The Australasian Registry for Severe Cutaneous Adverse Reactions (AUS-SCAR) - Providing a roadmap for closing the diagnostic, patient, and healthcare gaps for a group of rare drug eruptions.

Background: Severe cutaneous adverse reactions (SCAR) are a group of delayed presumed T-cell mediated hypersensitivities associated with significant morbidity and mortality. Despite their shared global healthcare burden and impact, the clinical phenotypes, genomic predisposition, drug causality, and treatment outcomes may vary. We describe the establishment and results from the first Australasian registry for SCAR (AUS-SCAR), that via a collaborative network advances strategies for the prevention, diagnosis and treatment of SCAR. Methods: Prospective multi-center registry of SCAR in Australian adult and adolescents, with planned regional expansion. The registry collects externally verified phenotypic data drug causality, therapeutics and long-term patient outcomes. In addition, biorepository specimens and DNA are collected at participating sites. Results: we report on the first 100 patients enrolled in the AUS-SCAR database. DRESS (50%) is the most predominant phenotype followed by SJS/TEN (39%) and AGEP (10%), with median age of 52 years old (IQR 37.5, 66) with 1:1 male-to-female ratio. The median latency for all implicated drugs is highly variable but similar for DRESS (median 15 days IQR 5,25) and SJS/TEN (median 21 days, IQR 7,27), while lowest for AGEP (median 2.5 days, IQR 1,8). Antibiotics (54.5%) are more commonly listed as primary implicated drug compare with non-antibiotics agent (45.5%). Mortality rate at 90 days was highest in SJS/TEN at 23.1%, followed by DRESS (4%) and AGEP (0%). Conclusion: In the first prospective national phenotypic and biorepository of SCAR in the southern hemisphere we demonstrate notable differences to other reported registries; including DRESS-predominant phenotype, varied antibiotic causality and low overall mortality rate. This study also highlights the lack of standardised preventative pharmacogenomic measures and in vitro/in vivo diagnostic strategies to ascertain drug causality. Trial registration: ANZCTR ACTRN12619000241134. Registered 19 February 2019.

Calcinosis Cutis Associated with Chronic Sclerodermoid Graft versus Host Disease: A Case and Review of the Literature.

We present a rare case of calcinosis cutis associated with chronic sclerodermoid graft versus host disease in a 59-year-old male, 13 years following allogenic bone marrow transplantation. The etiology of calcification was thought to be dystrophic. Further research is needed to understand the link between calcinosis cutis and chronic sclerodermoid graft versus host disease to assist with selecting appropriate management for these patients.

Bone marrow transplant recipients and their compliance with sun-protection measures in Queensland, Australia.

There is no current published work on the sun-protection practices of bone marrow transplant recipients. The burden of post-transplant skin malignancy is a growing public health concern. This paper evaluates the compliance of bone marrow transplant recipients with advice about sun protection through a cross-sectional observational study involving participants completing a questionnaire regarding sun protection. Whilst most bone marrow transplant patients recalled receiving education (94%) and understood why they required additional precautions (84%), half did not practice adequate photoprotection. Future research should explore barriers to non-compliance with sun-protection measures in bone marrow transplant patients so additional interventions can be more targeted within this population.

Candida albicans colonisation, continence status and incontinence-associated dermatitis in the acute care setting: a pilot study.

Candida albicans is the most prevalent human fungal commensal organism and is reported to be the most frequent aetiological organism responsible for infection associated with incontinence-associated dermatitis. However, it remains unclear whether incontinence predisposes a patient to increased Candida colonisation or whether incontinence acts as a trigger for Candida infection in those already colonised. The purpose of this observational cross-sectional study was to estimate colonisation rates of C. albicans in continent, compared to incontinent patients, and patients with incontinence-associated dermatitis. Data were collected on 81 inpatients of a major Australian hospital and included a pelvic skin inspection and microbiological specimens to detect C. Albicans at hospital admission. The mean age of the sample was 76 years (SD = 12.22) with 53% being male. Incontinent participants (n = 53) had a non-significant trend towards greater Candida colonisation rates at the perianal site (43% versus 28%) χ2 (1, N = 81) = 4·453, p = ·638 and the inguinal site (24% versus 14%) χ2 (1, N = 81) = 6·868, p = ·258 compared to continent patients (n = 28). The incontinent subgroup with incontinence-associated dermatitis (n = 22) showed no difference in colonisation rates compared to those without incontinence-associated dermatitis. Understanding the epidemiology of colonisation may have implications for the prevention of Candida infection in these patients.

