Describing skin health and disease in urban-living Aboriginal children: co-design, development and feasibility testing of the Koolungar Moorditj Healthy Skin pilot project.

BACKGROUND: Indigenous children in colonised nations experience high rates of health disparities linked to historical trauma resulting from displacement and dispossession, as well as ongoing systemic racism. Skin infections and their complications are one such health inequity, with the highest global burden described in remote-living Australian Aboriginal and/or Torres Strait Islander (hereafter respectfully referred to as Aboriginal) children. Yet despite increasing urbanisation, little is known about the skin infection burden for urban-living Aboriginal children. More knowledge is needed to inform service provision, treatment guidelines and community-wide healthy skin strategies. In this pilot study, we aimed to test the feasibility and design of larger multi-site observational studies, provide initial descriptions of skin disease frequency and generate preliminary hypotheses of association. METHODS: This project has been co-designed with local (Noongar) Elders to provide an Australian-first description of skin health and disease in urban-living Aboriginal children. In collaboration with an urban Aboriginal Community Controlled Health Organisation (Derbarl Yerrigan Health Service), we conducted a week-long cross-sectional observational cohort study of Aboriginal children (0-18 years) recruited from the waiting room. Participants completed a questionnaire, skin examination, clinical photos, and swabs and received appropriate treatment. We assessed the feasibility and impact of the pilot study. RESULTS: From 4 to 8 October 2021, we recruited 84 Aboriginal children of whom 80 (95%) were urban-living. With a trusted Aboriginal Health Practitioner leading recruitment, most parents (or caregivers) who were approached consented to participate. Among urban-living children, over half (45/80, 56%) of parents described a current concern with their child's skin, hair and/or nails; and one-third (26/80, 33%) reported current itchy skin. Using a research-service model, 27% (21/79) of examined urban-living participants received opportunistic same-day treatment and 18% (14/79) were referred for later review. CONCLUSIONS: This co-designed pilot study to understand skin health in urban-living Aboriginal children was feasible and acceptable, with high study participation and subsequent engagement in clinical care observed. Co-design and the strong involvement of Aboriginal people to lead and deliver the project was crucial. The successful pilot has informed larger, multi-site observational studies to more accurately answer questions of disease burden and inform the development of healthy skin messages for urban-living Aboriginal children.

Dermatological aspects of ticks in Australia: An update.

Ticks are ectoparasites that cause dermatologic reactions directly by their bites and indirectly as vectors of bacterial, protozoal and viral diseases. Consequences vary from minor local reactions to significant systemic sequelae and are therefore of clinical relevance to dermatologists. In this article, Australian ticks of medical importance are reviewed through the lens of dermatology.

The burden of atopic dermatitis and bacterial skin infections among urban-living Indigenous children and young people in high-income countries: A systematic review.

BACKGROUND: A high burden of bacterial skin infections (BSI) is well documented in remote-living Indigenous children and young people (CYP) in high-income countries (HIC). Atopic dermatitis (AD) is the most common chronic inflammatory skin condition seen in CYP and predisposes to BSI. Despite the rate of urbanization for Indigenous people increasing globally, research is lacking on the burden of AD and BSI for urban-living Indigenous CYP in HIC. Indigenous people in HIC share a history of colonization, displacement and subsequent ongoing negative impacts on health. OBJECTIVE: To provide a global background on the burden of AD and BSI in urban-living Indigenous CYP in HIC. METHODS: A systematic review of primary observational studies on AD and BSI in English containing epidemiologic data was performed. MEDLINE, EMBASE, EMCARE, Web of Science, and PubMed databases were searched for articles between January 1990 and December 2021. RESULTS: From 2278 original manuscripts, 16 were included: seven manuscripts documenting eight studies on AD; and nine manuscripts documenting nine studies on BSI. Current and severe symptoms of AD were more common in urban-living Indigenous CYP in HIC compared with their non-Indigenous peers, with children having a higher prevalence than adolescents. Urban-living Indigenous CYP in HIC had a higher incidence of all measures of BSI compared with their non-Indigenous peers, and were over-represented for all measures of BSI compared with their proportion of the background population. Limitations include incomplete representation of all Indigenous populations in HIC. CONCLUSION: A significant burden of AD and BSI exists in urban-living Indigenous CYP in HIC.

The Troublesome Ticks Research Protocol: Developing a Comprehensive, Multidiscipline Research Plan for Investigating Human Tick-Associated Disease in Australia.

