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Apoptotic and necrotic cells, absence of acantholytic cells A Tzanck smear may be a rapid test to distinguish toxic epidermal necrolysis from SSSS III. Genodermatoses: Hailey-Hailey disease: Acantholytic cells without direct immunofluorescence positivity Direct immunofluorescence examination should be made for differentiation from pemphigus.
Acantholysis is the loss of intercellular connections, such as desmosomes, resulting in loss of cohesion between keratinocytes, [1] seen in diseases such as pemphigus vulgaris. [2] It is absent in bullous pemphigoid , making it useful for differential diagnosis .
In pemphigus, autoantibodies form against desmoglein, which forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called acantholysis.
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.
Substrates used in indirect immunofluorescence studies for the different pemphigus variants Pemphigus variant Substrate Pemphigus foliaceous: Guinea pig esophagus Pemphigus vulgaris: Monkey esophagus IgA pemphigus: Cultured skin Paraneoplastic pemphigus: Rat bladder
In people with pemphigus, however, the immune system mistakenly attacks the cells in the epidermis, or top layer of the skin, and the mucous membranes. The immune system produces antibodies against proteins in the skin known as desmogleins. These proteins form the glue that keeps skin cells attached and the skin intact.
Endemic pemphigus (endemic pemphigus foliaceus, fogo selvagem) Epidermolysis bullosa acquisita; Grover's disease (benign papular acantholytic dermatosis, persistent acantholytic dermatosis, transient acantholytic dermatosis) Grover's disease; IgA pemphigus; Intraepidermal neutrophilic IgA dermatosis
Mucosal pemphigus vulgaris Pemphigus vegetans. Mucocutaneous pemphigus vulgaris [2] Desmoglein 4: Localized autosomal recessive hypotrichosis Autosomal recessive monilethrix: Desmoplakin: Striate palmoplantar keratoderma Carvajal syndrome Skin fragility–wooly hair syndrome Lethal acantholytic epidermolysis bullosa ARVD Paraneoplastic pemphigus