In severe ocular cicatricial pemphigoid (OCP), conjunctival biopsy shows direct immunofluorescence with linear deposits of IgG and C3 at the basement membrane zone. The HLA type associated with the most severe progressive form of OCP is:

• A HLA-DR4
• B HLA-A29
• C HLA-B27
• D HLA-DQw7 (DQB1*0301) ✓

Explanation

Ocular cicatricial pemphigoid is a type II hypersensitivity (autoimmune bullous disease) with IgG autoantibodies against basement membrane antigens (primarily beta-4 integrin subunit). HLA-DQw7 (DQB1*0301) is associated with the aggressive progressive form of OCP. The disease causes subconjunctival fibrosis, symblepharon, ankyloblepharon, limbal stem cell deficiency, and eventually blindness. Systemic immunosuppression (cyclophosphamide or dapsone) is required; local procedures worsen inflammation.

Reference: Khurana Comprehensive Ophthalmology, 7th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.