Skin-nontumor / Clinical dermatology
Blistering disorders
Cicatricial pemphigoid

Author: Mowafak Hamodat, M.D., MB.CH.B, MSc., FRCPC (see Authors page)

Revised: 10 June 2016, last major update July 2011

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Cicatricial pemphigoid [title]

Cite this page: Cicatricial pemphigoid. website. Accessed October 27th, 2016.
Definition / General
  • Also called benign mucous membrane pemphigoid
  • Cicatrix means scar
  • Usually elderly, F / M = 2 : 1
  • 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
  • Suppress blister formation; topical or systemic steroids, possibly immunosuppressants (eMedicine #1, #2)
Clinical Images

Images hosted on other servers:

Various bullae

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

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

Cicatricial pemphigoid

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