In Australia, there is a paucity of data about the extent and impact of zoonotic tick-related illnesses. Even less is understood about a multifaceted illness referred to as Debilitating Symptom Complexes Attributed to Ticks (DSCATT). Here, we describe a research plan for investigating the aetiology, pathophysiology, and clinical outcomes of human tick-associated disease in Australia. Our approach focuses on the transmission of potential pathogens and the immunological responses of the patient after a tick bite. The protocol is strengthened by prospective data collection, the recruitment of two external matched control groups, and sophisticated integrative data analysis which, collectively, will allow the robust demonstration of associations between a tick bite and the development of clinical and pathological abnormalities. Various laboratory analyses are performed including metagenomics to investigate the potential transmission of bacteria, protozoa and/or viruses during tick bite. In addition, multi-omics technology is applied to investigate links between host immune responses and potential infectious and non-infectious disease causations. Psychometric profiling is also used to investigate whether psychological attributes influence symptom development. This research will fill important knowledge gaps about tick-borne diseases. Ultimately, we hope the results will promote improved diagnostic outcomes, and inform the safe management and treatment of patients bitten by ticks in Australia.

Late-onset eccrine syringofibroadenoma of the feet in a patient with hypohidrotic ectodermal dysplasia.

A 56-year-old woman with hypohidrotic ectodermal dysplasia presented with a 10-year history of persisting wart-like skin lesions on her feet. Biopsy revealed changes of eccrine syringofibroadenoma. These lesions are rare, with only nine case reports describing an association with ectodermal dysplasia of hidrotic type (Clouston and Schopf's syndrome). To our knowledge, this is the first case of eccrine syringofibroadenoma developing in the hypohidrotic/anhidrotic subtype of ectodermal dysplasia.

Validation of a physician global assessment tool for vitiligo extent: Results of an international vitiligo expert meeting.

Currently, vitiligo lacks a validated Physician Global Assessment (PGA) for disease extent. This PGA can be used to stratify and interpret the numeric scores obtained by the Vitiligo Extent Score (VES). We investigated the interrater reliability of a 5-point PGA scale during an international vitiligo workshop. Vitiligo experts from five different continents rated photographs of non-segmental vitiligo patients with varying degrees of extent with the PGA score. Good interrater agreements (intraclass correlation coefficient >0.6) were observed between the raters overall and within each continent. All hypotheses to evaluate construct validity were confirmed. Median VES values per category were for limited 1.10 [IQR: 0.21-1.67], moderate 3.17 [IQR: 1.75-6.21], extensive 9.58 [IQR: 6.21-13.03] and very extensive 42.67 [IQR: 21.20-42.67]. Defined categories for vitiligo extent can be valuable for inclusion criteria and may impact future reimbursement criteria.

Melanocyte abnormalities and senescence in the pathogenesis of idiopathic guttate hypomelanosis.

BACKGROUND: Idiopathic guttate hypomelanosis (IGH) is a pigmentary disorder of unknown pathogenesis characterized by small discrete white macules. In the skin, epidermal melanin unit between melanocytes and keratinocytes is responsible for melanin synthesis and equal distribution of melanin pigment. OBJECTIVE: Therefore, this study was designed to check the role of melanocytes in the pathogenesis of IGH. METHODS: For this study, six IGH patients and six controls were enrolled. Melanin content was checked in the skin sections and in the cultured melanocytes. Senescence was checked in the lesional skin of IGH patients by comparing the mRNA and protein expression of senescence markers p16, hp1, and p21. RESULTS: Cultured melanocytes from the IGH patients showed morphological changes in comparison to the control melanocytes. Melanocytes from IGH patients were bigger in size with very small and retracted dendrites as compared to the control melanocytes. Melanin accumulation was more in the IGH patients as compared to the controls. Our results showed that expression of p16, p21, and hp1 was significantly higher in lesional skin of IGH patient as compared to healthy controls. CONCLUSION: This study revealed large-sized melanocytes with small and retracted dendrites in IGH patients. Accumulation of more melanin in the IGH melanocytes might be due to problem in the transfer of melanin from melanocytes to keratinocytes. Accumulation of melanin can lead to the senescence in the melanocytes of IGH patients.

Repigmentation in vitiligo: position paper of the Vitiligo Global Issues Consensus Conference.

The Vitiligo Global Issues Consensus Conference (VGICC), through an international e-Delphi consensus, concluded that 'repigmentation' and 'maintenance of gained repigmentation' are essential core outcome measures in future vitiligo trials. This VGICC position paper addresses these core topics in two sections and includes an atlas depicting vitiligo repigmentation patterns and color match. The first section delineates mechanisms and characteristics of vitiligo repigmentation, and the second section summarizes the outcomes of international meeting discussions and two e-surveys on vitiligo repigmentation, which had been carried out over 3 yr. Treatment is defined as successful if repigmentation exceeds 80% and at least 80% of the gained repigmentation is maintained for over 6 months. No agreement was found on the best outcome measure for assessing target or global repigmentation, therefore highlighting the limitations of e-surveys in addressing clinical measurements. Until there is a clear consensus, existing tools should be selected according to the specific needs of each study. A workshop will be conducted to address the remaining issues so as to achieve a consensus.