Heterogeneity of blastomycosis-like pyoderma: A selection of cases from the last 35 years.

Blastomycosis-like pyoderma is a form of pyoderma with variable clinical findings and histopathological features. We present a case series of 39 patients collected over a 35-year period to demonstrate its clinical features and histological findings. The most common clinical presentations found were solitary plaques, solitary nodules, sinuses, crypts, verrucous plaques and discharge, usually on sun-exposed skin. The most common histopathological findings were chronic granulomatous inflammation, suppurative inflammation, sinus and abscess formation, pseudoepitheliomatous hyperplasia, transepidermal elimination and scarring. We discuss its treatment and the recent literature that has focused on its response to acitretin.

Acute cutaneous graft-versus-host disease resembling type II (atypical adult) pityriasis rubra pilaris.

We present a case of cutaneous acute graft-versus-host disease (aGVHD) with confluent erythematous perifollicular hyperkeratosis and ichthyosiform scale in the clinical pattern of type II (atypical adult) pityriasis rubra pilaris (PRP), which developed 26 days after allogeneic peripheral blood stem cell transplant. Skin histology confirmed features of both aGVHD and PRP. The skin lesions were refractory to oral prednisolone and cyclosporine and only partially responsive to a combination of i.v. methylprednisolone, oral tacrolimus, oral mycophenolate mofetil, and infusions of anti-thymocyte globulin and the tumour necrosis factor-α inhibitor, etanercept.

Warfarin-induced erythroderma.

Erythroderma is a potentially serious and life-threatening skin disease with a number of possible aetiologies. Drug reactions are well-documented causes, with carbamazepine, penicillin and allopurinol being the most commonly implicated. This case describes a unique presentation of warfarin-induced erythroderma in a 78-year-old female patient.

Origin of Langerhans cells in normal skin and chronic GVHD after hematopoietic stem-cell transplantation.

Chronic graft-versus-host disease (cGVHD) is a common complication following allogeneic stem-cell transplantation (SCT). Past studies have implicated the persistence of host antigen-presenting cells (APCs) in GVHD. Our objective was to determine the frequency of host Langerhans cells (LCs) in normal skin post-SCT and ask if their persistence could predict cGVHD. Biopsies of normal skin from 124 sex-mismatched T-cell-replete allogenic SCT recipients were taken 100 days post-transplant. Patients with acute GVHD and those with <9 months of follow-up were excluded and prospective follow-up information was collected from remaining 22 patients. CD1a staining and X and Y chromosome in-situ hybridization were performed to label LCs and to identify their host or donor origin. At 3 months, 59 ± 5% of LCs were host derived. The density of LCs and the proportion of host-derived LCs were similar between patients that did or did not develop cGVHD. Most LCs in the skin remained of host origin 3 months after SCT regardless of cGVHD status. This finding is in line with the redundant role of LCs in acute GVHD initiation uncovered in recent experimental models.

Irritant bullous contact dermatitis caused by a rove beetle: an illustrated clinical course.

A 26-year-old Australian female traveller in Sierra Leone presented with an irritant bullous contact dermatitis consistent with paederus dermatitis. The lesions were treated with a potent topical corticosteroid with good effect. The affected area resolved in 6 weeks and hyperpigmention persisted for months until complete resolution. This dermatitis occurs when beetles of the genus Paederus (rove beetles) are crushed on the skin, releasing pederin. The same dermatitis ensues with Australian Paederus species. Serial clinical photographs are presented which will aid Australian dermatologists in the diagnosis of this dermatitis, which presents in regional Australian patients and returned overseas travellers.

Poor health but not absent: prevalence, predictors, and outcomes of presenteeism.

