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Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus.Pemphigus was derived from the Greek word pemphix, meaning blister. [1] It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other.
If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In contrast, in Pemphigus, the epithelium tends to disintegrate rather than form a bulla. Nikolsky's sign is present in pemphigus and mucous membrane pemphigoid, but not in bullous pemphigoid.
The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. Skin lesions caused by pemphigus can lead to fatal infections, so treatment is extremely important. Pemphigus vulgaris (PV - ICD-10 L10.0) is the most common form of the disorder ...
Oral mucosa is the most common site being affected in mucous membrane pemphigoid. For the mild oral mucosa lesion, high potency topical steroids such as 0.05% Clobetasol propionate is used. Patients are instructed to apply the ointment or gel 2-3 times a day after drying the oral mucosa to enhance the adherence of mediation to oral mucosa.
A 2010 (updated in 2023) meta-analysis of 14 randomized controlled trials showed that oral steroids and potent topical steroids are effective treatments, although their use may be limited by side-effects, while lower doses of topical steroids are safe and effective for treatment of moderate bullous pemphigoid. [10]
The term "full width gingivitis" usually refers to the oral lesions of orofacial granulomatosis however. [4] The color is another dissimilarity between typical marginal gingivitis and desquamative gingivitis, in the latter it is dusky red. [3] Plasma cell gingivitis is another form of gingivitis which affects both the attached and free gingiva. [1]
Mucosal pemphigus vulgaris: IgG: Desmoglein 3: 130 Mucocutaneous pemphigus vulgaris: IgG: Desmoglein 3 Desmoglein 1: 130 160 Pemphigus foliaceus: IgG: Desmoglein 1: 160 Paraneoplastic pemphigus: IgG: Desmoglein 3 Desmoglein 1 BPAG1 [nb 1] Plectin Desmoplakin I [nb 2] Desmoplakin II Envoplakin Periplakin Alpha-2-macroglobulin-like-1: 130 160 230 ...
In more severe treatment it is treated similarly to pemphigus vulgaris. Pemphigus foliaceus is an autoimmune blistering disease of the skin. [ 1 ] Pemphigus foliaceus causes a characteristic inflammatory attack at the subcorneal layer of epidermis , which results in skin lesions that are scaly or crusted erosions with an erythematous (red) base ...