Cicatricial pemphigoid

    Article Contributors: 
    Sean Klepper M.D.
    Artur Zembowicz M.D....

    Clinical Features:

    • 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.

    Histologic Features:

    • 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.
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    Cases associated with this book:

  • Cicatricial pemphigoid
    Author: Artur Zembowicz M.D. Ph.D.

    Conference: Dr. Z's Consultations