Bullous systemic lupus erythematosus:
a case with features of epidermolysis bullosa acquisita and review of the literature
SNEJINA VASSILEVA, MD, PHD1, KOSSARA DRENOVSKA, MD, PHD1, KYRILL PRAMATAROV, MD, PHD2
1 Dep. of Dermatology and Venereology, Faculty of Medicine, Sofia, Bulgaria
2 University Hospital Lozenetz
KEY WORDS: Bullous lupus erythematosus, epidermolysis bullosa acquisita
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.
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