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Mucous Membrane Pemphigoid

Benign Mucous Membrane Pemphigoid


Presentation

  • A biopsy is required in cases of cicatrising conjunctivitis so that even atypical cases such as the one presented herein can be appropriately managed.[ncbi.nlm.nih.gov]
Limited Mobility
  • When lesions involve the frenulum, ankyloglossia or limited mobility of the tongue may result.[jofs.in]
Sore Throat
  • She presented with a severe sore throat, odynophagia, dysphonia, dyspnoea, ocular irritation and weight loss over the last 3 months.[ncbi.nlm.nih.gov]
  • A 37-year-old man presented to his primary care physician with a chief concern of a sore throat and oral ulcers. The patient was treated with a course of antibiotics followed by a nystatin oral solution.[mdedge.com]
  • Three months post transplant, he presented with a 3-week history of recurrent febrile episodes, sore throat, wt loss and a productive cough with copious mucopurulent sputum. In addition, he developed conjunctivitis with discharge from his eyes.[nature.com]
  • throat, hoarseness Scarring, stenosis of airway Breathing difficulty Esophagus In 5%–15% of patients Dysphagia, odynophagia, esophageal reflux Stricture formation Disability in eating Death Anogenital area In 20% of patients Erosion and ulceration Bleeding[pocketdentistry.com]
  • Clinical Manifestations General manifestations Fever, which may be high, headache, sore throat, anorexia and dysphagia. Skin manifestations Skin lesions may appear in different forms mainly: Exanthematous reaction. Urticarial lesions.[drmhijazy.com]
Pharyngitis
  • Herein we report what we believe to be the youngest patient diagnosed with anti-laminin-332 MMP, a 9-year-old girl with disease affecting only the oral, pharyngeal, and laryngeal mucosa, with no skin involvement.[ncbi.nlm.nih.gov]
  • Al-Shihabi BM, Jackson JM (1982) Dysphagia due to pharyngeal and oesophageal lichen planus. J Laryngol Otol 96:567–571 PubMed CrossRef Google Scholar 4.[link.springer.com]
Respiratory Distress
  • We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress.[ncbi.nlm.nih.gov]
  • The patient was started on methotrexate and oral prednisone by the rheumatology department, but after 1 week, he presented in respiratory distress and was taken for an emergency tracheostomy.[mdedge.com]
Stridor
  • 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.[ncbi.nlm.nih.gov]
Oral Ulcers
  • A 37-year-old man presented to his primary care physician with a chief concern of a sore throat and oral ulcers. The patient was treated with a course of antibiotics followed by a nystatin oral solution.[mdedge.com]
  • Oral ulceration can present a diagnostic problem. [ 6 ] The main sites and types of involvement are: Mouth Painful and recurrent erosions present anywhere in the mouth, starting as blisters.[patient.info]
Gingival Ulceration
  • Oral manifestations of pemphigoid include desquamative gingivitis, ulcers, erythematous patches, erosions, vesicles and bullae located on the attached gingiva, palate, buccal mucosa, labial mucosa, and tongue.[ncbi.nlm.nih.gov]
  • Mucous membrane pemphigoid.Note the gingival ulceration and bleeding. Mucous membrane pemphigoid.Low-power photomicrograph showing separation of the epithelium and connective tissue, resulting in a subepithelial blister.[dentistry.uiowa.edu]
Microstomia
Visual Impairment
  • Patients with younger onset ( ocular cicatricial pemphigoid were found to have disease evolution similar to that of an older group ( 70 years) but were visually impaired earlier in life.[journals.lww.com]
Blister
  • Clinical examination revealed extensive mucosal lesions as well as generalized blisters and erosions that healed with scar formation. The disease was intractable to treatment with systemic steroids.[ncbi.nlm.nih.gov]
  • Clinical Information A chronic autoimmune disorder characterized by the development of blisters and ulcers in mucous membranes.[icd9data.com]
Alopecia
  • Her scalp had bullae of similar morphology, crusted erosions, and cicatricial alopecia at the site of previous lesions involving a large area of scalp (Figure 2). Examination of the other mucosae did not reveal any abnormality.[ncbi.nlm.nih.gov]
  • […] blistering Red and blistered mouth, gums, palate Tender gums, may bleed easily May affect oesophagus and larynx Difficulty swallowing Conjunctivitis, one eye at a time Skin may be involved with blisters or plaques that bleed or itch Hyperpigmentation Alopecia[allthingsvagina.com]
  • Alopecia follows scalp involvement. [ 8 ] Genitalia and perianal mucosae Typically painful with pruritus and scarring. Involves the clitoris, labia, glans and shaft of penis.[patient.info]
  • […] fluids to erosions on erythematous and/or urticarial base, but skin can appear normal Mucosal involvement occurs in 10-40% of cases Pruritus is common Involvement of ocular conjunctivae – blindness due to scarring (ocular cicatricial pemphigoid) Scarring alopecia[arupconsult.com]
  • Presentations and Potential Complications Skin In 25%–30% of patients Localized erythematous plaque near affected mucosal surfaces, generalized bullous eruption particularly on the head and upper body, or vegetating lesions occasionally Scarring, cicatricial alopecia[pocketdentistry.com]
Eczema
  • , mucosynechial, atrophic, cicatricial; pemphigoid, eczema; bullous, mucosynechial, atrophic, pemphigoid; cicatricial, scar; pemphigoid, Mucosynechial atrophic bullous dermatitis Dutch cicatriciaal pemfigoïd, benigne slijmvliezenpemfigoïd, bulleus; eczeem[fpnotebook.com]
  • Tabs Content Clinical Overview Diagnosis Indications for Testing Chronic blistering skin lesions not attributable to more common skin disorder Unexplained recurring pruritus, erythroderma, eczema, urticaria, desquamative gingivitis, or mucositis (including[arupconsult.com]
  • In most patients, spontaneous regression of the disease will be seen 6 months to 2 years after onset. (27 Sep 1997) lichen agrius Acute papular eczema of severe type.[kmle.co.kr]
Epistaxis
  • Ocular involvement can lead to blindness because of inflammation and scarring involving the: Lens Cornea [ 7 ] Iris Lobe Some patients with CP have only eye involvement. [ 1 ] Nose This may manifest first with epistaxis.[patient.info]

