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  • 1
    Electronic Resource
    Electronic Resource
    The scleroatrophic syndrome of Huriez: a cancer-prone genodermatosis (1996)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 134 (1996), S. 0 
    Details
    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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.1996.41773.x
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  • 2
    Electronic Resource
    Electronic Resource
    Acute generalized exanthematous pustulosis following oral nystatin therapy: a report of three cases (1997)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 137 (1997), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary We report three cases of acute generalized exanthematous pustulosis (AGEP) following oral administration of nystatin. All cases showed similar clinical features and hislopathological findings, and a delnyed-type hypersensitivily to nystatin could be demonstrated in patch and prick testing. Drug eruptions to nystatin are extremely rare, and, to our knowledge, AGEP has not been reported previously.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2133.1997.19582038.x
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  • 3
    Electronic Resource
    Electronic Resource
    Scalp necrosis in temporal (giant cell) arteritis: implications for the dermatologic surgeon (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical and experimental dermatology 21 (1996), S. 0 
    Details
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Temporal arteritis, a variant of giant cell arteritis, is a systemic granulomatous vasculitis of large and medium-sized arteries. Usually the clinical Features arc dominated by ophthalmological and neurological complications. In rare instances, ischaemic necrosis, especially of the scalp, may lead patients to the dermatologist. We report a 76-year-old woman presenting with a unilateral scalp necrosis, accompanied by a dramatic ipsilateral impairment of vision. Immediately after duplex-sonography of the extracranial vessels and after initiation of corticosteroid therapy, the diagnosis of temporal arteritis was confirmed by temporal art en biopsy. One month later, because of insufficient secondary healing of the ulcer, the defect was covered by a mesh graft. The taking of the graft was delayed due to immunosuppressive therapy, but was complete. The patient unfortunately died as a result of complications related to surgical removal of an aspergilloma in the sphenoid cavity secondary to immunosuppressive therapy. We discuss the technique of artery biopsy and the possibility of surgical management of scalp necrosis in temporal arteritis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2230.1996.tb00043.x
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  • 4
    Electronic Resource
    Electronic Resource
    Lebensbedrohliche Angioödeme durch erworbenen C1-Inhibitor-Mangel, assoziiert mit Paraproteinämie und Livedo racemosa (1995)
    Springer
    Der Hautarzt 46 (1995), S. 339-342 
    Details
    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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050263
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  • 5
    Electronic Resource
    Electronic Resource
    “Selbstheilende” Form eines Kollodiumbabys Transiente Verhornungsstörung oder Minimalform einer lamellären Ichthyose? (1998)
    Springer
    Monatsschrift Kinderheilkunde 146 (1998), S. 1070-1073 
    Details
    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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050367
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