Safety Data in Patients with Autoimmune Diseases during Treatment with High Doses of Vitamin D3 According to the "Coimbra Protocol".

BACKGROUND: In 2013, the group of Cicero Coimbra, Brazil, reported the clinical efficacy of high doses of vitamin D3 in patients suffering from autoimmune skin disorders ("Coimbra protocol", CP). However, hypercalcemia and the subsequent impaired renal function may be major concerns raised against this protocol. METHODS: We report for the first time for a broad spectrum of autoimmune diseases in 319 patients (mean age (±SD) 43.3 ± 14.6 years, 65.5% female, 34.5% male) safety data for high doses of orally applied vitamin D3 (treatment period: up to 3.5 years) accompanied by a strict low-calcium diet and regular daily fluid intake of at least 2.5 L. RESULTS: Mean vitamin D3 dose was 35,291 ± 21,791 IU per day. The measurement of more than 6100 single relevant laboratory parameters showed all mean values (±SD) within the normal range for total serum calcium (2.4 ± 0.1 mmol/L), serum creatinine (0.8 ± 0.2 mg/dL), serum creatinine associated estimated GFR (92.5 ± 17.3 mL/min), serum cystatin C (0.88 ± 0.19 mg/L), serum TSH (1.8 ± 1 mIU/L), and for 24 h urinary calcium secretion (6.9 ± 3.3 mmol/24 h). We found a very weak relationship between the dosage of oral vitamin D3 and the subsequent calcium levels, both in serum and in urinary excretion over 24 h, respectively. CONCLUSIONS: Our data show the reliable safety of the CP in autoimmune patients under appropriate supervision by experienced physicians.

Serum 25-hydroxyvitamin D levels in patients with skin diseases including psoriasis, infections, and atopic dermatitis.

The pathogenetic role of vitamin D as well as its clinical correlation in inflammatory skin diseases is still uncertain. This study aimed to compare serum levels of 25(OH) vitamin D (calcidiol) in outpatients suffering from different skin diseases using the same laboratory method in one study. In routine serum samples of 1,532 patients from the previous 12 months we identified retrospectively 180 (mean age 49.4 years, 80 female, 100 male) and 205 (mean age 36.3 years, 116 female, 89 male) patients with psoriasis (PSO) and atopic dermatitis (AD), respectively. Clinical disease activity and quality of life was evaluated using Physicians Global Assessment Scores (PGA), Dermatology Life Quality Index (DLQI), and a Visual Analog Scale for pruritus in AD, respectively. The median 25(OH)D serum level of all patients (22.97 ng/mL, range 2.61-96.0, n = 1,461) was significantly lower in comparison to healthy controls (41.6 ng/mL, range 16.9-77.57, p < 0.0001, n = 71). In PSO and AD we measured 21.05 ng/mL (44% < 20 ng/mL) and 22.7 ng/mL (39% < 20 ng/mL), respectively (p = 0.152). Among all subgroups, patients with severe acute or chronic infectious skin diseases had the lowest median 25(OH)D serum levels (17.11 ng/mL, n = 94, 66% <20 ng/mL, p < 0,001 vs. AD, p = 0,007 vs. PSO). For PSO and AD there was no significant correlation between 25(OH)D levels and PGA scores and DLQI values, respectively, or the extent of pruritus in AD. 25(OH)D serum levels in inflammatory skin diseases might correlate more with the type of disease and the degree of inflammation than with clinical activity itself.

Pre-inpatient evaluation on quality and impact of care in systemic mastocytosis and the influence of hospital stay periods from the perspective of patients: a pilot study.

BACKGROUND: The quality and impact of care in patients with mastocytosis has not yet been investigated in detail. PATIENTS AND METHODS: Here, we report monocentric findings of a retrospective questionnaire from 84 adult inpatients with mastocytosis regarding the quality and impact of pre-inpatient clinical care after a period of inpatient stay. It was possible to analyse the data derived from 50 patients (40 female, 10 male; 74 % with systemic mastocytosis, 26 % with cutaneous mastocytosis). RESULTS: Correct diagnoses were discovered to have taken over 2 years for 68 % of patients (up to 11 years). 58 % of patients required the consultation of three or more clinicians until the final diagnosis was reached; 3 patients consulted more than 9 doctors. The correct diagnosis was finally made in 94 % of patients by a dermatologist. The main symptoms expressed by the patients were skin disorders (92 %), itching (66 %) and exhaustion (48 %). From a patient perspective, the main triggering factors given were food and food additives (63 %), emotional stress (46 %) and physical exertion (44 %). Interdisciplinary inpatient treatment provided the patients with a better understanding of their disease in 90 %. Outpatient supervision was performed mainly by dermatologists (46 %), family doctors (40 %) and other clinicians (14 %). 22 % required the assistance of various doctors for their outpatient care. Only 14 % of patients reported that their daily life was relatively unaffected by mastocytosis. 22 % felt the disease slightly affected their quality of life, 20 % moderately, 26 % strongly and 18 % said the disease had a very strong impact on their quality of life. 82 % of patients browsed the Internet in order to find information regarding mastocytosis, although for 26 % this information was not deemed useful and 10 % felt rather unsettled. CONCLUSIONS: Patients with severe mastocytosis requiring hospitalization reported clear restrictions in their daily life. Such patients require an improved interdisciplinary diagnostic approach and therapy.

