Skin nontumor
Vesiculobullous and acantholytic reaction patterns
Cicatricial pemphigoid
Last author update: 1 July 2011
Last staff update: 11 November 2020
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PubMed Search: Cicatricial pemphigoid [title]
See Also: Ocular cicatricial pemphigoid
Table of Contents
Definition / general | Terminology | Epidemiology | Etiology | Clinical features | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Differential diagnosisCite this page: Hamodat M. Cicatricial pemphigoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorcicatricialpemphigoid.html. Accessed March 22nd, 2023.
Definition / general
- Rare autoimmune bullous disease with mucosal lesions, including conjunctiva; skin involvement in 30%
Terminology
- Also called benign mucous membrane pemphigoid
- Cicatrix means scar
Epidemiology
- Usually elderly, F:M = 2:1
Etiology
- 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
Clinical features
- 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)
Case reports
- 53 year old man with multiple sites of mucosal involvement (Acta Otorhinolaryngol Ital 2009;29:222)
Treatment
- Suppress blister formation; topical or systemic steroids, possibly immunosuppressants (eMedicine #1, #2)
Clinical images
Images hosted on other servers:
Various bullae
Microscopic (histologic) 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
Microscopic (histologic) images
Images hosted on other servers:
Vacuolar change and subepidermal bullae with perivascular infiltration in dermis; indirect immunofluorescence on NaCl split skin shows antibodies on dermal side
Linear IgG deposition
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 (pseudoocular cicatricial pemphigoid), which is self limiting and usually develops after long term use of glaucoma medication
