Case Study
Snejina Vassileva
Bullöser Lupus Erythematodes: Eine Fallbeobachtung mit Merkmalen der Epidermolysis Bullosa Acquisita nebst Literaturübersicht
Bullous systemic lupus erythematosus: a case with features of epidermolysis bullosa acquisita and review of the literature
Keywords | Summary | Correspondence | Literature
Keywords
Bullous lupus erythematosus, epidermolysis bullosa acquisita
Schlüsselworte
Bullöser Lupus erythematodes, Epidermolysis bullosa acquisita
Summary
Bullous systemic lupus erythematosus (BSLE) and epidermolysis bullosa acquisita (EBA) are phenotypically distinct, but immunologically closely related disorders characterized by autoimmunity to type VII collagen. Despite their immunologic overlap, BSLE and EBA usually manifest with clinical and histological features that allow differentiation between them. We observed a 33-year-old man with 2 years history of systemic lupus erythematosus (SLE) who simultaneously developed two types of blistering eruptions compatible with BSLE and classical EBA, respectively. Both types of lesions improved after treatment with dapsone and systemic corticosteroids without any recurrence during the 12-years follow- up. The present case illustrates the possibility that autoimmunity to type VII collagen in patients with SLE may be expressed by two different clinical phenotypes, such as BSLE and classical EBA, rather than to be merely coexistent EBA in a patient with BSLE.
Zusammenfassung
Phänotypisch unterscheiden sich der bullöse Lupus erythematodes (BSLE) und die Epidermolysis bullosa acquisita (EBA), immunologisch handelt es sich um nah verwandte Autoimmunerkrankungen gegen Kollagen Typ VII. Trotz des immunologischen Overlaps sind im Allgemeinen beide Krankheiten klinisch und histologisch zu differenzieren. Wir sahen einen 33-jährigen Mann mit einem seit 2 Jahren bestehenden systemischen Lupus erythematodes (SLE), der zwei unterschiedliche Blasenbildungen zeigte, die mit BSLE und der klassischen EBA vereinbar waren. Beide Blasentypen sprachen auf Dapson und systemische Kortikosteroide an. In einer Nachbeobachtungszeit von 12 Jahren kam es nicht zum Rezidiv. Der Fall verdeutlicht, dass die Autoimmunität gegenüber Kollagen Typ VII sich bei SLE-Patienten phänotypisch unterschiedlich äußern kann – BSLE und klassische EBA – und nicht nur als simple Koexistenz von EBA und BSLE.
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Korrespondenz-Adresse
Snejina Vassileva, MD, PhD
Department of Dermatology and Venereology
Sofia Faculty of Medicine
1, G. Sofiiski Street
1431 Sofia, Bulgaria
snejina.vassileva@gmail.com
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