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  • 1
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    German Medical Science GMS Publishing House; Düsseldorf
    In:  5th International Conference for Research in Medical Education (RIME 2017); 20170315-20170317; Düsseldorf; DOCO8 /20170307/
    Publication Date: 2017-03-07
    Keywords: ddc: 610
    Language: English
    Type: conferenceObject
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  • 2
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    German Medical Science GMS Publishing House; Düsseldorf
    In:  Jahrestagung der Gesellschaft für Medizinische Ausbildung (GMA); 20160914-20160917; Bern; DOCP3-347 /20160905/
    Publication Date: 2016-09-05
    Keywords: ddc: 610
    Language: German
    Type: conferenceObject
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  • 3
    ISSN: 1432-1173
    Keywords: Key words Acquired — C1-inhibitor deficiency — Angioneurotic oedema — Paraprotein — Livedo reticularis ; Schlüsselwörter Erworbener C1-Inhibitor-Mangel — Angioödem — Paraprotein — Livedo racemosa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 61-year-old patient with life-threatening angioneurotic oedema was found to have an acquired C1-inhibitor (C1-INH) deficiency. In addition to lowered serum levels of C1-INH (both protein concentration and enzymatic activity), C2, C4 and CH50, which are characteristic for the hereditary form of angioneurotic oedema, markedly lowered C1q was found, which is typical for the acquired form. There were no antibodies against C1-INH. Repeated thorough examinations disclosed no neoplasm, though the presence of neoplasm has often been reported to be associated with the acquired C1-INH deficiency. However, the patient showed persistent paraproteinaemia and paraproteinuria and developed livedo reticularis. Treatment with danazol resulted in a rise in the complement fraction levels and cessation of angioneurotic oedema. Paraproteinaemia and livedo reticularis persisted unchanged.
    Notes: Zusammenfassung Wir berichten über einen 61jährigen Patienten mit lebensbedrohlichen Angioödemen, bei dem wir ursächlich einen erworbenen C1-Inhibitor-(INH)-Mangel feststellten. Neben erniedrigten Werten für C1-INH (Proteinkonzentration und enzymatische Aktivität), C2, C4 und CH50, wie sie auch für das hereditäre angioneurotische Ödem charakteristisch wären, wies unser Patient gleichzeitig eine ausgeprägte C1q-Verminderung auf. Dieser Befund ist diagnostisch wegweisend für die erworbene Form des Angioödems. Antikörper gegen C1-INH lagen nicht vor. Bei wiederholten Durchuntersuchungen fand sich kein Anhalt für ein Neoplasma, welches häufig mit einem akquirierten Angioödem assoziiert ist. Allerdings lagen eine Paraproteinämie und Paraproteinurie ohne den Nachweis eines Plasmozytoms vor. Im weiteren Verlauf entwickelte der Patient eine Livedo racemosa. Unter Danazoltherapie (Winobanin) kam es zu einem Anstieg bzw. zu einer Normalisierung der Komplementfraktionen und einem Ausbleiben der Angioödeme. Paraproteinämie und Livedo racemosa blieben unverändert bestehen.
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  • 4
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Lineare IgA-Dermatose ; Bullöses Pemphigoid ; Basalmembran ; Bullöse Autoimmundermatosen ; Autoantikörper ; Keywords Linear IgA disease ; Bullous pemphigoid ; Basement membrane ; Autoimmune blistering diseases ; Autoantibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract A 29-year-old female patient with an autoimmune subepidermal blistering disease had linear deposits of both IgA and IgG at the basement membrane zone. Clinically, the patient presented with tense blisters on the face, trunk, extremities and oral mucosa. Histologically, we found a subepidermal blister formation and a predominantly neutrophilic infiltrate. Direct immunofluorescence showed linear deposits of IgA along the basement membrane zone, as well as linear deposits of IgG and C3 as typically found in bullous pemphigoid. Indirect immunofluorescence demonstrated circulating IgA and IgG autoantibodies. This case extends previous reports on a subgroup of patients with subepidermal blistering diseases characterized by the presence of both IgA and IgG anti-basement membrane antibodies. These patients reveal clinical, histological and immunopathological features of linear IgA disease and bullous pemphigoid.
