Joen Iannucci Haring, DDS,
History
When questioned about the involved areas, the patient claimed the bleeding gums had been present for approximately 10 days. The patient described the involved area as "raw and painful" and did not recall a history of injury to the area. When questioned about blister formation, the patient stated that blisters had formed on the gums and then collapsed within a day or so. The patient denied the use of any cinnamon-flavored oral products (chewing gum, mints, or mouth rinse).
The patient had a previous history of regular and routine dental care. At the time of the dental appointment, the patient appeared to be in an overall good state of health. No significant problems were noted during the medical history.
Examinations
Physical examination of the head and neck region revealed no abnormal findings. The patient`s vital signs were all found to be within normal limits. Several enlarged, palpable, and tender lymph nodes were detected in the cervical region. No other abnormal extraoral findings were noted. Intraoral examination revealed large, diffuse areas of gingival involvement. The areas appeared to be erosive, ulcerated, and bleeding (see photo). Several intact bullae were noted at the time of the examination.
Clinical diagnosis
Based on the clinical information presented, which of the following is the most likely diagnosis?
* erosive lichen planus
* carcinoma in situ
* discoid lupus erythematosus
* benign mucous membrane pemphigoid
* pemphigus vulgaris
Diagnosis
benign mucous membrane pemphigoid
Discussion
Benign mucous membrane pemphigoid (BMMP) is a chronic disease that is believed to be autoimmune in origin. The term benign refers to the fact that this disease is not deadly; mucous membrane refers to the tissues of involvement, and pemphigoid means resembling pemphigus (another bullous autoimmune disease).
BMMP is characterized by the formation of bulla, followed by the appearance of painful erosions and ulcerations. BMMP is relatively uncommon, although it may occur more frequently as a result of misdiagnosis. This disease has a distinct female predilection; females are affected twice as often as males. BMMP is diagnosed in middle-aged and older adults, most often over the age of 60.
The bullous lesions of BMMP involve the oral mucous membranes and conjunctiva of the eyes. Cutaneous lesions, although uncommon, may be found on the skin of the head and neck region as well as the extremities. Other areas that may be involved include the mucous membranes of the nose, larynx, esophagus, and genitalia.
Clinical features
BMMP is characterized by multiple intraoral vesicles and bullae. These lesions develop slowly and are thick-walled. The bullae usually persist for one to two days and then rupture. A raw, eroded, and bleeding surface results. The oral lesions of BMMP are typically seen on the attached gingiva. Other sites of involvement include the palate, alveolar mucosa, tongue, floor of the mouth, and buccal mucosa.
"Sore, bleeding gums" is often the chief complaint of a patient with BMMP. The severity of the pain experienced by the patient parallels the extent of the mucosal involvement. In severe cases, eating and oral hygiene may cause extreme pain. It is important to note that the oral lesions of BMMP are chronic and may persist for months, if left untreated. BMMP is also referred to as cicatricial pemphigoid; the term cicatricial refers to scarring (cicatrix means scar). After the erosions heal, the affected area may remain erythematous for weeks or months.
In 25 percent of patients with BMMP, the conjunctiva, or delicate membrane that lines the eyelid and covers the eyeball, is also affected. The lesions that affect this mucous membrane heal with scarring. As a result, fibrous bands of tissue are seen extending from the lid to the sclera. This ocular finding, along with intraoral bullae, is diagnostic for BMMP. Ocular involvement may result in blindness, which is, by far, the most serious complication of this disease.
Diagnosis and treatment
The oral lesions of BMMP may resemble other vesiculo-bullous diseases. Diseases to consider in the differential diagnosis include pemphigus vulgaris, erythema multiforme, and erosive lichen planus. Biopsy and histologic examination, as well as a specialized test known as immunofluorescence, is required in order to establish a definitive diagnosis. Histologic examination of BMMP exhibits subepithelial clefting. Direct immunofluorescence studies of perilesional oral mucosa exhibit a linear pattern of immune reactants consisting of lgG and C3.
In mild cases of BMMP, topical corticosteroid preparations (such as Lidex gel) may be recommended to provide symptomatic relief and aid resolution of the lesion. If there is extensive involvement of the oral mucosa, systemic corticosteroids plus other immunosuppressive agents are indicated.
Prognosis
BMMP has a variable clinical course and prognosis. The oral lesions are chronic and characteristically exhibit exacerbation and remissions over a period of many years. Although BMMP does not cause death, BMMP that involves the conjunctiva of the eyes is serious. Early ocular treatment is critical because lesions that result in scarring may progress rapidly and cause blindness. A patient with ocular lesions must be referred to an ophthalmologist for evaluation and treatment. Patients with oral lesions should also be evaluated and followed by an ophthalmologist. A patient with severe oral lesions may be referred to an oral pathologist for long-term management.
Joen Iannucci Haring, DDS, MS, is an associate professor of clinical dentistry, Section of Primary Care, The Ohio State University College of Dentistry.
