Pyodermatitis pyostomatitis vegetans is a rare variant of neutrophilic disorders, affecting the intertriginous areas such as axilla and groin, umbilicus, as well as the oral mucosa. We herein describe 2 female patients, in both of whom the umbilicus was restrictedly involved.
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.
A total of 301 patients were recruited and consented to be involved in this study. All participants were invited to complete the HiSQOL questionnaire along with basic demographic and disease information, the DLQI and HADS anxiety and depression scale. Participants were then asked to repeat the questionnaires 14 days later to assess test–retest reliability.
The mean HiSQOL score was 46.5 out of a possible total score of 76 (SD = 24.2) indicating a very large impact on quality of life. Based upon the published validity bands this corresponds to a very large impact on quality of life. Validation statistics indicated a high degree of internal consistency (Cronbach's alpha = 0.956) with expected levels of convergent validity and excellent test–retest reliability (p = 0.95). Multiple regression analysis indicated individuals with a younger age of onset and positive family history had significantly greater quality-of-life impact as measured by the HiSQOL.
The HiSQOL is a valid, reliable measure for assessing the quality-of-life impact of HS. Significant factors influencing quality of life include age of onset and family history. Longitudinal measurements will enable evaluation of the impact of therapy upon QOL in the Australian context.
Sirolimus is being increasingly employed to manage specific vascular anomalies. We performed an exploratory cost-utility analysis to evaluate sirolimus as a treatment for vascular malformations from the Australian healthcare system perspective. Over a one-year time horizon, sirolimus treatment was associated with an increased expenditure of AU$2832.80 and a gain of 0.08 quality-adjusted life years (QALYs) when compared to supportive care, resulting in an incremental cost-effectiveness ratio of AU$35,410/QALY. By most metrics, sirolimus would be considered a cost-effective treatment for vascular malformations.
Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma characterized by an asymmetric, infiltrative growth pattern and a high risk of local recurrence. This study aims to evaluate the effectiveness of various imaging modalities in the assessment and management of DFSP. Nine imaging modalities were reviewed including: Ultrasound (US), High-Frequency Doppler Ultrasound (HFUS), Computed tomography (CT), Positron emission tomography–computed tomography (PET-CT), and Magnetic Resonance Imaging (MRI), High-resolution-MRI (HR-MRI), Magnetic Resonance Spectroscopy (MRS), Optical Coherence Tomography (OCT), and Dermatoscopy. Imaging is mainly used for preoperative assessment and surgical planning, not routine diagnosis. US is effective for initial evaluations, demonstrating superior ability in detecting muscle invasion and defining tumour boundaries (sensitivity – 81.8%, specificity – 100%). MRI is valuable for preoperative evaluation, surgical planning, and monitoring DFSP recurrence. It more accurately assesses tumour depth than palpation, with a sensitivity of 67% and specificity of 100%, but was inferior when compared to US. CT is utilized in cases of suspected bone involvement or pulmonary metastasis. For advanced or recurrent DFSP, PET-CT helps manage treatment responses and imatinib therapy. Emerging technologies like MRS and OCT show potential in improving diagnostic accuracy and defining surgical margins, though more data are needed. US, MRI, and CT are the primary imaging modalities for DFSP. Emerging technologies like HR-MRI, PET-CT, MRS, and OCT hold promise for refining diagnostic and management strategies. Integrating multiple technologies could enhance management, particularly in atypical or aggressive cases. Further studies are required to refine imaging protocols and improve DFSP outcomes.
Apremilast is a relatively new oral treatment for psoriasis, which reduces expression of pro-inflammatory factors, including tumour necrosis factor-α (TNFα), critical to the immune control of Mycobacterium tuberculosis infection. In randomised controlled trials (RCTs) for apremilast no new cases of active tuberculosis (TB) were identified, thus, screening for latent TB infection (LTBI) is not currently recommended prior to apremilast initiation. We describe a case of M.tuberculosis reactivation shortly after commencement of apremilast for psoriasis. We are recommending clinicians perform LTBI risk assessment in all patients, and appropriate LTBI screening in select populations prior to apremilast initiation.
A survey of Mohs surgery specialists in Australia showed diazepam was the preferred agent and felt to be the safest oral benzodiazepine for perioperative anxiolysis.
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