PMC6362419_01
Bronchoscopy showing multiple ulcers of the pharynx, highly vulnerable mucous membranes and granulomatous changes of the vocal cords.
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F1
To exclude an involvement of trachea a bronchoscopy was done revealing multiple ulcers of the pharynx, highly vulnerable mucous membranes and granulomatous changes of the vocal cords (Figure 1).
Immunoblotting of extracellular matrix showing circulating IgG4-autoantibodies to gamma2-chain of laminin-332.
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F2
As serology testings were negative, immunoblotting of extracellular matrix was performed, which was positive for circulating IgG4-autoantibodies to gamma2-chain of laminin-332 (Figure 2).
Active bilateral stage 4 symblepharon according to Tauber und Foster classification.
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F3
Given both, the critical laryngal involvement causing dyspnoea and the ocular bilateral stage 4 symblepharon according to Tauber und Foster classification (Figure 3), systemic treatment was initiated.
Post-treatment pictures showing a stable ocular involvement and no new enoral lesions 5 month after rituximab treatment.
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F4
The follow-up examination 8 weeks later revealed a stable disease with no new oral lesions (Figure 4).