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]

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]

• Malaise

  Mycobacterium tuberculosis Difference between infection and active disease of TB Infection - healthy person can be infected and keep it under control Disease - person can spread the disease Symptoms of TB (had this a boards flashcard question) fever, malaise [quizlet.com]

• 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]

• 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]

• Dry Eyes

  This result emphasizes the role of evaporative dry-eye condition in patients with ocular MMP. Tear osmolarity may be considered as a useful test in the diagnostic assessment of dry eye associated with MMP and for targeting therapeutic decisions. [ncbi.nlm.nih.gov]

  Because dry eye syndrome is often associated with MMP, dry eye therapy might be beneficial. [hopkinsmedicine.org]

  Eyes This may start with conjunctivitis and grittiness of the eye. Dry eyes may result from lacrimal gland involvement. [patient.info]

  The scarring of the conjunctiva can also results in dry eye and disruption of the corneal epithelium Eyelids may also turn in at the lid margin resulting from the scarring process of the conjunctiva. [pemfriends.co.uk]

  It may be confused with other eye conditions, including ocular rosacea and dry eye syndrome, so it is important for these patients to have ongoing follow up with an ophthalmologist experienced in anterior ocular surface disease, ideally. [dermcoll.edu.au]

• Conjunctival Hyperemia

  Patients usually show decreased conjunctival hyperemia within weeks of starting treatment. Immunosuppressive treatment is usually continued 2 to 3 years into remission. [healio.com]

• 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]

  Intense, often generalised, pruritus accompanies skin involvement (tense blisters or erosions on normal or erythematous skin). Head and neck involvement is common. [patient.info]

• Dermatitis

  cicatricial pemphigoid (diagnosis), mucosynechial atrophic bullous dermatitis (diagnosis), cicatricial pemphigoid, Pemphigoid, Benign Mucous Membrane [Disease/Finding], Benign Mucosal Pemphigoids, Mucosal Pemphigoids, Benign, Pemphigoid, Benign Mucosal [fpnotebook.com]

  It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement Applies To Cicatricial pemphigoid Mucosynechial atrophic bullous dermatitis [icd9data.com]

  […] herpetiformis L13.1 Subcorneal pustular dermatitis Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes. [icd10data.com]

  Benign mucous membrane pemphigoid with no eye involvement, Benign mucous membrane pemphigoid without mention of ocular involvement, Benign mucous membrane pemphigoid without ocular involvement, Cicatricial pemphigoid, Mucosynechia atrophic bullous dermatitis [dermis.net]

• 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]

• Oral Blisters

  The blisters persisted for weeks before rupturing spontaneously. Four years later, she developed tense oral blisters, resulting in painful persistent erosions. [ncbi.nlm.nih.gov]

  Figure 1 (a) A man with HIV presenting oral blister and erosions; (b) symblepharon formation on eye. [casesjournal.biomedcentral.com]

  Blisters in the oral cavity occur in up to 30% of patients, and they usually occur when the cutaneous disease is extensive. [clevelandclinicmeded.com]

• Suggestibility

  The clinical picture and the histopathology of a vulvar biopsy were suggestive of erosive lichen planus. Direct immunofluorescence, however, revealed findings diagnostic of mucous membrane pemphigoid. [ncbi.nlm.nih.gov]

• 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]

• Irritability

  A 64-year-old man presented with redness and irritation of both eyes. The patient also had blistering lesions on the roof of his mouth, trunk, and buttocks as well as bilateral conjunctival ulceration and symblepharon formation. [ncbi.nlm.nih.gov]

  Early symptoms are hyperemia and irritation; progression leads to eyelid and corneal damage and sometimes blindness. Diagnosis is sometimes confirmed by biopsy. Treatment often requires systemic immunosuppression. [merckmanuals.com]

  In the early stages, people with OCP generally experience chronic or relapsing conjunctivitis that is often characterized by tearing, irritation, burning, and/or mucus drainage. [autoimmune.org]

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]

Patients usually show decreased conjunctival hyperemia within weeks of starting treatment. Immunosuppressive treatment is usually continued 2 to 3 years into remission. [healio.com]

The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other. [ncbi.nlm.nih.gov]

The condition usually begins in late adulthood (e.g. 50’s or 60’s), affects more women than men, and has a variable prognosis. Scarring of the affected mucosa of the eye may lead to blindness and tends to be the most feared complication. [autoimmune.org]

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]

The clinical presentation, etiology, and management options for mucous membrane pemphigoid are also discussed. [ncbi.nlm.nih.gov]

[…] 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 Usually elderly, F / M = 2 : 1 Etiology [pathologyoutlines.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]

[…] dc.subject Blister dc.subject +etiology dc.subject Eye dc.subject Hemidesmosomes dc.subject Mouth dc.subject Nasal Cavity dc.subject Pemphigoid, Benign Mucous Membrane dc.subject +complications dc.subject +diagnosis dc.subject +drug therapy dc.subject +epidemiology [helda.helsinki.fi]

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]

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]

[…] 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]

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

[…] 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]

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]

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

The cornea (the clear layer in front of the iris and pupil) can become cloudy, preventing light from reaching the retina and decreasing vision. [merckmanuals.com]