Skin disorders are prominent features in primary immunodeficiency diseases: A systematic overview of current data.

Primary immunodeficiency diseases (PIDs) are characterized by an increased risk of infections, autoimmunity, autoinflammation, malignancy, and allergic disorders. Skin disorders are also common clinical features in PIDs and may be among the presenting manifestations. Recognition of specific PID-associated skin conditions in combination with other clinical features as described in the currently used warning signs could raise suspicion of an underlying PID. We aimed to provide a systematically obtained overview of skin disorders and their prevalence in PIDs. Secondary, the prevalence of Staphylococcus (S.) aureus-associated skin disorders and atopy was reviewed, as these are the most prominent skin features in PIDs. A systematic search was performed in EMBASE, MEDLINE, Web of Science, Cochrane, and Google Scholar (up to May 9, 2018). All original observational and experimental human studies that address the presence of skin disorders in PIDs were selected. We rated study quality using the Institute of Health Economics Quality Appraisal Checklist for Case Series Studies. Sixty-seven articles (5030 patients) were included. Study quality ranged from 18.2% to 88.5%. A broad spectrum of skin disorders was reported in 30 PIDs, mostly in single studies with a low number of included patients. An overview of associated PIDs per skin disorder was generated. Data on S. aureus-associated skin disorders and atopy in PIDs were limited. In conclusion, skin disorders are prominent features in PIDs. Through clustering of PIDs per skin disorder, we provide a support tool to use in clinical practice that should raise awareness of PIDs based on presenting skin manifestations.

Trends in incidence rate, health care consumption, and costs for patients admitted with a humeral fracture in The Netherlands between 1986 and 2012.

INTRODUCTION: This study aimed to examine long-term population-based trends in the incidence rate of patients with a humeral fracture admitted to a hospital in the Netherlands from 1986 to 2012 and to give a detailed overview of the health care consumption and productivity loss with associated costs. MATERIALS AND METHODS: Age and gender-standardised incidence rates of hospital admissions for patients with a proximal, shaft, or distal humeral fracture were calculated for each year (1986-2012). Injury cases, length of hospital stay (LOS), trauma mechanism, and operation rate were extracted from the National Medical Registration. An incidence-based cost model was applied to calculate costs for direct health care and lost productivity in 2012. RESULTS: Between 1986 and 2012 112,910 patients were admitted for a humeral fracture. The incidence rate increased from 17.8 in 1986 to 40.0 per 100,000 person years in 2012. Incidence rates of proximal fractures increased the most, especially in elderly women. Operation rates decreased in patients aged 70 years or older. The mean LOS decreased from nine days in 1997 to five days in 2012. The cumulative LOS of all patients in 2012 was 28,880 days of which 73% were caused by women and 81% were caused by patients aged 50 years or older. Cumulative medical costs in 2012 were M€55.4, of which M€43.4 was spent on women. Costs increased with age. Costs for hospital care contributed most to the overall costs per case until 70 years of age. From 70 years onwards, the main cost determinants were hospital care, rehabilitation/nursing care, and home care. Cumulative costs due to lost productivity were M€23.5 in 2012. Costs per case increased with age in all anatomic regions. CONCLUSIONS: The crude number of patients admitted for a humeral fracture increased 124% in 27 years, and was associated with age and gender. Proximal fractures in elderly women accounted most significantly for this increase and most of the costs. The main cost determinants were hospital care and productivity loss.