We discovered a significantly increased frequency of MS among patients with BP. At the time of diagnosis, patients with BP had an excessive number of comorbidities and an increased mortality rate over the following years.

Background: The autoimmune profile of Chronic Urticaria (CU) patients is an increasing topic of interest. Associated diseases suggest shared pathogenic pathways, and they may provide important knowledge on specific targets for future treatment models. In this study we examined the prevalence and risk of comorbidities in CU. Methods: The Danish National Patient Registry was used to identify all CU patients from 1994 to 2015. Five of 5 specialized dermatological units in Denmark were covered. Analyses were conducted as a nested case control study and a matched cohort study. CSU patients were matched 1:10 on age and sex to an otherwise random group of people from the background population. Results: A total of 12,185 CU patients were identified, with an overweight of female cases (69% versus 32%). There was an overrepresentation of mast cell mediated diseases including mastocytosis and anaphylaxis, as well as atopic diseases including type 1 allergies and atopic dermatitis. The prevalence of rheumatoid arthritis, systemic lupus erythematosus, thyroiditis and vitiligo was also increased, as was the prevalence of depression. CU patients who did not have any of the comorbidities at the time of their CU diagnosis had an increased risk of developing both mast cell mediated diseases, atopic diseases, and autoimmune diseases excluding thyroiditis and diabetes. Conclusion: The autoimmune profile of the comorbidities of CU was demonstrated with an evident risk of developing rheumatoid arthritis. CU patients were also at increased risk of either having or achieving depression. Mast cell related diseases seemed to be overrepresented, although registry data within this disease category are questionable and similar to symptoms of CU to the untrained eye. Thus, CU patients constitute a multimorbid group of patients, which must be recognized among treating physicians.

Bullous pemphigoid (BP) is a common blistering disease caused by antibodies directed against hemi-desmosomal proteins BPAG1 and BPAG2. The disease is characterised by intense pruritus and blistering of the skin. The systemic treatment with the highest level of evidence for BP is systemic glucocorticoids. However, since the disease often occurs in the elderly patients, and since the most common co-morbidities are diabetes and neurological diseases, glucocorticoid-sparing drugs are often introduced. We retrospectively identified all BP patients admitted to our tertiary clinic over a 7-year period in order to register demography, treatment and co-morbidities. The most common steroid-sparing drugs were azathioprine (87%) and methotrexate (11%). Less than 2% were treated with dapsone, rituximab and cyclosporin A. As expected, we found a relatively high rate of neurological disorders, diabetes, and malignancies, but surprisingly we also found an increased rate of cardiovascular diseases compared to the Danish population in general.

Bullous Pemphigoid is an autoimmune skin blistering disease. It is caused by deposition of auto antibodies along the dermal-epidermal border leading to inflammation. The antibodies are directed against anchoring filaments in the epidermis, but these antigens are also present in the neurological tissues and this has led to speculation of an association between multiple sclerosis and bullous pemphigoid. Additionally recent epidemiological studies have pointed at an increased risk of cardio-vascualr diseases and an increased moratality among the patients with bullous pemphigoid. In this mini review we present the recent findings in this area and as well as the treatment strategies when comorbidities are taken into consideration.

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BackgroundChronic spontaneous urticaria is a debilitating disease for the patients and often considered by the doctors a very difficult disease to treat. In 2009 the European Academy of Allergy and Clinical Immunology/Global Allergy and Asthma European network/European Dermatology Forum/World Allergy Organization (EAACI/GA2LEN/EDF/WAO) published a revised version of the guidelines for the treatment of urticaria which included an algorithm for the treatment.ObjectiveThe objective of this study was to examine the clinical effect and the practical use of the algorithm.MethodsThe study was performed as a case-series study of all newly referred patients to our urticaria clinic over a period of 18 months.ResultsOur results show that the single most important and efficient treatment of urticaria patients is up dosing non-sedating antihistamines as recommended by the EAACI/GA2LEN/EDF/WAO guidelines. We did not find any predicting factors for responders to the antihistamine treatment, but about one third of the patients did not respond to the up-dosing of the antihistamines.ConclusionAntihistamines seems to be the most efficient treatment for urticaria; other treatments such as montelukast, azathioprine, and mycophenolate mofetil may also be used, but only in combination with antihistamines and only in the case of an independent antihistamines treatment failure. The EAACI/GA2LEN/EDF/WAO guideline for the treatment of urticaria offers an efficient and simple guidelines for the treatment of urticaria.

PurposeCongenital epidermolysis bullosa (CEB) is a group of rare monogenic genodermatoses. Phenotypically, the diseases vary in both severity and dissemination, which complicates studies of their epidemiology. To investigate the potential of using the Danish National Patient Registry (DNPR) for epidemiological research on CEB, we examined the positive predictive value (PPV) of a first-time diagnosis of CEB.MethodsWe identified patients with a record of CEB in DNPR and the Danish Pathology Registry (DPR) during January 1, 1977, until December 31, 2015. We restricted diagnoses from two dermatological departments and one regional hospital. Diagnoses in the DNPR are coded by the eighth and tenth revisions of the ICD (ICD-8 and ICD-10) and in the DPR by the Systematized Nomenclature of Medicine (SNOMED). We used clinical description in medical records, family history, histological findings, and molecular genetic investigations to validate diagnoses and classified them as rejected and confirmed. We estimated PPVs for any diagnosis, according to coding systems used, and for additional subdivisions of ICD-10 codes.ResultsWe identified 116 cases from the hospital departments investigated and evaluated 96 medical records for validity. The overall PPV for probable CEB was 62.5% (95% CI: 52.5–71.5). For ICD-8, ICD-10, and SNOMED codes, the PPVs were 30.8% (95% CI: 11.4–57.7), 76.7% (95% CI: 65.8–84.9), and 0.0% (95% CI: 0.0–21.7), respectively. For the ICD-10 codes, we found the highest PPVs for diagnoses arising from the dermatological departments. For subdivisions of ICD-10 codes, PPVs were high for epidermolysis bullosa simplex and dystrophica.ConclusionThe PPVs for first-time diagnoses of CEB registered in the two Danish nationwide registries investigated, DNPR and DPR, ranged from low to average. We therefore recommend that these data be used with caution and restricted to ICD-10 diagnoses from specialized dermatological departments.

Background There are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population. Objective This study aimed to analyze the incidence and mortality of mastocytosis. Methods A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model. Results A total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0–1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6–2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8–2.5). The Charlson Comorbidity Index–adjusted mortality among adult patients with mastocytosis was HR Cutaneous Mastocytosis 1.2 (95% CI, 0.8–1.9), HR Indolent Systemic Mastocytosis 1.9 (95% CI 1.4–2.5), and HR Systemic Mastocytosis 4.2 (95%, CI 1.9–9.4), respectively. Conclusion Based on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years.