OBJECTIVE: The objective of this study was to examine the prevalence of presenteeism, to develop and test a model of the relationship between workplace factors and presenteeism, and to assess the perceived influence of manager, coworkers, and self on presenteeism. METHODS: We used survey data collected for 6309 employees from seven different organizations. RESULTS: Nearly 60% of the sample reported presenteeism during a 3-month period. The model was supported, with presenteeism linking workplace factors and health outcomes to productivity, as predicted. The majority of participants (67%) indicated that the primary pressure to attend work while sick came from themselves. A substantial minority (20%) also indicated the manager as a source of pressure. CONCLUSIONS: Psychosocial workplace factors are predictive of presenteeism, and efforts to control them, including the use of more effective management, may impact presenteeism rates and the resulting levels of productivity.

Cutaneous hypersensitivity reactions to freshwater cyanobacteria--human volunteer studies.

BACKGROUND: Pruritic skin rashes associated with exposure to freshwater cyanobacteria are infrequently reported in the medical and scientific literature, mostly as anecdotal and case reports. Diagnostic dermatological investigations in humans are also infrequently described. We sought to conduct a pilot volunteer study to explore the potential for cyanobacteria to elicit hypersensitivity reactions. METHODS: A consecutive series of adult patients presenting for diagnostic skin patch testing at a hospital outpatient clinic were invited to participate. A convenience sample of volunteers matched for age and sex was also enrolled. Patches containing aqueous suspensions of various cyanobacteria at three concentrations were applied for 48 hours; dermatological assessment was made 48 hours and 96 hours after application. RESULTS: 20 outpatients and 19 reference subjects were recruited into the study. A single outpatient produced unequivocal reactions to several cyanobacteria suspensions; this subject was also the only one of the outpatient group with a diagnosis of atopic dermatitis. No subjects in the reference group developed clinically detectable skin reactions to cyanobacteria. CONCLUSION: This preliminary clinical study demonstrates that hypersensitivity reactions to cyanobacteria appear to be infrequent in both the general and dermatological outpatient populations. As cyanobacteria are widely distributed in aquatic environments, a better appreciation of risk factors, particularly with respect to allergic predisposition, may help to refine health advice given to people engaging in recreational activities where nuisance cyanobacteria are a problem.

Older people's well-being as a function of employment, retirement, environmental characteristics and role preference.

The life satisfaction and affective well-being of employed, unemployed and retired men and women aged between 50 and 74 were examined as a function of characteristics of their environment and the degree to which their current role was personally preferred. Early-retired and late-employed individuals had particularly high affective well-being. Role preference (e.g. to be in a job) was significantly associated with both indicators, with better well-being in those individuals who wanted to be in their current role. Both forms of well-being were a function of the features experienced in a role (opportunity for control, clarity, etc.), over and above the identification of role membership on its own, with the relationship between older people's role occupancy (employed, unemployed or retired) and well-being being mediated by perceived environmental characteristics.

Progression of a myxoid pleomorphic fibroma to myxofibrosarcoma.

A 75-year-old man presented with a protuberant stable lesion of many years on his left second toe. Histology on an incisional biopsy showed myxoid pleomorphic fibroma. Over the subsequent 21 months, the lesion enlarged and became exophytic and irregular. Repeat biopsy showed features consistent with a grade III myxofibrosarcoma. Treatment involved amputation of his toe. Six months later he remains well, with no evidence of local recurrence or systemic spread.

Birt-Hogg-Dubé syndrome.

We present three members of a Queensland family with clinical and histopathological features consistent with Birt-Hogg-Dubé syndrome. Two of the three family members were able to be screened for associated disorders. The mother of the family was found to have a solitary colonic polyp, a large ovarian cyst and two chorioretinal scars. No associated disorders were found on investigation of one of the two affected sons.

Disseminated lobular capillary haemangioma.

A 58-year-old woman presented with a 5-week history of multiple widespread vascular lesions. Histological examination confirmed the clinical diagnosis of disseminated lobular capillary haemangioma. No underlying cause for this eruption could be found. The lesions resolved over the following 2 months without intervention. Reports of disseminated lobular capillary haemangioma are scarce in the literature. In general, this condition does not appear to be related to an underlying disorder and in particular is not a marker for underlying malignancy. No unifying concept as to the aetiology of disseminated lobular capillary haemangioma has been identified. The condition appears to follow a benign course, with spontaneous resolution over 6-12 months.