Workup

  • RESULTS: Thirty-six of 52 patients (69%) who underwent only 1 biopsy at the first workup were positive. In 13 cases, the initial single biopsy was negative, and later biopsies were positive.[ncbi.nlm.nih.gov]
  • An ophthalmologic workup was negative for ocular mucosal disease involvement. Direct immunofluorescence findings showed IgG and C3 deposits at the basement membrane zone.[guwsmedical.info]

Treatment

  • Obvious progression was observed in 2 patients who had refused systemic treatment at an early stage. 10 eyes showed progression while on systemic treatment.[ncbi.nlm.nih.gov]

Prognosis

  • The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other.[ncbi.nlm.nih.gov]
  • Treatment, Complications, and Prognosis Depending on the severity of the disease process, topical or systemic corticosteroids may be used.[guwsmedical.info]
  • Prognosis We agree with other authors that severe keratoconjunctivitis sicca and advanced ocular cicatricial disease and especially active inflammation have a poor prognosis.[healio.com]
  • Complications Severe sight impairment Oesophageal strictures Airway obstruction Prognosis The disease is chronic, progressive and difficult to treat. There may be exacerbations and remissions with waning disease activity.[patient.info]

Etiology

  • The clinical presentation, etiology, and management options for mucous membrane pemphigoid are also discussed.[ncbi.nlm.nih.gov]
  • LOSS OF SENSE OF TASTE Etiology Lingual nerve trauma. Xerostomia Drugs such as penicillamine. Psychotic disorders.[drmhijazy.com]

Epidemiology

  • This review describes the clinical features, epidemiology, etiology, pathogenesis and management for each condition.[ncbi.nlm.nih.gov]
  • Epidemiology CP is rare although precise figures for incidence are unknown. There appears to be an association between human leukocyte antigens and CP. [ 4 ] The reported incidence in European countries is about 1 per million per year.[patient.info]
  • Epidemiology Frequency The incidence of mucous membrane pemphigoid (MMP) has been estimated to be 2 cases per million in studies performed in France and Germany. [2, 3, 4] Pure ocular mucous membrane pemphigoid occurs in less than 1 case per million in[emedicine.medscape.com]
  • According to an epidemiologic study by Thorne and colleagues, the most common presumed causes of pseudopemphigoid include glaucoma medications, rosacea blepharoconjunctivitis, atopic keratoconjunctivitis and conjunctival lichen planus.[healio.com]
  • Definition / general Rare autoimmune bullous disease with mucosal lesions, including conjunctiva; skin involvement in 30% See also ocular cicatricial pemphigoid Terminology Also called benign mucous membrane pemphigoid Cicatrix means scar Epidemiology[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • […] role of Hsp90 as drug target in preclinical in vivo murine models of autoimmune diseases such as subepidermal bullous and fibrotic autoimmune disorders comprising epidermolysis bullosa acquisita and systemic sclerosis, respectively, which exhibit some pathophysiological[ncbi.nlm.nih.gov]
  • Pathophysiology The exact cause of MMP is unknown. It is an autoimmune disorder (i.e. it occurs when the body produces autoantibodies against healthy ‘self’ tissue).[news-medical.net]
  • There is variety in the way CP presents clinically and some variety in the pathophysiological detail between patients. [ 2, 3 ] It can be difficult to distinguish from other autoimmune blistering diseases which may have mucosal involvement (for example[patient.info]
  • Pathophysiology As in other autoimmune diseases, environmental factors combined with genetic susceptibility lead to development of autoantibodies.[emedicine.medscape.com]
  • […] or submucosal) blisters with or without inflammation Eosinophils are characteristic, but a cell-poor variant exists IgG and complement are found at the BMZ in perilesional tissue IgE BMZ antibodies are difficult to detect but are likely important in pathophysiology[arupconsult.com]

Prevention

  • Early diagnosis and treatment could prevent severe consequences of the disease in their full-blown forms.[ncbi.nlm.nih.gov]

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