Autoimmune blistering diseases confined to the eye

Abstract

Ophthalmic involvement in autoimmune bullous diseases (AIBDs) can lead to permanent visual loss and blindness. Ocular manifestation usually parallel to active mucocutaneous disease. Apart from mucous membrane pemphigoid (MMP), pure ocular involvement in other AIBDs is exceedingly rare. We report three cases of AIBDs consisting of immunoglobulin (Ig) A/IgG pemphigus, linear IgA disease (LABD) and pemphigus vulgaris (PV) in which all patients presented with chronic cicatrizing conjunctivitis, mimicking MMP, without skin involvement. Our first patient’s conjunctival biopsy revealed separation of the stratified squamous epithelium with acantholysis. Direct immunofluorescence (DIF) demonstrated intercellular IgA deposition. Indirect immunofluorescence (IIF) was also positive for intercellular IgA. IgG enzyme-linked immunosorbent assay was positive for Desmoglein-1 (Dsg1) and negative for Anti-Dsg3. The diagnosis of IgA/IgG pemphigus was made. The second patient’s conjunctival biopsy showed fibrosis, chronic inflammation at subepithelial junction with separation.   DIF  revealed linear  basement membrane IgA,  leading to the  diagnosis of LABD.

Conjunctival biopsy of the final case showed chronic conjunctivitis with focal suprabasal separation of the stratified squamous epithelium. Immunoperoxidase showed intercellular IgG and C3. IIF was positive for IgG. The final diagnosis was ocular PV. All patients received systemic corticosteroids together with azathioprine or dapsone with satisfactory results. To the best of our knowledge, this is the first reported case for ocular IgA/IgG pemphigus. The presence pure ocular involvement is extremely rare for LABD and PV. We emphasize that AIBDs exclusive to the eye is a diagnostic dilemma. Early recognition and treatment can halt the progression and prevent permanent visual loss.