cicatricial pemphigoid

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Related to cicatricial pemphigoid: Ocular Cicatricial Pemphigoid

cicatricial pemphigoid

Etymology: L, cicatrix, scar; Gk, pemphix, blister or bubble + eidos, form

a benign, chronic, usually bilateral, subepidermal blistering disease chiefly involving the mucous membranes, especially those of the mouth and eye. It heals by scarring and may lead to slow shrinkage of the affected tissues, and to blindness if untreated. Also called benign mucosal pemphigoid.

Cicatricial pemphigoid

cic·a·tri·cial pem·phi·goid

(sik'ă-trish'ăl pem'fi-goyd)

A chronic disease that produces adhesions and progressive cicatrization and shrinkage of the conjunctival, oral, and vaginal mucous membranes.

pemphigoid, cicatricial

A rare, idiopathic, chronic systemic disease, most commonly of the elderly and characterized by recurrent blisters and bullae of the skin and mucous membranes, with subsequent scarring and shrinkage. The disease may affect only the conjunctiva (ocular pemphigoid). In this case, the clinical picture is a conjunctivitis with hyperaemia, mucous discharge and small vesicles, which on bursting result in ulceration, pseudomembranes, conjunctival subepithelial fibrosis and conjunctival shrinkage. The disease may give rise to adhesion between the palpebral and bulbar conjunctiva (symblepharon), ankyloblepharon, xerophthalmia, keratoconjunctivitis sicca, entropion, trichiasis and dry eye with corneal ulcer. There is pain or irritation and blurred vision. Treatment includes corticosteroids, surgery for entropion and trichiasis, and keratoprosthesis if vision is affected. Syn. benign mucous pemphigoid. See pseudomembranous conjunctivitis; Stevens-Johnson syndrome; ocular ferning test.

be·nign mu·cous mem·brane pem·phi·goid

(bĕ-nin myūkŭs membrān pemfi-goyd)

Blistering disease chiefly involving the subepidermal mucous membranes of mouth and eye (ocular pemphigus); usually chronic, benign, and typically bilateral. Scarring can occur and may lead to progressive shrinkage and tightening of the affected mucous membranes and connective tissues.
Synonym(s): cicatricial pemphigoid, ocular cicatricial pemphigoid.

cicatricial pemphigoid (sikətrishəl pem´figoid´),

n See pemphigoid, benign mucous membrane.

pemphigoid

1. resembling pemphigus.

2. a group of skin disorders similar to but clearly distinguishable from pemphigus.

bullous pemphigoid

an autoimmune disease of skin and oral mucosa with vesicles, bullae and ulcerations; occurs rarely in dogs and humans.

cicatricial pemphigoid

a form that involves the oral and ocular mucosa.

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References in periodicals archive ?

Wieme N, Lambert J, Moerman M, Geerts ML, Temmerman L, Naeyaert JM: Epidermolysis bullosa acquisita with combined features of bullous pemphigoid and cicatricial pemphigoid.

The clinical diagnosis of autoimmune bullous diseases/Otoimmun bulloz hastaliklarin klinik tanisi

The main differential diagnosis of cicatricial pemphigoid includes pemphigus, erosive lichen planus, and linear IgA disease, among other rare autoimmune diseases.

Clinical, historic, and therapeutic features of cicatricial pemphigoid.

Oral cicatricial pemphigoid

Electron microscopy of cicatricial pemphigoid lesions typically shows that the bullae are located between the basal cell membrane and the basal lamina.

In this article, we describe five cases of clinical cicatricial pemphigoid that featured otorhinolaryngologic manifestations-that is, all patients exhibited involvement of the oral, nasal, and ocular mucosae.

Findings on analysis of biopsy specimens of the skin and oral cavity lesions were consistent with cicatricial pemphigoid.

A biopsy analysis of one of the skin lesions confirmed the diagnosis of cicatricial pemphigoid.

Findings on biopsy of the jugal and nasal mucosae were compatible with cicatricial pemphigoid.

Biopsy samples of the nasal and oral mucosae were consistent with cicatricial pemphigoid.

7) In 1857, Cooper was the first to adequately describe cicatricial pemphigoid of the eye, which he called conjunctival pemphi goid.

Cicatricial pemphigoid is a systemic disease with both ocular and extraocular manifestations.

The pathogenesis of cicatricial pemphigoid involves a defect in immunoregulation and a resultant production of autoantibodies that affect the basal membrane.