(Figure 3.27A, B) - Rare autoimmune disease involving the mucous membrane with subsequent scarring
- Autoantigen:
- BPAG2 (collagen XVII): mucosa and skin
- β4 subunit of α6β4 transmembrane hemidesmosomal protein (pure ocular)
- Laminin 5 (aka epiligrin): ↑ risk cancer
| | | | | | Anti-epiligrin CP binds
DERMAL side | | | | | |
|
- Clinical: ocular symptoms (conjunctivitis, burning or foreign body sensation), entropion, trichiasis with subsequent corneal irritation, corneal neovascularization, scarring, fusion of palpebral and bulbar conjunctivae, symblepharon, and blindness; may also involve mouth (erosions, ulcers, desquamative gingivitis), oropharynx, nasopharyngeal, esophageal, rectal and genital mucosa; skin lesions in 25% cases
- Drug-induced (similar to BP): thiol-containing (captopril, gold thiosulfate, D-penicillamine), NSAIDs (indomethacin), topical glaucoma solutions, β-blockers (practolol), clonidine, sulfadoxine
- Histology/DIF: similar to BP
- IIF: + in 20% cases; IIF on (SSS) shows binding to epidermal side of split (roof), however, anti-laminin 5 CP binds to dermal side
- Treatment: dapsone first line for oral with cutaneous disease, topical/ intralesional/oral corticosteroid, cyclosphosphamide, azathioprine
| | Figure 3.27
A: Cicatricial pemphigoid
(Courtesy of Dr. Iris K.
Aronson)
B: Cicatricial pemphigoid
(Courtesy of Dr. Iris K.
Aronson)
C: LABD, DIF
(Courtesy of Dr. Lawrence
Chan) |
|
