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Mucous Membrane Pemphigoid (mucous + membrane_pemphigoid)

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Medical Sciences	100%

Selected Abstracts

Mucous membrane pemphigoid, thymoma, and myasthenia gravis

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 9 2000
Haideh Yazdani Sabet
In November 1997, approximately 1 year before being evaluated at the Mayo Clinic, Rochester, a 63-year-old woman presented with erosive tongue lesions that were diagnosed by her physician as oral lichen planus. The lesions responded well to 3 months of treatment with systemic and topical corticosteroids and topical antiyeast medication. She stopped taking the medications and had a relapse. A few months after the oral lesions developed, her left eyelid became ptotic. Results of magnetic resonance imaging of her brain were normal, and the ptosis resolved spontaneously after 2 weeks. One year later, her right eyelid began to droop, and the results of edrophonium testing were positive. She was prescribed prednisone, 30 mg daily, and pyridostigmine, as needed. The ptosis improved, but never fully resolved. Radiography revealed a left ,,thyroid nodule,'' but computed tomography did not show a mediastinal mass. She was advised to have the ,,nodule'' removed surgically and came to the Mayo Clinic, Rochester, for a second opinion. Her medical history was significant for the following: tinnitus, glaucoma, early bilateral cataracts, and long-standing hypertension, for which she took losartan, 50 mg twice daily. Other medications included: prednisone, 30 mg daily; pyridostigmine as needed; famotidine, 40 mg daily; and eyedrops for glaucoma. She denied any history of hyperthyroidism or hypothyroidism, head and neck irradiation, family history of thyroid disease, or diplopia. Hepatitis serologic studies revealed hepatitis B exposure and recovery, hepatitis C immunity, and a previous hepatitis A viral infection. On examination at the Mayo Clinic, Rochester, an erosive hypertrophic plaque was noted on the posterior dorsal half of the tongue, and vesicles and erythematous erosions on the hard and soft palates ( Fig. 1a). A lace-like white pattern was seen on the buccal mucosa bilaterally, and a small erosive patch on the left buccal mucosa ( Fig. 1b). Ocular and nasal mucous membranes were normal in appearance, and there were no pertinent skin findings. Dermatopathologic examination of an excisional biopsy specimen from the left dorsum of the tongue demonstrated an ulcer with epitheliomatous hyperplasia and a granulomatous reaction, presumably due to yeast infection. Silver staining showed hyphae and yeast at the base of the tongue ulcer. The results of the direct immunofluorescence study were negative and revealed no lichenoid changes on hematoxylin and eosin staining. Indirect immunofluorescence testing of the serum revealed a 1 : 80 titer of basement membrane zone antibodies, reflecting pemphigoid. This test was positive on repeat study. Salt-split skin on monkey esophagus revealed an epidermal pattern of basement membrane zone antibodies. Treatment included fluocinonide gel applied to the involved areas four times daily and oral antiyeast therapy (fluconazole, 200 mg once daily by mouth) while the rest of the evaluation was being completed. Figure 1(a). Erosive hypertrophic tongue plaque. Figure (b) ,. Erosive patch on the buccal mucosa. As part of the evaluation of the ptosis, a myasthenia gravis antibody panel was performed. It revealed the following abnormalities: striated muscle antibody at 1 : 480 (reference range, <1 : 60), acetylcholine receptor binding antibody at 6.33 nmol/L (reference range, ,,0.02 nmol/L), acetylcholine receptor blocking antibody at 31% (reference range, 0,25%), and acetylcholine receptor modulating antibody at 100% (reference range, 0,20%), suggesting thymoma. Treatment included pyridostigmine, 30,45 mg 3,4 times daily, to control the myasthenia symptoms, while the ill-defined neck mass was being evaluated. A mildly enlarged thyroid was noted on physical examination. Hematology panel revealed thyroid-stimulating hormone (TSH) levels in the low normal range; the thyroid microsomal antibody was normal. Chest radiography showed minor tracheal deviation, and a previous computed tomogram showed what appeared to be a 3-cm enlarged mass in the thyroid. Ultrasonographically guided thyroid biopsy did not show malignancy, but a benign mesenchymal-type tumor was found and surgical excision was planned. Intraoperatively, a thymoma of the left cervical thymic tongue was found. At 6 months' follow-up, the ptosis and oral mucosal lesions had improved significantly, although she continued topical corticosteroid therapy intermittently for minor erosive oral disease. [source]