Mastocytosis - an update.

Mastocytosis (MC) encompasses a range of disorders characterized by a clonal, pathological accumulation of mast cells having a somatic activating mutation of the tyrosine kinase receptor Kit (exon 17, codon 816; D816V) in more than 90 % of adult patients. The mutation is much less common in children. Skin and bone marrow are most often affected. Symptoms and clinical course are very heterogeneous due to a variable degree of local or systemic mediator release or organ dysfunction as a result of mast cell infiltrates. Pruritus, wheals, flushing and gastrointestinal symptoms are often reported. The majority of pediatric patients experience spontaneous remission of MC. Adults usually have chronic disease, rarely transforming into an aggressive or lethal type. Indolent systemic MC with involvement of skin and bone is the most common type. In MC the risk for anaphylactic reactions following an insect sting (and other causes of mast cell activation) is increased significantly. Diagnostic hallmarks are biopsies from skin and bone marrow using tryptase antibodies for staining as well as serum tryptase levels. At present a curative treatment for MC is not available. Systemic histamine H(1) receptor antagonists are widely used. Aggressive types of MC respond partially to IFN-alpha or cladribine. A variety of receptor tyrosine kinase inhibitors is still under critical evaluation for systemic treatment of MC. After introduction of the WHO classification for MC and the development a German MC guideline, as well as the foundation of national and international competence networks for MC, a significantly improved quality of medical care for MC patients can be expected for the future.

Cutaneous infection with Leishmania infantum in an infant treated successfully with miltefosine.

A two-year-old girl presented with a 20 month history of a facial nodule which had appeared after a vacation on Mallorca. Various topical treatments at other hospitals for the working diagnosis of mastocytoma failed to prevent a slow increase in size and the onset of systemic signs and symptoms. An indurated crusted nodule evolved. Histology, tissue PCR and serologic analysis proved the presence of Leishmania infantum. She was treated with oral miltefosine 10 mg p.o. t.i.d. for 28 days. Regression was apparent after 8 weeks and complete healing after 6 months.

[Implementation and development of quality management in hospitals and private practice -- present state of the art]. / Einführung und Weiterentwicklung von Qualitätsmanagement in Klinik und Praxis -- eine aktuelle Situationsanalyse.

Since 1. 1. 2004 both hospitals and private practices in Germany are required by law to implement a quality management (QM) system within their organization. Different QM systems with a number of advantages and disadvantages are available. It has been well-documented that implementing QM im-proves the quality of both processes and results, thus improving patient care. At present certification of the organization is not yet part of the official requirements, but seems a likely step.

German adaptation of the Skindex-29 questionnaire on quality of life in dermatology: validation and clinical results.

BACKGROUND: Health-related quality of life (HRQOL) has increasingly been recognized as an important aspect of a comprehensive clinical assessment in dermatology. OBJECTIVE: The aim of the present study was to translate and validate one of the most frequently used and established skin disease-specific HRQOL questionnaires originally developed in English for the German language area: the Skindex-29. METHODS: 295 in-patients with psoriasis and atopic dermatitis completed the German translation of the Skindex as well as a number of additional skin disease-specific questionnaires. Data from 2 subsamples were analysed separately to test for the robustness of results. RESULTS: Results from principal component analyses supported the scale structure of the original Skindex. Internal consistency coefficients were high for all scales. Further analyses supported the convergent validity of the German adaptation of the Skindex-29 as well as its sensitivity to change. CONCLUSION: The study provides evidence for the validity and reliability of the Skindex-29.

Validation of a comprehensive Freiburg Life Quality Assessment (FLQA) core questionnaire and development of a threshold system.

Health related quality of life (HRQOL) has become an important re- search topic in dermatology. We developed a series of modular, dermatology-specific HRQOL questionnaires: the Freiburg Life Quality Assessment (FLQA). Each FLQA questionnaire consists of a core module (FLQA-c) applicable to all skin diseases plus a number of items specific to distinct dermatological diseases. The objectives of the present study were: 1) to test the psychometric properties of the FLQA-c, and 2) to define a threshold indicating a significantly reduced HRQOL. 394 psoriasis patients, 253 atopic dermatitis patients, and 240 control subjects completed the FLQA-c. Cronbach's alphas were above 0.75 for all six scales except for "treatment". Discriminant validity, sensitivity to change, and convergent validity were satisfactory. On the different scales, the threshold indicating a marked reduction in HRQOL detected 33% to 68% of the patients. These data support the reliability and validity of the FLQA-c.