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Benign mucous membrane pemphigoid is a relatively rare disease characterised by the presence of bullous lesions and erosions of the mucous membranes leading to scarring. Oral and conjunctival mucosae are most frequently affected. Occasionally, the oesophagus is involved and two radiological patterns, webs and smooth strictures, are seen.
Cicatricial pemphigoid is a chronic mucocutaneous bullous condition. It is a heterogenous autoimmune disease, characterized by the production of autoantibodies against basement membrane zone antigens. The target antigens in cicatricial pemphigoid appear to be lamina lucida proteins involved in human keratinocyte adhesion to extracellular matrix. Cicatricial pemphigoid primarily affects persons older than 40 years and appears to have a 2:1 predilection for women, without racial or geographic bias.
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Benign mucous membrane pemphigoid (BMMP) is a relatively rare, chronic vesiculobullous disease. It frequently affects postmenopausal women, although cases have been reported in younger individuals. Benign mucous membrane pemphigoid has a predilection to affect multiple mucosal surfaces including the gingivae, hard and soft palate, alveolar ridge, nose, pharynx, gastrointestinal tract, genitalia and the conjunctiva. It is seen clinically as bullae or erosions on the mucosae or gingivae. The bullae rupture after 24-48 hours and the erosions heal within 7-14 days, sometimes with scar formation. Scarring frequently occurs with ocular mucosa involvement and may contribute to blindness. In order to make a diagnosis, the clinical features must be correlated with microscopic and immunopathological findings. Benign mucous membrane pemphigoid is treated with high doses of corticosteroids and immunosuppressive agents. This paper is a presentation of a case report in a 36-year-old woman.
This case of benign mucous membrane pemphigoid (BMMP) is unusual in that blistering, scarring lesions were confined to the skin for 15 years before mucous membranes were involved. The onset of this disorder at the age of 38 is also unusual. Detailed immunological investigation was performed on this patient but the results in no way clarify the present confusion regarding the immunopathological processes in BMMP related to those operative in bullous pemphigoid.
Three cases of benign mucous membrane pemphigoid were investigated by direct and indirect immunofluorescence. The findings of the direct immunofluorescent examination of perilesional tissue are studied in detail. Finally the relationships between bullous pemphigoid and benign mucous membrane pemphigoid are discussed.
Benign mucous membrane pemphigoid, in the form of desquamative gingivitis, occurred in a 57-year-old woman. The disease then developed in her 59-year-old husband 16 months later. The clinical manifestations were mild and similar in both cases. Direct immunofluorescence microscopy tests were consistent with the diagnosis. Sequential occurrence of this disease in spouses suggests the possibility that it is transmissible.
A relatively rare systemic disease, benign mucous membrane pemphigoid (cicatricial pemphigoid) usually starts in the mouth and is clinically characterized by bullae that rupture and form an ulcer. A distinctive Nikolsky's sign is apparent using gentle air blasts or finger pressure. The pharynx, larynx, nose, esophagus, genitals and eyes can also be affected. Involvement of the conjunctivae can lead to scarring and ensuing blindness. The following case of a healthy 77-year-old man, diagnosed as suffering from mucous membrane pemphigoid, is of particular interest since several confusing clinical observations, including poor oral hygiene, the possibility of a contact dermatitis or an adverse antibiotic reaction, made the diagnosis more difficult. A careful medical history, examination and consultation process is paramount to initiating proper treatment and subsequent relief of symptoms. Benign mucous membrane pemphigoid must always be considered in any patient with desquamative epithelium of the oral mucosa.
A 67-year-old white man with benign mucous membrane pemphigoid OU developed, over a period of two months, a slightly pedunculated fleshy mass in the bulbar conjunctiva OS. The mass was excised and found histopathologically to be squamous cell carcinoma of the conjunctiva. The association of benign mucous membrane pemphigoid and squamous cell carcinoma of the conjunctiva has not been previously reported in the English literature to our knowledge. The significance of the simultaneous occurrence of these two conditions is discussed.
The painful oral lesions of benign mucous membrane pemphigoid often make the wearing of complete dentures difficult or impossible. A mandibular split-thickness skin graft vestibuloplasty was performed on a patient with benign mucous membrane pemphigoid. This procedure may be indicated in those cases which do not respond to meticulous denture construction and steroid therapy.
OBJECTIVE: To determine the location of the subepithelial split in benign mucous membrane pemphigoid (BMMP) and its relationship to the anchoring filaments and their receptors. MATERIALS AND METHODS: Frozen sections of lesional and perilesional oral mucosa from 10 cases of BMMP were stained, using an immunofluorescence method, for the beta-1, beta-4, alpha-3 and alpha-6 integrin subunits and for their ligands, laminin I and laminin V (kalinin). In all cases the diagnosis was confirmed by the demonstration of linear staining for IgG at the basement membrane zone. Six specimens of normal mucosa were stained for comparison. RESULTS: Staining for integrins, laminin and kalinin in perilesional mucosa was similar to normals, although one case showed loss of alpha-6 and beta-4 . In lesional mucosa, laminin and kalinin showed strong linear staining localised to the floor of the bullae. The alpha-6 and beta-4 subunits were expressed only on the roof of the bullae but staining was weak and patchy with areas of loss. In some sections alpha-6 showed a punctate intracellular distribution similar to IgG. The distribution of alpha-3 and beta-1 was similar to that seen in normals. CONCLUSIONS: In all cases kalinin was found on the connective tissue side of the lesions and alpha-6 beta-4 localised to the epithelial side. This shows that the split occurs at a location which separates anchoring filaments from the hemidesmosomes. Loss of the alpha-6 beta-4 integrin in the lesions and the similar intracellular staining of alpha-6 and IgG, suggest that disruption of hemidesmosomes may be a key event in the immunopathogenesis of the lesions and that the alpha-6 integrin subunit is a potential antigen in oral mucosal BMMP.
Benign mucous membrane pemphigoid is a rare autoimmune disorder affecting the upper aero-digestive tract and conjunctivae. This is a case presentation of benign mucous membrane pemphigoid affecting the oral mucosa, pharynx, oesophagus and larynx, leading to cicatricial lesions in the pharynx and larynx, causing dysphagia, hoarseness and stridor. The alternative forms of management for laryngeal scarring due to this disease are explained. The patient was later diagnosed with advanced renal cell carcinoma, raising the possibility of cicatricial pemphigoid manifesting as a paraneoplastic syndrome of underlying renal cell carcinoma.
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A patient with benign mucous membrane pemphigoid was found to have metastatic carcinoma of the lung. With so few similar case reports in the literature, the possible association of this blistering disorder with internal malignancy cannot be disclaimed totally, as appears to be the case for bullous pemphigoid which has been studied much more extensively.
This paper describes a case of benign mucous membrane pemphigoid which affected the periodontal tissues and complicated ocular prosthesis placement. The clinical findings, diagnostic criteria, and therapeutic management of this disorder are reviewed.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.