    Notes: Zusammenfassung Wir berichten über eine 29jährige Patientin mit einer subepidermal blasenbildenden Autoimmundermatose, die durch lineare Ablagerungen von IgA- und IgG-Antikörpern an der dermoepidermalen Junktionszone gekennzeichnet ist. Klinisch fanden sich pralle Bläschen im Gesicht, am Stamm, an den Extremitäten und an der Mundschleimhaut. Histologisch sahen wir eine subepidermale Blasenbildung und ein neutrophilenreiches Entzündungsinfiltrat. Mittels direkter Immunfluoreszenz fanden sich in der Haut der Patientin lineare IgA-Ablagerungen an der Basalmembranzone. Ungewöhnlich war jedoch der gleichzeitige Nachweis von linearen IgG- und C3-Ablagerungen, die typischerweise beim bullösen Pemphigoid vorkommen. Auch im Serum der Patientin fanden sich Autoantikörper sowohl der IgA- als auch der IgG-Klasse. Der Fall dieser Patientin bestätigt frühere Berichte, daß es bei den subepidermal blasenbildenden Autoimmundermatosen eine Untergruppe von Patienten mit gleichzeitigem Nachweis von IgA- und IgG-Antikörpern gibt, bei denen sich klinische, histologische und immunpathologische Merkmale von linearer IgA-Dermatose und bullösem Pemphigoid überlappen.
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  • 5
    ISSN: 1432-1076
    Keywords: Alopecia ; Contiguous gene syndrome ; Ichthyosis ; Photophobia ; X-linked recessive inheritance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe an 18-month-old male infant suffering from the ichthyosis follicularis, atrichia, and photophobia (IFAP) syndrome and further delineate the clinical phenotype. Severe retardation of growth and psychomotor development, chill-like seizures, bronchial asthma, urticaria, a proneness to skin infections and transient nail dystrophy observed in our patient are nonobligatory manifestations of this disorder. Histological examination of the atrichia revealed poorly developed, shortened hair follicles and a complete absence of sebaceous glands. The sex ratio of published cases suggests an X-linked recessive inheritance. The marked clinical variability of the IFAP syndrome might be the expression of a contiguous gene defect.
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  • 6
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Selbstheilendes Kollodiumbaby ; Lamelläre Ichthyose ; Elektronenmikroskopie ; Key words Self-healing collodion baby ; Lamellar ichthyosis ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Collodion baby is a clinical description for a transient condition of the newborn which is caused by various disorders of cornification. The majority of cases are due to autosomal recessive lamellar ichthyosis. In up to 10% of cases „self-healing” of the skin occurs. We report about a mature male baby who at birth was covered entirely with a shining, collodion-like membrane. There also were marked ectropion of the eyelids and eversion of the lips. After shedding of the membrane almost complete clearing of the skin occurred until the age of three months. In the case reported here skin biopsies taken on the 20th day of life showed discrete and non-specific ultrastructural changes, with no features of lamellar ichthyosis or other cornification disorders. Discussion: From these findings and those reported in the literature we conclude that the prognosis of a collodion baby is unpredictable on clinical evaluation. However an ultrastructural examination can contribute to early assessment of prognosis in this heterogeneous neonatal condition.
    Notes: Zusammenfassung Das klinische Bild des Kollodiumbabys wird durch verschiedene Verhornungsstörungen hervorgerufen; in der Regel entwickelt sich später eine lamelläre Ichthyose. Eine Selbstheilung wird in bis zu 10% der Fälle beobachtet. Wir berichten über ein männliches Reifgeborenes, welches bei der Geburt vollständig in eine glänzende, kollodiumähnliche Membran gehüllt war. Weiterhin fanden sich ektropionierte Augenlider und evertierte Lippen. In den ersten Lebenswochen löste sich die Membran gänzlich ab und bis zum Alter von 3 Monaten war die Haut des Patienten weitgehend erscheinungsfrei. Die elektronenmikroskopische Untersuchung einer am 20. Lebenstag entnommenen Hautbiopsie zeigte bei unserem Patienten keinen auf eine lamelläre Ichthyose oder eine andere Verhornungsstörung hinweisenden Strukturdefekt der Keratinozyten. Es fanden sich lediglich unspezifische Veränderungen im Sinn einer Abräumreaktion. Diskussion: Unter Berücksichtigung der Literatur läßt sich folgern, daß das klinische Bild eines Kollodiumbabys keine Rückschlüsse auf den weiteren Verlauf zuläßt. Dagegen kann die Elektronenmikroskopie prognostisch richtungsweisende Aussagen beim ätiologisch heterogenen Erscheinungsbild des Kollodiumbabys erlauben.
