Mucous membrane pemphigoid and oral blistering diseases

Abstract

Summary The autoimmune blistering disorders present with variable frequency in the oral cavity. Recognition of their key clinical features at presentation is important, as there are many causes of oral ulceration. Careful history‐taking, clinical examination, an understanding of pathogenesis and appropriate investigations are essential. With the exception of the rare genodermatoses that may lead to blistering and oral ulceration, the majority of patients have an acquired disorder. These include the rare autoimmune blistering diseases mucous membrane pemphigoid (MMP), pemphigus vulgaris (PV), linear IgA disease, epidermolysis bullosa acquisita and paraneoplastic pemphigus. Important clinical differential diagnoses include erythema multiforme, which may be mistaken for PV in appearance, while oral lichen planus may be indistinguishable from MMP. Angina bullosa haemorrhagica may also present with tense haemorrhagic bullae, and in the absence of diagnostic tests, requires an astute clinical diagnosis based upon the history. Newer laboratory techniques have facilitated identification of target antigens and epitopes in the autoimmune blistering diseases, particularly in MMP. Current interest is in whether these relate to clinical presentation and outcomes. There have also been recent investigations into the use of saliva as an alternative medium to serum for the diagnosis of oral vesiculobullous lesions. Assessment of disease severity and measurement of quality of life at presentation and subsequent follow‐up is paramount to interpreting therapeutic response. Furthermore, combining these scores with serological and/or salivary biomarkers is valuable in the assessment of clinical response. In this paper, we discuss MMP and its important differential diagnoses.

Mucous membrane pemphigoid in a patient with chronic hepatitis B virus infection

“…In the majority of patients, the oral mucosa is the site of onset and is the most frequently involved (85%) site. [ 4 , 11 ] As in our case, detecting intact vesicles or bullae on the mucosa is uncommon. [ 2 ] Gingival presentation is often desquamative, and in mild cases, manifests as erythema or edema.…”

“…The areas involved ranged from the gingiva, oral mucosa, tongue, palate, eyes, nose, nasopharynx, hypopharynx, larynx, esophagus, genitals, to the anus. [ 4 ] The cutaneous involvement typically included the head, neck, or upper trunk. The disease is broadly heterogeneous regarding site and severity of involvement in patients.…”

“…As the literature describes, the disease course is fluctuating with relapses and remissions. [ 4 ] The broadly heterogeneous involvement and severity of MMP is the main challenge for medical practitioners to make a tentative diagnosis. Moreover, the patient presenting to different specialties (oral medicine, otolaryngology, rheumatology, ophthalmology, dermatology, and family medicine) makes this case noteworthy.…”

Approach to the aphthous lesions of the oral mucosa

“…52 Lesions may affect oral mucosa, conjunctiva, anogenital tissues, and upper aerodigestive tract and may lead to scarring in mucous membranes, skin, or both. 53 In most patients, the oral mucosa is the site of onset and the most frequent area of involvement (85%) in the disease process. 54 Gingiva (80%), buccal mucosa (58%), and palate (26%) are the most affected localizations in the oral mucosa.…”

Mucous membrane pemphigoid in a patient treated with nivolumab for Hodgkin's lymphoma

“…autoreactive T cell proliferation and to restricting Treg activity. 4,6 Another theory is that MMP might be triggered by malignancy as a paraneoplastic disease, and nivolumab can be an incidental agent in this process. 3 F I G U R E 1 Erythematous, erosive patches on the maxillary, A, and mandibular gingivae, B MMP is potentially a devastating disease leading to tissue destruction, fibrosis, and loss of function; however, limited disease to the oral mucosa is an indicator of good prognosis and well response to the treatment.…”