Oral mucous membrane pemphigoid in a 6-year-old boy: diagnosis, treatment and 4 years follow-up

INTERNATIONAL JOURNAL OF PAEDIATRIC DENTISTRY, Issue 1 2010
MOSTAFA I. MOSTAFA
Background., Childhood oral pemphigoid is extremely rare and usually takes the form of desquamative gingivitis. Case report., We describe a 6-year-old boy who presented with gingival bleeding, pain, eating difficulty, and peeling of the gums. Clinical examination revealed desquamative gingivitis with no extra-oral involvement. The diagnosis was established as oral pemphigoid based on the clinical, histological, and immunofluorescence findings. Symptoms resolved on treatment with occlusive topical corticosteroids. The patient was a carrier of the HLA-DQB1*0301 allele. Conclusion., Mucous membrane pemphigoid should be considered in the differential diagnosis of chronic desquamative gingivitis in childhood. Occlusive therapy with topical fluocinonide may alleviate the symptoms. [source]

Number III Mucous membrane pemphigoid

ORAL DISEASES, Issue 4 2005
J Bagan
Mucous membrane pemphigoid (MMP) is a sub-epithelial vesiculobullous disorder. It is now quite evident that a number of sub-epithelial vesiculobullous disorders may produce similar clinical pictures, and also that a range of variants of MMP exist, with antibodies directed against various hemidesmosomal components or components of the epithelial basement membrane. The term immune-mediated sub-epithelial blistering diseases (IMSEBD) has therefore been used. Immunological differences may account for the significant differences in their clinical presentation and responses to therapy, but unfortunately data on this are few. The diagnosis and management of IMSEBD on clinical grounds alone is impossible and a full history, general, and oral examination, and biopsy with immunostaining are now invariably required, sometimes supplemented with other investigations. No single treatment regimen reliably controls all these disorders, and it is not known if the specific subsets of MMP will respond to different drugs. Currently, apart from improving oral hygiene, immunomodulatory,especially immunosuppressive,therapy is typically used to control oral lesions. The present paper reviews pemphigoid, describing the present understanding of this fascinating clinical phenotype, summarising the increasing number of subsets with sometimes-different natural histories and immunological features, and outlining current clinical practice. [source]

Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity

BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2006
N. Oyama
Summary Background, Mucous membrane pemphigoid (MMP), a chronic autoimmune subepithelial blistering disease, is associated with circulating IgG and/or IgA autoantibodies against several basement membrane zone antigens. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically. Objectives, To analyse serum autoantibody profile and HLA class II alleles in MMP patients and to correlate this with the clinical presentation of disease. Methods, Well-defined subgroups consisting of 124 patients with MMP were examined for IgG and IgA reactivity with immunoblotting using human epidermal, dermal and placental amnion proteins. The results were further analysed on the basis of detailed clinical (sites of involvement and disease severity) and immunopathological criteria (immunofluorescence study and HLA class II alleles). Results, Immunoblot assay revealed that the majority of MMP patients had IgG (93 of 124, 75%) and/or IgA autoantibodies (63 of 124, 51%) to BP180 (including its soluble ectodomains, 120-kDa LAD-1 and 97-kDa LABD97 antigens). Other antigens targeted predominantly by IgG autoantibodies included: BP230 in 34 (27%), ,4 integrin in 26 (21%), and laminin 5 in three (2%). All the BP230+ sera and 23 (88%) ,4 integrin+ sera also reacted with at least one of the BP180 antigens. Over 85% of patients with reactivity to ,4 integrin had ocular involvement. In most cases of MMP, more severe clinical features were associated with antibody reactivity to multiple basement membrane zone antigens, as well as reactivity to multiple BP180 component antigens. Dual BP180/LAD-1 reactivity with IgG and IgA was associated with a more severe phenotype. In addition, the subset-dependent autoantibody reactivity correlated well with specific HLA class II alleles, DQB1*0301, DRB1*04 and DRB1*11. Conclusions, Our results confirmed that BP180 is a major autoantigen targeted by the sera of patients with MMP. The disease-prevalent HLA class II alleles and humoral autoimmune response against the particular subsets of antigenic epitope(s) within BP180 ectodomain may contribute to the clinicopathological significance and disease severity of MMP. [source]

