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cicatricial pemphigoid (benign mucous membrane pemphigoid)

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: 1) deposition of C3 & IgG in a linear pattern along the basement membrane (similar to bullous pemphigoid) 2) IgA deposition in addition to C3 & IgG in 20% 3) anti-epiligrin antibodies (laminin-5 Ab) [3] Clinical manifestations: 1) vesiculobullous lesions, erosions, & milia with eventual scarring 2) mucosal with limited or no cutaneous lesions 3) affects oral & ocular mucous membranes & less frequently genital, pharyngeal or upper respiratory mucosa 4) oral musosa almost always involved 5) conjunctival lesions common * images [4,5] Laboratory: 1) biopsy (oral mucosal tissue better than conjunctiva) 2) serum IgG antibasement membrane antibodies detected by indirect immunofluorescence a) routine serum testing generally performed with monkey esophagus as substrate b) sensitivity increased by use of NaCl-split human skin as substrate * image [4] 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 [5] Management: treatment to prevent permanent ocular or oral scarring 1) systemic corticosteroids 2) dapsone 2) azathioprine 4) cyclophosphamide (especially in patients with ocular involvement) 5) similar to bullous pemphigoid

Related

bullous pemphigoid; parapemphigus

General

pemphigoid

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 167
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015.
  3. Egan CA, Lazarova Z, Darling TN, Yee C, Cote T, Yancey KB. Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet. 2001 Jun 9;357(9271):1850-1. PMID: 11410196
  4. Freiman A, Elston DM (images) Medscape: Cicatricial Pemphigoid http://emedicine.medscape.com/article/1062534-overview
  5. DermNet NZ. Mucous membrane pemphigoid (images) http://dermnetnz.org/immune/cicatricial-pemphigoid.html
  6. Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022