Dupilumab was first approved for the treatment of atopic dermatitis (AD) and blocks the signaling of interleukin (IL)-4 and -13. Several other chronic skin conditions share mechanistic overlaps with AD in their pathophysiology, i.e., are linked to type 2 inflammation. Most recently, dupilumab was approved by the U.S. Food and Drug Administration for prurigo nodularis (PN). Given its relatively good safety profile, effective off-label use of dupilumab has been reported for a multitude of dermatologic diseases and several clinical trials for dermatologic skin conditions are currently ongoing. We conducted a systematic review of applications of dupilumab in dermatology other than AD and PN by searching the databases PubMed/Medline, Scopus, Web of Science and Cochrane Library as well as the clinical trial registry ClinicalTrials.gov. We found several reports for effective treatment of bullous autoimmune diseases, eczema, prurigo, alopecia areata, chronic spontaneous urticaria, Netherton syndrome and a variety of other chronic inflammatory skin diseases.
Recurrent blister formation in children may be a sign of hereditary epidermolysis bullosa even if no salient family history can be elicited. In a case of a 5-year-old boy with recurrent occasional skin blistering, we diagnosed epidermolysis bullosa simplex and found a causative rare homozygous mutation in EXPH5. Precise molecular genetic analysis is a prerequisite for the accurate diagnosis and adequate counselling of affected families.
Ein 5-jähriger aus Syrien stammender Junge präsentierte sich mit Bildung vereinzelter praller Blasen auf gesunder Haut alle 2-3 Wochen seit dem 9. Lebensmonat. Die Blasen seien auf den ganzen Körper mit Aussparung des Kopfes verteilt und würden unter nachfolgender Hyperpigmentierung narbenfrei abheilen. Die Mundschleimhaut sei nicht betroffen. Geschwister, Eltern und andere Familienangehörige seien hautgesund. Die Eltern seien nicht konsanguin.
HAUTBEFUNDAn den Beinen und Armen betont streckseitig sowie am Stamm (p.m. Unterschenkel streckseitig) zeigten sich disseminierte postinflammatorisch hyperpigmentierte Plaques und z. T. verkrustete Erosionen auf erythematösem Grund (Abbildung 1a, b). Am Malleolus medialis rechts sah man eine ca. 1 × 0,5 cm messende pralle Blase mit klarer Flüssigkeit. Das Nikolski-Zeichen war negativ. Keine Milien. Mundschleimhaut, Zähne, Haare und Nägel unauffällig.
DERMATOHISTOPATHOLOGIELokalisationstypisch kompakt verhornende Epidermis mit ausgeprägter Vakuolisierung der Basalzellschicht und einer Blasenbildung im Bereich des Biopsierandes. Die Spaltbildung findet innerhalb der basalen Keratinozyten statt (Abbildung 2). Im oberen Korium immer wieder Civatte-This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
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