Scleromyxedema is a rare chronic skin disorder of unknown etiology, often accompanied by monoclonal gammopathies. Clinically the disease is characterized by generalized thickening and stiffening of the skin due to dermal mucin deposition. We report on a 56-year-old women with scleromyxedema, whose skin findings markedly improved following high-dose intravenous immunoglobulin therapy (Sandoglobulin((R)), 2 g/KG). The pronounced clinical benefit was documented by increased flexibility of the fingers, reduction of skin induration, and a decrease in number and size of mucinous papules. All previous therapeutic approaches were ineffective. High-dose intravenous immunoglobulin appears to be a promising treatment option for patients with scleromyxedema. Clinical studies are desirable, but difficult to perform in such a rare disease. Long-term follow-up on individual patients so treated is desirable.

H&G Zeitschrift für HautkrankheitenVolume 77, Issue 1 p. 51-54 9. Erlanger Dermatologen-Nachmittag (EDN) am 24. 1. 2001 in der Dermatologischen Klinik mit Poliklinik der Universit?t Erlangen-N?rnberg 9th Dermatological Afternoon in Erlangen on January 24th 2001 at the Department of Dermatology, University Erlangen-N?rnberg M. Simon Jr., M. Simon Jr.Search for more papers by this authorH. Schell, H. SchellSearch for more papers by this author M. Simon Jr., M. Simon Jr.Search for more papers by this authorH. Schell, H. SchellSearch for more papers by this author First published: 28 June 2008 https://doi.org/10.1046/j.1439-0353.2002.01071.xAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Volume77, Issue1January, 2002Pages 51-54 RelatedInformation

Zusammenfassung Bei Patienten mit schmerzhaften, evtl. hämorrhagischen Vesikeln oder schwarzen Krusten bzw. Nekrosen im Bereich der Hände oder der Gesichts- und Halsregion sowie mit typischer Anamnese (Kontakt zu Kühen oder freilebenden Katzen) muß auch an eine Kuh-/Katzenpockeninfektion gedacht werden. Der klinische Verdacht wird durch den Virusnachweis (Elektronenmikroskopie) bestätigt, weitere Hinweise ergeben die Histologie (intrazytoplasmatische Einschlußkörperchen), die Virusisolierung, die PCR sowie der serologische Antikörpernachweis. Wir berichten über zwei Patientinnen mit typischem klinischen Bild der Erkrankung und anamnestischem Kontakt zu Kühen bzw. Katzen. Aktuelle diagnostische sowie therapeutische Möglichkeiten werden erörtert.

Polymorphonuclear leucocytes (PMNL) or microphages resp. are essentially involved, on account of their ubiquity within the entire organism as well as their phagocytic and intracellular killing properties, in the complex organization of the antimicrobial and antitumoral protective capacity of man. Positive chemotaxis triggers the migration of PMNL to the specific danger area and thus initiates an immune reaction. An either inborn or acquired defect or functional weakness of PMNL may entail a state of persisting viral, fungal and/or bacterial infections. Impairment of various PMNL functions may result from different metabolic disorders, internal malignancies, and/or immunosuppressive therapy. Disturbances of various PMNL functions can be especially analysed in vitro. After delineation of different techniques available to check the immunological defense system of the human organism, we report on our results of PMNL function analysis in patients suffering from chronically recurrent pyoderma (n = 22), oral precancer and cancer (n = 13), chronic mucocutaneous candidosis (n = 10), recurrent aphthous ulcers (n = 33), as well as Behcet's disease (n = 20).