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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 and occurs when antibodies attack desmoglein 3 .
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.
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. [2] Mucosal involvement is absent even with widespread disease. [3]
The histological picture involves thinner, weaker attachments of the skin lesion itself to the normal skin – resulting in easier dislodgement. The formation of new blisters upon slight pressure (direct Nikolsky) and shearing of the skin due to rubbing (indirect Nikolsky) is a sign of pemphigus vulgaris, albeit not a 100% reliable diagnosis. [8]
Bullous pemphigoid (a type of pemphigoid) is an autoimmune pruritic skin disease that typically occurs in people aged over 60, that may involve the formation of blisters in the space between the epidermal and dermal skin layers.
Other forms of pemphigus present with oral blisters, which are often the first symptoms of the disease. Pemphigus erythematosus, however, does not produce oral ulcers, or any other mucosal lesions. [2] Pemphigus erythematosus targets desmoglein 1, which is primarily found in the skin. Desmoglein 3 is present in higher numbers in the mucosa.
While the presence of lesions is the denominator among patients with PNP, the characteristics of the lesions differ. The five clinical presentations of lesions associated with PNP include: "Pemphigus-like": Flaccid blister (discrete), crusts over the raw exuding skin lesions "Pemphigoid-like": Tense blister(s) on brick red erythema
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 .
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