cicatricial pemphigoid (benign mucous membrane pemphigoid)

Introduction

A phenotype for a variety of different immunologically different patterns.

Etiology

• autoimmune
• drug-induced
  • penicillamine
  • indomethacin
  • practolol
  • clonidine
  • topical pilocarpine

Epidemiology

• middle-age to elderly
• rare, 1 case/million/year

Pathology

• deposition of C3 & IgG in a linear pattern along the basement membrane (similar to bullous pemphigoid)
• IgA deposition in addition to C3 & IgG in 20%
• anti-epiligrin antibodies (laminin-5 Ab)

Clinical manifestations

• vesiculobullous lesions, erosions, & milia with eventual scarring
• mucosal with limited or no cutaneous lesions
• affects oral & ocular mucous membranes & less frequently genital, pharyngeal or upper respiratory mucosa
• oral musosa almost always involved
• conjunctival lesions common

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Laboratory

• biopsy (oral mucosal tissue better than conjunctiva)
• serum IgG antibasement membrane antibodies detected by indirect immunofluorescence
  • routine serum testing generally performed with monkey esophagus as substrate
  • sensitivity increased by use of NaCl-split human skin as substrate

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Complications

• untreated ocular involvement may lead to blindness
• may be increased risk of malignancy

Differential diagnosis

• cicatricial pemphigoid can resemble pemphigus vulgaris but usually involves only oral mucosal tissues

Management

treatment to prevent permanent ocular or oral scarring

• systemic corticosteroids
• dapsone
• azathioprine
• cyclophosphamide (especially in patients with ocular involvement)
• similar to bullous pemphigoid

More general terms

• pemphigoid

Additional terms

• bullous pemphigoid; parapemphigus

Patient information

cicatricial pemphigoid patient information