Oral mucous membrane pemphigoid in a 6-year-old boy: diagnosis, treatment and 4 years follow-up

Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany

JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 5 2009
Franziska Bertram
Summary Background: Only limited epidemiologic data are available on autoimmune bullous diseases. Improved diagnostic tools should have led to an increased incidence. To test this hypothesis, all patients with autoimmune bullous disorders who were treated in the Department of Dermatology at the University of Würzburg, Germany, between January 2001 and June 2002 were analysed prospectively. Patients and Methods: Epidemiologic data of patients diagnosed with an autoimmune bullous disease during this time period were registered and statistically evaluated. Diagnosis was based on the clinical picture and specific immunopathological findings. Only patients from Lower Franconia, a well-defined administrative region of Southern Germany, were included into this study. Results: During the study period, 41 patients with an autoimmune bullous disease were diagnosed, including 27 with bullous pemphigoid, 4 with pemphigoid gestationis and mucous membrane pemphigoid, 2 with dermatitis herpetiformis and linear IgA disease, and 1 with epidermolysis bullosa acquisita and pemphigus vulgaris, respectively. The highest incidence was calculated for bullous pemphigoid (13.4 per 1 million inhabitants per year) followed by pemphigoid gestationis (2.0) and mucous membrane pemphigoid (2.0). Patients with mucous membrane pemphigoid were found to have the highest mean age at disease onset (76 years) followed by patients with bullous pemphigoid (74 years). Conclusions: This is the first prospective study on the incidence of autoimmune bullous disorders. Subepidermal blistering autoimmune diseases were shown to be more frequent than previously reported for Central Europe. This is most likely due to improved diagnostic tools for and increased awareness of these diseases. [source]

Desquamative gingivitis: retrospective analysis of disease associations of a large cohort

ORAL DISEASES, Issue 6 2008
JC Leao
Background:, Desquamative gingivitis (DG) is usually a manifestation of immunologically mediated mucocutaneous disorders, although it was previously suggested to be hormonally related. Methods:, One hundred and eighty-seven Caucasian UK residents with clinical features of DG (126 female, median age of 51 years, range 23,93 years) were retrospectively evaluated. Results:, It was established that, in this population, the largest cohort yet reported, oral lichen planus was most common (70.5%) while mucous membrane pemphigoid (14%), pemphigus vulgaris (13%), linear IgA disease (1.6%), dermatomyositis (0.5%) and mixed connective tissue disease (0.5%) were less common. Conclusion:, Oral lichen planus is the main disorder associated with DG. However, DG may be a feature of bullous disease and connective tissue disease. [source]

OC8 The short-term efficacy of osseointegrated implants in patients with non-malignant oral mucosal disease: a case series

