Mucous membrane pemphigoid as a cause of acute dysphagia – an endoscopic study

Mucous membrane pemphigoid (MMP, formerly cicatricial pemphigoid) is a chronic autoimmune, subepithelial blistering disease usually of middle aged or elderly patients, more common in women. The prevalence is uncertain and the aetiology is still unknown. Target antigens are epithelial basement membrane proteins BPAG-1, BPAG-2, laminin-331, and laminin-332 [1]. Oral and conjunctival membranes are most frequently affected. Oesophageal involvement is rare but may be complicated with persistent dysphagia [2].
Herein, we present a patient with MMP with acute dysphagia evaluated by upper gastrointestinal (GIT) endoscopy. This study is based on a case report of a 75-year-old patient with MMP. Clinical and endoscopic examinations as well as direct immunofluorescence (DIF) of conjunctival biopsy were used as methods in this study.
A 75-year-old woman was referred to the Dermatology Clinic in November 2011 due to painful erosions in the oral cavity and dysphagia. Her past medical history was significant for dry-eye sensations with visual disturbances diagnosed as ocular MMP in 2007 confirmed by DIF (IgA+ deposits) and treated with dapsone. On physical examination there were erosions and haemorrhagic blisters on the hard palate (Figure 1 A). Inspection of the eyes revealed bilateral conjunctival hyperaemia and fibrous adhesions between bulbar and palpebral conjunctiva (symblepharon) (Figure 1 B). No laboratory changes were found, and autoantibodies in the blood (indirect immunofluorescence) were negative. An endoscopic examination of the upper GIT was performed and revealed: 1) white fibrin with or without flat erosions on the mucosa of cricopharynx, along the oesophagus, and on the lining of the stomach body and prepyloric region; 2) two flat, linear erosions, about 10 mm long on the lower third of the oesophagus; 3) hiatal hernia with line “Z” 4 cm above the diaphragm; and 4) spastic but passable pylorus and erythematous mucosa of duodenal bulb (Figure 1 C). An endoscopic biopsy from the oesophagus showed separation between epithelium and mucosa (Figure 1 D). Given the final diagnosis of MMP with prominent conjunctival and oro-oesophageal involvement, the patient was started on methylprednisolone 16/8 mg per day, which resulted in resolution of oral lesions and dysphagia. She remains stable on maintenance therapy with low-dose methylprednisolone. At 18-months’ follow-up the patient remains under control.
Most patients (89–100%) manifest with oral...


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