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]

Our patient presented an even greater challenge because the disease progressed to tracheal and bronchial involvement. A 37-year-old man presented to his primary care physician with a chief concern of a sore throat and oral ulcers. [mdedge.com]

We describe here a new case of this entity presenting as DG in a 4-year-old girl. [medicaljournals.se]

• 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

  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]

  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]

• Microstomia

  There was no recurrence of microstomia for 2 years. [ncbi.nlm.nih.gov]

• Blister

  Mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting mucosal surfaces. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. [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]

  Visualize the most recent topics in cutaneous pathology such as sporothrix and cutaneous t-cell lymphoma as well as classic problems like alopecia and neurofibromatosis, informed by the latest developments in molecular biology and histologic imaging. [euro-libris.ro]

  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]

• 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]

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]

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]

In conclusion, appropriate clinical examination and cooperation among medical specialists are important for the early diagnosis and treatment of patients with recurrent and chronic stomatitis and for their good prognosis. [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]

Sogame R, Ohzono A, Krol RP, Kawakami T, Furumura M, Ohata C, Li X, Hashimoto T 雑誌名 J Invest Dermatol 巻 : 134 ページ : 2283-2287 DOI 関連する報告書 査読あり [雑誌論文] Repression of cathepsin E expression increases the risk of mammary carcinogenesis and links to poor prognosis [kaken.nii.ac.jp]

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]

This review describes the clinical features, epidemiology, etiology, pathogenesis and management for each condition. [ncbi.nlm.nih.gov]

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]

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]

Epidemiological studies showed some triggers to the development of autoimmune dysregulation, e.g. drugs. Furthermore, it has been shown that genetic factors play a role in the pathogenesis of the disease. [clinicaltrials.gov]

IgA to IgG, or both classes of antibodies may develop – can have implications for disease severity and treatment considerations Levels of IgG antibodies to BP180 antigens may be useful in monitoring disease activity and response to therapy Background Epidemiology [arupconsult.com]

[…] 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]

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 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]

[…] and response to therapy Background Epidemiology Incidence – 6-14/million (Baum, 2014) Age – onset ≥60 years; rare in children ≥70 years – incidence significantly increases ≥90 years – relative risk is 300-fold higher than ≤60 years Sex – M:F, equal Pathophysiology [arupconsult.com]

The pathophysiologic mechanisms of ocular cicatricial pemphigoid (OCP), as well as conjunctival fibrosis, are not completely understood. [emedicine.medscape.com]

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

Eyelashes which start to grow inwards need to be removed either by plucking or lasering to destroy the follicles in order to prevent corneal damage. [dermcoll.edu.au]

The severity of the mucous membrane pemphigoid can be assessed using the mucous membrane pemphigoid disease area index (MMPDAI). 1 Treatment of mucous membrane pemphigoid The primary aim of treatment is to stop blister formation, promote healing and prevent [dermnetnz.org]