Cicatricial pemphigoid vegetans

Area of Science:

• Dermatology
• Immunology
• Gastroenterology

Background:

• Ulcerative colitis (UC) is a chronic inflammatory bowel disease.
• Cicatricial pemphigoid (CP) is a rare, chronic autoimmune subepithelial blistering disease affecting mucous membranes and skin.

Observation:

• A 58-year-old woman with a 5-year history of UC presented with oral erosions and conjunctival scarring.
• Two years later, she developed skin pustules and erosions, initially suspected as pyoderma gangrenosum.
• Immunofluorescence studies revealed IgG and IgA at the dermal-epidermal junction and circulating anti-basement membrane zone antibodies.

Findings:

• Laser scanning confocal microscopy confirmed cicatricial pemphigoid.
• Histopathology showed features consistent with autoimmune blistering, including subepidermal blisters and immune complex deposition.
• Despite initial treatment for UC, the patient developed distinct mucocutaneous manifestations of CP.

Implications:

• This case underscores the importance of considering autoimmune blistering diseases in patients with inflammatory bowel disease flares.
• Early diagnosis and combined immunosuppressive therapy are crucial for managing CP in UC patients.
• Further research is needed to elucidate the pathogenic links between UC and CP.