Skin nontumor
Vesiculobullous and acantholytic reaction patterns
Cicatricial pemphigoid


Topic Completed: 1 July 2011

Minor changes: 23 July 2020

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PubMed Search: Cicatricial pemphigoid [title]

See Also: Ocular cicatricial pemphigoid

Mowafak Hamodat, M.B.Ch.B., M.Sc.
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Cite this page: Hamodat M. Cicatricial pemphigoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorcicatricialpemphigoid.html. Accessed October 24th, 2020.
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
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
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