cicatricial pemphigoid

Question 1

manif tx

Answer 1

■ Arare disease, largely o the elderly.
■ Ocular involvement may initially mani est as unilateral or bilateral conjunctivitis with burning, dry-
ness,and oreign-bodysensation.
■ Blisters that rupture easily and erosions resulting rom epithelial ragility in the conjunctivae, mouth,
oropharynx, and, more rarely, the nasopharyngeal, esophageal, genital, and anal mucosae.
■ Chronic involvement results in scarring, symblepharon (Fig. 6-15), and, in severe disease, usion o
thebulbarandpalpebralconjunctiva.Entropionandtrichiasisresultincornealirritation,super cial
punctate keratinopathy, corneal neovascularization, ulceration, and blindness.
■ Scarring also in the larynx; stricture ormation in the esophagus, dysphagia, or dynophagia.
■ Blisters on the skin in roughly 30% o patients.
■ Brunsting-Perrypemphigoiddescribesasubseto patientswhoseskinlesionsrecuratthesamesites,
mainly on the head and neck and scalp, which can also lead to scarring.
■ AntigenstowhichautoantibodiesmaybedirectedincludeBPAG1,BPAG2,integrinsubunitsβ4 and
α 6 , t y p e VI I c o l l a g e n , a n d l a m i n i n 3 3 2 ( s e e F i g . 6 - 1 ) .
■ Management:Mildinvolvement—topicalcorticosteroids,calcineurininhibitors(tacrolimus,pimecro-
limus). Moderate and severe involvement: dapsone in combination with prednisone.