Nasreen Amanat.
Mucous Membrane Pemphigoid.
J Coll Physicians Surg Pak Jan ;12(3):182-4.

Many conditions present as intra-oral ulcerative and vesiculobullous lesions. A case report of mucous membrane pemphigoid is presented which highlights the importance of early diagnosis. A selective literature review with special reference to pathophysiology and treatment is also presented.

Case Report: A very distressed 45 years old male presented with the complaint of persistent oral ulceration which he had initially noticed when he went to Saudi Arabia 3 years ago. It had become progressively worse since then. The severe ulceration, associated pain and difficulty in swallowing prevented him from taking adequate diet, resulting in general physical weakness which jeopardized his job. He had been treated with a variety of mouthwashes and topical steroids in Pakistan and in Saudi Arabia with no improvement. On intra-oral examination he had partial dentition with generalized periodontitis. There were erosions of attached gingiva, buccal and palatal mucosa extending into soft plate and uvula with surrounding erythema. The Nikolsky`s sign was positive. Genital, ocular and cutaneous involvement was negative. Hematological investigations revealed hypochromic, microcytic anemia with Hb of 7.6 gms/dl. A provisional diagnosis of mucous membrane pemphigoid was made. Incisional biopsy of buccal mucosa was performed under local anesthesia and the specimen sent for direct immunofluorescense, which revealed linear binding of autbantibody and C3 to the basement membrane zone of the oral epithelium. The result confirmed the provisional diagnosis. In consultation with Department of Medicine it was decided that the severity and extensive nature of the oral ulceration, associated pain and dysphagia warranted initial treatment with systemic steroids. The patient was given the following prescription and was asked to report back on day 7. 1. Prednisolone 40 mg / day for 6 days. 2. Multivitamin with iron supplement. 3. Chlorhexidine mouthwash.

On the following appointment patient reported marked relief in oral discomfort. Examination revealed healing ulcers and absence of new lesions in the mouth. This improvement allowed the patient to take soft oral diet. Prednisolone was then tapered off as follows: 30 mg/day for 3 days. 20 mg/day for 3 days. 10 mg/day for 3 days. Patient was reviewed again on day 10. He was clinically asymptomatic. Oral prednisolone was discontinued and replaced with triamcinolone in orabase, to be applied intraorally 4 times a day. He was advised to continue with the nutritional supplement and chlorhexidine mouthwash. Two weeks after discontinuation of systemic steroid and maintenance on topical steroid, he continued to remain asymptomatic. Subsequently, he failed to keep up the followup appointment scheduled for 2 month later.

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