Ocular involvement in anti-epiligrin cicatricial pemphigoid. Eur J Ophthalmol 2006;16(6):867-9
Date
12/28/2006Pubmed ID
17191196DOI
10.1177/112067210601600615Scopus ID
2-s2.0-33846601888 (requires institutional sign-in at Scopus site) 6 CitationsAbstract
PURPOSE: To report an anti-epiligrin cicatricial pemphigoid (AECP) patient with severe ocular involvement and to provide a practical approach to distinguishing AECP patients from those with other subepidermal blistering diseases.
METHODS: Techniques included direct and indirect immunofluorescence microscopy, Western blot and immunoprecipitation studies, as well as interdisciplinary examinations of mucous membranes and skin.
RESULTS: This study describes a patient with clinical features of cicatricial pemphigoid, circulating anti-basement membrane zone IgG antibodies, and subepidermal blisters. Histopathology and immunofluorescence analysis suggested the diagnosis of a cicatricial pemphigoid-like type of epidermolysis bullosa acquisita. However, Western blot and immunoprecipitation studies demonstrated that the patient's serum contained autoantibodies against laminin 5 alpha3 subunit, leading to the diagnosis of an AECP.
CONCLUSION: Since patients with AECP have an increased relative risk for malignant tumors, it is important to distinguish this entity within the spectrum of cicatricial pemphigoid patients by additional studies such as Western blot or immunoprecipitation.
Author List
Nischler C, Sadler E, Lazarova Z, Stoiber J, Ruckhofer J, Pohla-Gubo G, Emberger M, Bauer JW, Grabner G, Hintner HMESH terms used to index this publication - Major topics in bold
AutoantibodiesAutoimmune Diseases
Blotting, Western
Cell Adhesion Molecules
Conjunctival Diseases
Epidermolysis Bullosa Acquisita
Female
Fluorescent Antibody Technique, Direct
Fluorescent Antibody Technique, Indirect
Humans
Immunoglobulin G
Middle Aged
Mucous Membrane
Pemphigoid, Benign Mucous Membrane
Skin
