Immunologic aspects of cicatricial pemphigoid

Area of Science:

• Dermatology
• Ophthalmology
• Immunology

Background:

• Cicatricial pemphigoid is a chronic autoimmune blistering disease.
• It characteristically affects mucous membranes, particularly the conjunctiva, leading to ocular complications.
• Ocular involvement can result in significant visual impairment due to scarring and symblepharon.

Purpose of the Study:

• To report a case of cicatricial pemphigoid with significant ocular involvement.
• To evaluate the efficacy of systemic and local corticosteroid therapy in managing the condition.
• To investigate the immunopathological basis of cicatricial pemphigoid.

Main Methods:

• A 56-year-old female patient presented with typical clinical manifestations of cicatricial pemphigoid.
• Treatment involved oral triamcinolone (80 mg/day) for systemic lesions and local triamcinolone acetonide injections for ocular symblepharon.
• Direct immunofluorescence microscopy of conjunctival biopsy was performed.

Main Results:

• Systemic corticosteroid therapy with oral triamcinolone effectively resolved skin and mucous membrane lesions.
• Local injections of triamcinolone acetonide showed no significant improvement in ocular symblepharon or dryness.
• Direct immunofluorescence revealed immunoglobulin G (IgG) deposition along the basement membrane of the conjunctiva.

Conclusions:

• Systemic corticosteroids are effective in managing the mucocutaneous manifestations of cicatricial pemphigoid.
• Local corticosteroid injections may have limited efficacy for established ocular complications like symblepharon.
• The presence of IgG at the basement membrane supports an autoimmune etiology within the pemphigus-pemphigoid spectrum.