INTRODUCTION
Mucous membrane pemphigoid (MMP) is a rare, chronic, immune-mediated,
subepithelial blistering mucocutaneous disorder. 1 Pemphigoid was
initially described by Wichmann in 1794 and has since undergone several
classifications. In 1951, Lever coined the term pemphigoid for this family
of disorders. 2 The term cicatricial pemphigoid is one of the oldest terms,
dating back to 1962, and refers to mucous membrane involvement. 2, 3
Now, this disorder is more commonly referred to as MMP.
In general, MMP affects oral mucosa, with predominant gingival involvement. 4
Typically, MMP affects people aged 60 to 80 years and is more prevalent in
females. 4, 6
Symptoms include pain, dysphagia, difficulty eating, erythematous gingiva that bleed
easily, and difficulty maintaining appropriate oral hygiene. Ocular symptoms
include chronic conjunctivitis, burning, irritation, photophobia, and excessive
tearing. 1 When only oral lesions are present, the condition is referred to as oral
mucous membrane pemphigoid. 5 Ocular mucosa is the next most commonly
affected area, followed by involvement of the skin, nasal cavity, anogenital area,
pharynx, larynx and, rarely, esophagus. 6, 7
Symptom severity varies with some cases presenting with mild oral lesions while
others exhibiting pervasive involvement . 7 Often, a positive Nikolsky sign may be seen,
which is the epithelial sloughing upon pressure from manipulating the affected
tissue. Clinical oral signs include bullae formation, which rupture and result in
ulceration. Patients can also present with desquamative gingivitis and mucosal
scarring. 1 The oral lesions of MMP can be confused with other ulcerative conditions
and chronic mucocutaneous disorders. Therefore, MMP must be differentiated
from these and other pemphigoid disorders, such as bullous pemphigoid,
pemphigoid gestationis, dermatitis herpetiformis, and linear IgA disease. 1 The
differential diagnoses include oral lichen planus, pemphigus, epidermolysis bullosa
acquisita, bullous systemic lupus erythematosus, and erythema multiforme. 6, 9
Mucosal scarring can have severe consequences, especially in ocular involvement.
Scarring may lead to blindness due to symblepharon or ankyloblepharon, which
involves fusion of the scleral and palpebral conjunctiva or of the superior and
inferior palpebrae, respectively. 1, 4, 6, 7 Other ocular manifestations include entropion
and trichiasis. 1 The annual risk of developing ocular lesions is 5% over the initial 5
years. 7 Other mucosal involvement such as esophageal and laryngeal scarring can
lead to stenosis and strictures. 8 In some cases, MMP is associated with a malignancy. 1,7
Without a careful history and knowledge of these disorders, the correct diagnosis
is often delayed. Therefore, these cases can present a diagnostic challenge for
most dentists and physicians. Patients often see several providers from different
specialties and undergo many diagnostic tests and procedures. This delay can
be prevented with a comprehensive history and understanding of the etiology and
clinical presentation of mucocutaneous disorders. This case describes the
diagnosis and management of a patient who reported a non-healing gingival
ulceration despite multiple treatments and medical