Epidermolysis bullosa acquisita with moderately severe Dysphagia due to esophageal strictures.

Epidermolysis bullosa acquisita (EBA) is a chronic, autoimmune condition involving the skin and mucous membranes. Symptomatic mucosal involvement is rare, but can impact on quality of life, due to esophageal strictures and dysphagia. We report a case involving a 60-year-old male presenting with bullous skin lesions on areas of friction on his hands, feet and mouth. Milia were visible on some healed areas. Biopsy showed a subepidermal vesicle. Direct immunofluorescence showed intense linear junctional IgG and C3 at the dermo-epidermal junction. Serological tests also supported the diagnosis of EBA. Screening tests for underlying malignancies were negative. Despite treatment with systemic steroids, the patient developed increasing dysphagia, requiring further investigation with esophagoscopy and a barium swallow. Confirmation of extensive esophageal stricturing prompted adjustment of medications including an increase in systemic steroids and addition of azathioprine. Currently, the patient's disease remains under control, with improvement in all his symptoms and return of anti-basement membrane antibody levels to normal, whilst he remains on azathioprine 150 mg daily and prednisolone 5 mg daily. This case highlights the fact that the treatment of a given patient with EBA depends on severity of disease and co-morbid symptoms. Newer immunoglobulin and biological therapies have shown promise in treatment resistant disease. Considering that long-term immunosuppressants or biologicals will be required, potential side effects of the drugs should be considered. If further deterioration occurs in this patient, cyclosporin A or intravenous immunoglobulin (IV Ig) will be considered. Vigilance for associated co-morbidities, especially malignancies, should always be maintained.

Late-stage human African trypanosomiasis in a Sudanese refugee.

A 19-year-old Sudanese woman, who had lived for about a decade in Ugandan refugee camps, was referred for investigation of a 12-month history of a generalised rash. Two months later, her condition had deteriorated to include cachexia and drowsiness. Despite initial negative findings on investigation, human African trypanosomiasis (HAT) was suspected, and parasites were found in a double-centrifuged sample of cerebrospinal fluid. Eflornithine, the appropriate drug for treatment of late-stage disease, was obtained through the World Health Organization. This case highlights the diagnostic and therapeutic difficulties in managing late-stage HAT in a non-endemic country.

Giant basal cell carcinoma masquerading as an osteogenic sarcoma.

An 88-year-old man presented to the dermatology outpatient clinic with an 11-month history of a rapidly growing mass overlying a clavicular fracture site. The lesion was 8 x 6 cm, painful, fixed to deeper structures and ulcerated. Superficial and deep biopsies yielded invasive basal cell carcinoma. Imaging demonstrated extensive soft tissue invasion into muscle, bone and potentially into the lung parenchyma. Due to complications arising from subsequent diagnostic procedures, the patient declined further invasive tests. The cutaneous lesion was treated with palliative radiotherapy. We explore the literature regarding the tumorigenic effects of peri-fracture cytokines on the biological behaviour of basal cell neoplasms.

Melanization in basal cell carcinomas: microscopic characterization of clinically pigmented and non-pigmented tumours.

Clinical and microscopic pigmentation may affect the treatment outcomes in basal cell carcinoma. However, there have not been any in-depth histopathological comparisons between clinically pigmented and non-pigmented basal cell carcinomas with regards to microscopic melanization. The aims of our study were to determine the proportion of pigmented basal cell carcinomas presenting to the National Skin Centre in Singapore, to characterize the histological pattern of melanization and to perform a semi-quantitative analysis of the degree of microscopic melanization of the tumours. Patients with clinical features and histologically confirmed basal cell carcinomas were recruited. Demographic data and clinical characteristics were recorded and basal cell carcinoma sections were examined for histological subtype and pattern of melanization. Twenty-five Chinese patients with 30 basal cell carcinomas were recruited. Three of the five clinically non-pigmented and all of the clinically pigmented basal cell carcinomas had microscopic evidence of melanization. Microscopic melanization in clinically non-pigmented basal cell carcinomas was present only focally or in the centre of the tumour mass. Both groups of basal cell carcinomas may be colonized by melanocytes. Two morphological types of melanocytes, a dendritic and round cell type, were identified. Future research is required to evaluate if the degree of microscopic melanization influences the treatment outcome of basal cell carcinomas.