ORAL DISEASES, Issue 2006
TA Hodgson
Purpose, The spectrum of patients who may wish or warrant osseointegrated implants is increasing, despite few reports of the impact of non-malignant oral mucosal disease upon implant placement. This report details the implant placement outcomes in three patients with pre-existing oral mucosal disease. Case reports,A: Four implants were placed in the lower anterior region of a 78-year-old female with longstanding mucous membrane pemphigoid (MMP) in 2004. The MMP had resulted in extensive cicatrisation of the upper and lower buccal and labial vestibules. One implant failed to osseointegrate, but was successfully replaced. There have been no other postplacement adverse events, despite the MMP remaining mildly active. B: A 36-years-old male with orofacial granulomatosis characterised by recurrent lip swelling and gingival enlargement, had a single implant placed in the upper canine region in 2001. Although still in situ significant peri-implant alveolar bone loss has occurred and has been stabilised by repeated debridement, local administration of topical minocycline and several courses of systemic metronidazole. C: A 53-years-old female with oral manifestations of diffuse systemic sclerosis and fibrosing alveolitis had four lower anterior mandibular implants placed in 1995 to support an 8 unit bridge. One episode of peri-implant inflammation was controlled with local debridement and topical chlorhexidine mouthrinse. The implants remain satisfactory 11 years postinsertion. Conclusion, The short-term failure of osseointegrated implant integration appears uncommon in patients with non-malignant oral mucosal disease. There remains a need to establish appropriate case selection criteria and monitor outcomes. [source]

Laryngeal mucous membrane pemphigoid: A systematic review and pooled-data analysis

THE LARYNGOSCOPE, Issue 3 2010
MSPH, Thomas S. Higgins MD
Abstract Objectives/Hypothesis: To perform a systematic pooled-data analysis of literature data involving laryngeal mucous membrane pemphigoid (MMP). Study Design: A systematic review and pooled-data analysis. Methods: We conducted a systematic literature search of MEDLINE, EMBASE, Cochrane Central Register of Clinical Trials, Cochrane Database of Systematic Reviews, clinicaltrials.gov, and the National Guideline Clearinghouse databases without language restriction for studies including combinations of relevant terms. All authors independently screened the abstracts of the search results, identified articles eligible for review, and critically appraised the full-text studies. Pooled-data analyses and Kaplan-Meier survival analyses were conducted using SPSS version 16.0 software (SPSS Inc., Chicago, IL). Results: Of the 2,524 citations reviewed, the included articles consisted of 63 case reports and 10 case series reporting on 141 patients with laryngeal MMP. No clinical trials or comparative trials were found. The overall calculated prevalence of laryngeal MMP was 12.2% (95% confidence interval [CI], 11.5-12.9%) of cases of MMP or one in 10 million persons in the general population. Mean age at laryngeal MMP onset was 59.7 years (95% CI, 57.9-61.1 years), and the supraglottis was the most commonly affected site (84.8%, 95% CI, 82.5-87.2%). Distribution among genders was equivalent (P = .655). The presence of antiepiligrin autoantibodies was associated with increased laryngeal involvement (Odds ratio 7.9, 95% CI, 3.9-16.0). The overall 5-year relative survival rate was 92.4% (standard error, 8.4) with a follow-up range of 1 to 221 months. Standard medical therapy alone occasionally improved the condition; however, relapses were frequent, and 10.5% eventually required tracheostomy. Laryngeal surgical interventions seemed to be effective in severe cases. Conclusions: Laryngeal MMP is a rare condition that can be life threatening without proper treatment and frequent follow-up. Laryngoscope, 2010 [source]

Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity

U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases

BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2004
R.M. Vodegel
Summary Background, Epidermolysis bullosa acquisita (EBA) can be differentiated from other subepidermal bullous diseases by sophisticated techniques such as immunoelectron microscopy, salt-split skin antigen mapping, fluorescence overlay antigen mapping, immunoblot and enzyme-linked immunosorbent assay. Objectives, To determine whether the diagnosis can also be made by routine direct immunofluorescence microscopy. Methods, We studied frozen skin biopsies from 157 patients with various subepidermal immunobullous diseases. Results, We found three distinct ,linear' fluorescence patterns at the basement membrane zone: true linear, n-serrated and u-serrated. The true linear pattern, often seen in conjunction with either the n- or the u-serrated pattern, was found in any subepidermal immunobullous disease with nongranular depositions. In bullous pemphigoid, mucous membrane pemphigoid, antiepiligrin cicatricial pemphigoid, p200 pemphigoid and linear IgA disease the n-serrated pattern was found, corresponding with depositions located in hemidesmosomes, lamina lucida or lamina densa. However, in EBA and bullous systemic lupus erythematosus the u-serrated staining pattern was seen, corresponding with the ultralocalization of type VII collagen in the sublamina densa zone. The diagnosis of EBA with IgG or IgA autoantibodies directed against type VII collagen was confirmed by immunoelectron microscopy, salt-split skin antigen mapping, fluorescence overlay antigen mapping or immunoblotting. Conclusions, Using this pattern recognition by direct immunofluorescence microscopy we discovered several cases of EBA which would otherwise have been erroneously diagnosed as a form of pemphigoid or linear IgA disease. [source]

4233: Outcomes of the Boston Keratoprosthesis Type II

ACTA OPHTHALMOLOGICA, Issue 2010
J CHODOSH
Purpose To report the long term outcomes of Boston keratoprosthesis type II implantation in the management of severe ocular surface disease and corneal blindness. Methods A retrospective review of medical records of patients after Boston keratoprosthesis type II implantation at the Massachusetts Eye and Ear Infirmary from January, 1, 2000 through December 31, 2009. Main outcome measures included visual acuity, device retention, and postoperative complications. Results A total of 29 eyes of 26 patients received a Boston keratoprosthesis type II during the study period. Operated patients had corneal blindness due to mucous membrane pemphigoid (51.7%), Stevens Johnson syndrome/toxic epidermal necrolysis (41.4%), or other ocular surface disease (6.9%). Visual acuity after surgery improved to at least 20/200 in 23 eyes (79.3%), and to 20/30 or better in 10 eyes (34.5%). In patients with at least 1 year of follow-up (n=21), visual acuity of 20/200 or better was maintained in 14 eyes (66.7%). Of these 14 eyes, 6 eyes (42.9%) retained visual acuity of 20/200 or better for more than 5 years. Overall, visual acuity of 20/200 or better was maintained for at least 5 years in 6 of 23 eyes (26.0%). Eyes that failed to improve to 20/200 or lost vision during follow-up were found to have end stage glaucoma, previous retinal detachment, or age related macular degeneration. Of the total of 29 eyes, 17 devices (58.6%) were retained without extrusion or replacement during a total follow-up time of 107.9 person-years. Conclusion The Boston keratoprosthesis type II is a viable option for corneal blindness from severe autoimmune ocular surface diseases. Complications require prompt recognition and management to promote retention of the device and preservation of vision. [source]

The Boston keratoprosthesis in autoimmune disease

ACTA OPHTHALMOLOGICA, Issue 2009
J CHODOSH
Purpose Patients with corneal blindness due to mucous membrane pemphigoid and Stevens Johnson syndrome who undergo corneal transplantation carry a poor prognosis for visual recovery. The Boston keratoprosthesis has been demonstrated to provide excellent retention rates and postoperative visual acuity in patients with corneal graft failure, however, poor visual outcomes still occur in patients with underlying autoimmune disease. Methods We reviewed the current literature to determine the results of keratoprosthesis in patients with blinding autoimmune diseases. Results Much of the published literature on keratoprosthesis fails to clearly differentiate outcomes on the basis of the underlying disorder. Based on available evidence, inflammation, retinal detachment, and glaucoma appear to be the most significant complications after keratoprosthesis in autoimmune patients, and a diagnosis of mucous membrane pemphigoid or Stevens Johnson Syndrome appears to be associated with a significantly higher complication rate than other preoperative conditions. Conclusion Patients with autoimmune diseases carry the worst prognosis for success with keratoprosthesis. Improvement in clinical outcomes might be achieved with changes in keratoprosthesis design and material, perioperative therapy, and/or surgical technique. Possible approaches to complications after Boston keratoprosthesis in patients with underlying autoimmune diseases will be discussed. [source]