Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita

Erik Letko, Kailash Bhol, Fahd Anzaar, Victor L Perez Quinones, A. Razzaque Ahmed, C. Stephen Foster

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Objective: To describe a nonconventional diagnostic technique used to diagnose a case of cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita. Methods: Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, intravenous immunoglobulin therapy was administered. Results: Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of intravenous immunoglobulin therapy and remained stable during follow-up. Conclusions: Epidermolysis bullosa acquisita can manifest in the eye as chronic cicatrizing conjunctivitis indistinguishable from ocular cicatricial pemphigoid. Anonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic cicatrizing conjunctivitis. Intravenous immunoglobulin therapy was effective against chronic cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita.

Original languageEnglish
Pages (from-to)1615-1618
Number of pages4
JournalArchives of Ophthalmology
Volume124
Issue number11
DOIs
StatePublished - Nov 1 2006
Externally publishedYes

Fingerprint

Epidermolysis Bullosa Acquisita
Conjunctivitis
Passive Immunization
Intravenous Immunoglobulins
Direct Fluorescent Antibody Technique
Immunoglobulin G
Conjunctiva
Immunoglobulin A
Autoimmune Diseases
Antibodies
Benign Mucous Membrane Pemphigoid
Skin
Complement C3
Fluorescein
Serum
Basement Membrane
Fibrinogen
Signs and Symptoms
Immunoglobulin M
Rabbits

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Letko, E., Bhol, K., Anzaar, F., Perez Quinones, V. L., Ahmed, A. R., & Foster, C. S. (2006). Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita. Archives of Ophthalmology, 124(11), 1615-1618. https://doi.org/10.1001/archopht.124.11.1615

Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita. / Letko, Erik; Bhol, Kailash; Anzaar, Fahd; Perez Quinones, Victor L; Ahmed, A. Razzaque; Foster, C. Stephen.

In: Archives of Ophthalmology, Vol. 124, No. 11, 01.11.2006, p. 1615-1618.

Research output: Contribution to journalArticle

Letko, E, Bhol, K, Anzaar, F, Perez Quinones, VL, Ahmed, AR & Foster, CS 2006, 'Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita', Archives of Ophthalmology, vol. 124, no. 11, pp. 1615-1618. https://doi.org/10.1001/archopht.124.11.1615
Letko, Erik ; Bhol, Kailash ; Anzaar, Fahd ; Perez Quinones, Victor L ; Ahmed, A. Razzaque ; Foster, C. Stephen. / Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita. In: Archives of Ophthalmology. 2006 ; Vol. 124, No. 11. pp. 1615-1618.
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