Sean Klepper M.D.
Artur Zembowicz M.D....
- Rare autoimmune blistering disorder
- Most common in the elderly, with a female predominance
- Presents as a vesiculobullous eruption, predominantly on mucosal surfaces, with skin involvement in 25% of cases
- The disease follows a chronic course, and the lesions heal slowly, with resultant pronounced scarring.
- The eye is involved in 65% of cases (ocular cicatricial pemphigoid), and blindness can result without treatment.
- If there is cutaneous involvement without mucosal involvement, which is rare, the term localized cicatricial pemphigoid (aka Brunsting-Perry cicatricial pemphigoid) is used. This form is more common in men, tends to affect the head and neck, and may cause scarring alopecia.
- Subepidermal blister with a mixed inflammatory infiltrate containing variable proportions of eosinophils, neutrophils and mononuclear cells within the blister cavity. Mononuclear cells often predominate in mucosal lesions.
- Upper dermal perivascular mixed inflammatory infiltrate
- Older lesions show granulation tissue which progresses to dense dermal fibrosis (scar formation).
- Immunofluorescence shows IgG and C3 along the dermal-epidermal junction in most cases, as well as IgA and IgM in some cases.
- On salt-split skin, most cases show localization to the roof of the blister, although in 20% of cases there is localization to either both the roof and the floor or the floor alone, due to the hetergeneity of implicated autoantibodies.