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Cicatricial pemphigoid presenting as upper airways obstruction
A S Yong1, J S Elborn, C F Stanford
1Royal Victoria Hospital, Belfast.
Summary
Cicatricial pemphigoid, a rare autoimmune condition, can cause upper airway obstruction. Immunosuppressive therapy effectively treated a patient with shortness of breath and stridor.
Area of Science:
- Ophthalmology
- Pulmonology
- Otolaryngology
Background:
- Cicatricial pemphigoid is a chronic autoimmune blistering disease that can affect mucous membranes.
- Ocular and laryngeal involvement can lead to significant morbidity.
Observation:
- A 53-year-old woman presented with progressive shortness of breath, stridor, and dry eyes.
- Pulmonary function tests, specifically flow volume loops, indicated upper airway obstruction.
Findings:
- Conjunctival and laryngeal biopsies confirmed cicatricial pemphigoid.
- The patient's respiratory symptoms and flow volume loop abnormalities resolved with immunosuppressive therapy.
Implications:
- This case highlights the importance of considering cicatricial pemphigoid in patients with unexplained upper airway obstruction and ocular symptoms.
- Early diagnosis and appropriate immunosuppressive treatment are crucial for managing respiratory complications of cicatricial pemphigoid.