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Skin-nontumor / Clinical Dermatology

Other dermatoses

Cicatricial pemphigoid

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 4 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.


● Rare autoimmune bullous disease with mucosal lesions, including conjunctiva; skin involvement in 30%
● See also ocular cicatricial pemphigoid


● Also called benign mucous membrane pemphigoid
● Cicatrix means scar


● Due to circulating autoantibodies against basement membrane proteins including BP180
● Complication of D- penicillamine therapy
● Occurs after acute severe ocular inflammation in patients with Stevens-Johnson syndrome


● Usually elderly, F/M = 2:1


● Affects mucosa of mouth and eyes; lesions heal with scarring
● May cause blindness, supraglottic stenosis or airway obstruction
● Cutaneous cases present with tense blisters and erosions, often on head, neck or sites of trauma
● May have low titer of circulating antibodies
Anti-epiligrin subtype: uncommon, primarily affects mucous membranes but also skin; involves autoantibodies against subunit of epiligrin (laminin 5, laminin 332); associated with higher incidence of solid cancers and mortality from treatment with systemic immunosuppressive drugs (Dermatol Online J 2008;14:3)

Clinical images


Various bullae

Case reports

● 53 year old man with multiple sites of mucosal involvement (Acta Otorhinolaryngol Ital 2009;29:222)


● Suppress blister formation; topical or systemic steroids, possibly immunosuppressants (eMedicine #1, #2)

Micro description

● Similar to bullous pemphigoid with subepidermal blisters
● Subepidermal vesicle contains edema fluid, fibrin and variable inflammatory cells
● Perivascular lymphohistiocytic infiltrate, plasma cells and neutrophils
● Fewer eosinophils than generalized bullous pemphigoid
● Conjunctival squamous metaplasia with foci of hyperkeratosis and parakeratosis, accompanied by goblet cell depletion; conjunctival vesicles or bulla are rare

Micro images

Vacuolar change and subepidermal bullae with perivascular infiltration in dermis; indirect immunofluorescence on NaCl split skin shows antibodies on dermal side

Various images

Linear IgG deposition

Indirect immunofluorescence on NaCl split skin shows IgG antibodies on dermal side

Positive stains

● Direct immunofluorescence shows linear C3 and IgG staining along basement membrane zone
● Salt-split skin indirect immunofluorescence shows C3 and IgG binding to dermal side of split

Electron microscopy description

● Antibodies in lamina lucida

Differential diagnosis

Bullous pemphigoid: may present with mucosal involvement but salt-split skin testing shows C3 and IgG binding to epidermal side of split
Epidermolysis bullosa acquisita: blisters form shortly after birth due to pressure, rubbing or trauma
● Ocular involvement should not be confused with drug induced pemphigoid (pseudo–ocular cicatricial pemphigoid), which is self-limiting, and usually develops after long term use of glaucoma medication

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Cicatricial pemphigoid

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