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  • 7
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary Background  Botulinum toxin type A (BTX-A) has been shown to be a safe and effective treatment for primary focal hyperhidrosis. However, the effect of BTX-A therapy on quality of life (QOL) in patients with this condition has only recently begun to be studied in controlled clinical trials. Objectives  To assess the impact on QOL of BTX-A treatment in patients with bilateral primary axillary hyperhidrosis. Methods  A multicentre, randomized, double-blind, placebo-controlled trial enrolled 320 patients who exhibited persistent, bilateral, primary axillary hyperhidrosis sufficient to interfere with daily activities. These patients were treated with either 50 U BTX-A (Botox ® , Allergan, Inc., Irvine, CA, U.S.A.) or placebo in each axilla. QOL was assessed using the Hyperhidrosis Impact Questionnaire © (HHIQ) at baseline and 1, 4, 8, 12 and 16 weeks post-treatment, as well as the Medical Outcomes Trust Short Form-12 Health Survey © (SF-12) at baseline and 16 weeks post-treatment. Results  At baseline, participants reported a marked negative impact of hyperhidrosis on various measures, including emotional status, ability to participate in daily and social activities, productivity at work and number of clothing changes per day. During the post-treatment period, statistically and clinically significantly greater improvements in all of these parameters were observed for the BTX-A group compared with the placebo group ( P  〈 0·01). The BTX-A group improvements were observed within 1 week of treatment, and were sustained with little or no decline throughout the 16-week follow-up period. Compared with the baseline HHIQ responses regarding treatment history, BTX-A treatment resulted in a greater level of overall treatment satisfaction than did many other hyperhidrosis treatments. In addition, patients treated with BTX-A exhibited statistically significantly greater improvement in the physical component summary score of the SF-12 at 16 weeks than did placebo-treated patients ( P  ≤ 0·019). Conclusions  Hyperhidrosis is associated with a substantial QOL burden; however, QOL is markedly improved with BTX-A treatment.
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  • 8
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report a 24-year-old woman, her 6-year-old son and her 1 7-month -old daughter, who all suffer from a rare congenital genodermatosis first delineated by Huriez et al. in the 1960s. The clinical features of this autosomal dominant condition include scleroatrophy of the hands and feel, nail hypoplasia, mild palmoplantar keratoderma and hypohidrosis. Histological changes are non-specific, but immunohistological and ultrastructural examination in our index patient revealed an almost complete absence of epidermal Langerhans cells in the affected skin. This new finding may be linked to the cancer proneness of the scleroatrophic skin. In this family, the grandmother had died at the age of 37 years from metastatic squamous cell carcinoma which had arisen on the thenar eminence.
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  • 9
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 152 (2005), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Congenital hyponychia and anonychia are rare malformations which may form part of syndromes such as nail–patella syndrome, ectodermal dysplasias and brachydactylies, or may occur as an isolated finding. Congenital hyponychia and anonychia are frequently accompanied by underlying skeletal abnormalities. A 20-year-old woman showed congenital bilateral hypoplasia or aplasia of the second, third and fourth toenails with corresponding phalanx dysplasia or aplasia of the affected toes. Malformations of the hands or other congenital defects were absent. The findings in this patient do not exactly fit any known entities. Our clinical observation prompted us to review the literature on congenital hyponychia/anonychia and to summarize recent advances in understanding molecular events in nail development. In conclusion, the association of nail anomalies with aplasia and/or hypoplasia of corresponding middle and/or distal phalanges supports the hypothesis of bone-dependent nail formation.
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  • 10
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background  Hyperhidrosis (primary or secondary) is excessive sweating beyond that required to return body temperature to normal. It can be localized or generalized, commonly affecting the axillae, palms, soles or face, and can have a substantial negative effect on a patient's quality of life.Impact of disease  Objective evaluation comprising quantitative assessment (gravimetric and Minor's iodine starch test) and subjective evaluation (Dermatology Quality of Life Index and Hyperhidrosis Impact Questionnaire) allow accurate assessment of the impact of hyperhidrosis on patients.Botulinum toxin type A  Botulinum toxin type A acts by inhibiting the release of acetylcholine at the presynaptic membrane of cholinergic neurones. It has proved useful in treating a number of diseases relating to muscular dystonia and is now proving beneficial in treating hyperhidrosis. Clinical trials investigating botulinum toxin type A use in axillary and palmar hyperhidrosis show significant benefits with few side-effects reported, with a favourable impact also being seen on patient quality of life. Botulinum toxin type A injections are generally well-tolerated with beneficial results lasting from 4 to 16 months.Conclusions  Botulinum toxin type A injections are an effective and well-tolerated treatment for hyperhidrosis. This paper proposes a positioning of this treatment along with current established treatments, and highlights the role of botulinum toxin type A as a valuable therapy for the treatment of hyperhidrosis.
    Type of Medium: Electronic Resource
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