title: Premonitory epidermolysis bullosa acquisita mimicking eyelid dermatitis: successful treatment with rituximab and protein A immunoapheresis.
abstract: We report a unique case of a 71-year-old female patient with epidermolysis bullosa acquisita. The patient initially presented with the clinical symptoms of bilateral eyelid dermatitis that occurred several months prior to the development of oral and pharyngeal erosions and blisters. While no contact allergy was found by patch testing, direct immunofluorescence microscopy demonstrated linear deposits of IgG at the basement membrane zone. By indirect immunofluorescence microscopy on human salt-split skin, IgG antibodies bound to the dermal side of the split. Immunoblot analysis showed predominant IgG4 reactivity of the patient's serum with the recombinant non-collagenous-1 domain of type VII collagen. Because of treatment resistance to systemic corticosteroids, dapsone, and colchicine, we initiated a combination treatment of protein A immunoapheresis and rituximab. With this treatment, complete remission was achieved within 4 months. Our case highlights that epidermolysis bullosa acquisita may initially mimic an eyelid dermatitis. Consequently, dermatologists should be aware of this rare differential diagnosis of eyelid dermatitis where a contact allergy or atopic dermatitis is absent.
authors.affiliation: Department of Dermatology and Venereology, Otto-von-Guericke-University Magdeburg, Magdeburg, Germany.
authors.forename: Ilja
authors.initials: I
authors.lastname: Kubisch
authors.forename: Philip
authors.initials: P
authors.lastname: Diessenbacher
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.forename: Harald
authors.initials: H
authors.lastname: Gollnick
authors.forename: Martin
authors.initials: M
authors.lastname: Leverkus
mesh_terms: D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D003131:Combined Modality Therapy; D003872:Dermatitis / Q000175:diagnosis*; D003937:Diagnosis, Differential; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D005141:Eyelid Diseases / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007155:Immunologic Factors / Q000627:therapeutic use; D008856:Microscopy, Fluorescence; D010956:Plasmapheresis / Q000379:methods; D012074:Remission Induction / Q000379:methods; D000069283:Rituximab; D013205:Staphylococcal Protein A / Q000276:immunology
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007074:Immunoglobulin G; D007155:Immunologic Factors; D013205:Staphylococcal Protein A; D000069283:Rituximab