Oral pemphigoid masquerading as necrotizing ulcerative gingivitis in a child

Area of Science:

• Autoimmune diseases
• Pediatric dermatology
• Oral medicine

Background:

• Cicatricial pemphigoid (benign mucous membrane pemphigoid) is a rare autoimmune blistering disease affecting mucosal tissues, predominantly in adults.
• Childhood cases are exceptionally rare, with only nine reported instances, typically presenting as oral lesions.
• This case highlights a unique presentation of childhood cicatricial pemphigoid mimicking necrotizing ulcerative gingivitis (NUG).

Observation:

• A 9-year-old girl presented with persistent gingival bleeding and discomfort unresponsive to standard NUG treatments.
• Initial clinical suspicion was NUG, leading to antibiotic and oral hygiene interventions.
• Lack of improvement prompted further diagnostic investigations, including histopathology and immunofluorescence.

Findings:

• Hematoxylin and eosin (H&E) staining revealed non-specific ulceration and chronic inflammation.
• Direct immunofluorescence demonstrated linear C3 deposition at the basement membrane zone.
• These immunofluorescence findings were diagnostic of cicatricial pemphigoid.

Implications:

• Cicatricial pemphigoid should be considered in the differential diagnosis of refractory ulcerative oral lesions in children.
• Immunofluorescence is essential for accurate diagnosis, distinguishing it from other oral ulcerative conditions.
• Early and accurate diagnosis is vital for appropriate management of this rare pediatric autoimmune disorder.