Epidermolysis bullosa acquisita with negative direct immunofluorescence

Epidermolysis bullosa (EB) appeared in a patient at the age of 54 years. Other bullous disorders could be excluded by electron microscopy, and there was no family history of EB. The patient would therefore best be classified as having EB acquisita. Repeated direct immunofluorescence studies were, however, negative for all tested serum samples, suggesting that there might be a subgroup lacking immunoglobulin deposits in the skin. Collagen IV, laminin, and fibronectin were expressed normally at the dermoepidermal junction.