Brunsting-Perry cicatricial pemphigoid

What is Brunsting-Perry cicatricial pemphigoid?

Brunsting-Perry cicatricial pemphigoid (or mucous membrane pemphigoid) is a rare variant of mucous membrane pemphigoid. It is an autoimmune blistering skin condition localised to the head and neck region. The most striking feature is the development of significant scarring. In contrast to the classic mucous membrane pemphigoid, mucous membrane involvement in less common, and is not associated with scarring.

Some authors believe Brunsting-Perry cicatricial pemphigoid is better termed the Brunsting-Perry variant of epidermolysis bullosa acquisita. Epidermolysis bullosa acquisita is another uncommon autoimmune blistering disorder.

What causes Brunsting-Perry cicatricial pemphigoid?

Brunsting-Perry cicatricial pemphigoid is an autoimmune disorder. This means that an individual’s immune system starts reacting against his or her own tissue. In this case, autoantibodies react with components of the basement membrane, particularly the anchoring filament component laminin 5, and the hemidesmosome-associated proteins BP180 and BP230. In some patients, antibodies to collagen VII have been described.

In 20 to 30% of the patients with Brunsting-Perry cicatricial pemphigoid, skin antibodies can be detected in their blood stream. (positive indirect immunofluorescence).

Clinical presentation of Brunsting-Perry cicatricial pemphigoid

The diagnosis of Brunsting-Perry cicatricial pemphigoid is based on the following features:

• An itchy urticated plaque localised to the head and neck (‘urticated’ means a wheal-like plaque appearing similar to urticaria
• The plaque enlarges over weeks to months
• Blisters arise around the edge of the plaque
• Occasionally a smaller secondary plaque appears
• The plaque slowly heals with significant scarring over months to years
• Lesions on the scalp are associated with scarring alopecia (hair loss)
• Mucous membrane lesions may occur months following the primary lesion

Biopsy features are similar to bullous pemphigoid, but without scarring.

What is the treatment for Brunsting-Perry cicatricial pemphigoid?

Treatment of Brunsting-Perry cicatricial pemphigoid is similar to that of classic cicatricial pemphigoid. The aim of treatment is to promote healing.

Ultra-potent topical steroids such as clobetasol propionate may be sufficient as a monotherapy, however many patients require systemic corticosteroids. Other immune-modulating agents used for Brunsting-Perry cicatricial pemphigoid have included:

• Methotrexate
• Dapsone
• Excision with full-thickness skin graft

Overall, patients with Brunsting-Perry cicatricial pemphigoid have a better prognosis than patients with classic cicatricial pemphigoid or with epidermolysis bullosa acquisita.