Skin-nontumor / Clinical Dermatology - Cicatricial pemphigoid

Home Chapter Home Jobs Conferences Fellowships Books

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.

Description
=========================================================================

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

Terminology
=========================================================================

● Also called benign mucous membrane pemphigoid
● Cicatrix means scar

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

Epidemiology
=========================================================================

● Usually elderly, F/M = 2:1

Clinical
=========================================================================

● 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)

Treatment
=========================================================================

● 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

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).