS who attended the clinic between April and October 2018.