Pemphigus is an autoimmune blistering disease resulting from loss of normal intercellular attachments in the skin and oral mucosal membrane. Circulating autoantibodies attack the cell surface adhesion molecule desmoglein (the “glue” of the skin) at the desmosomal cell junction in the suprabasal layer of the epidermis, resulting in the destruction of the adhesion molecules (termed acantholysis) and initiating an inflammatory response that causes epidermal blistering. The blisters can become open wounds with a propensity for developing infections. Pemphigus usually occurs between 40 and 60 years of age.
There are four major forms of pemphigus:
- Pemphigus vulgaris – autoantibodies attack desmoglein 3 or desmoglein 1 and 3; is the most common type and affects both skin (especially scalp, face, axilla, groins, trunk, and points of pressure) and mucous membranes; has primary cell adhesion loss in the deeper suprabasal layer. Patients usually present with painful oral mucosal erosions and flaccid blisters, erosions, crusts, and macular erythema in areas of skin involvement. The primary cell adhesion loss is at the deeper suprabasal layer.
- Pemphigus foliaceous – autoantibodies attack desmoglein 1; affects only the skin, in the more superficial epidermis, and is thus milder. Usually occurs on the face and chest.
- IgA pemphigus – autoantibodies attack desmoglein 1; forms grouped clusters with crusts in the epidermis
- Paraneoplastic pemphigus – autoantibodies attack plakins and desmogleins and weaken cell adhesion; inflammatory cells infiltrate the skin, thereby damaging the structure of the adjacent cells; is exclusively associated with underlying malignancies, usually a lymphoproliferative disorder. Because of the malignancy, the mortality rate in this type of pemphigus.,
Medical treatment of all four types consists of high-dose systemic corticosteroids, immune-suppressive agents, and intravenous immune globulin. For patients who have refractory pemphigus vulgaris, a combination of rituximab and intravenous immune globulin has been recommended.
Wound management is conservative with the goal of preventing infection and promoting re-epithelialization in blistered areas where denuding occurs. Flat antimicrobial dressings (e.g., Acticoat Flex, Smith & Nephew, Largo, FL) are useful over open areas, and hydrotherapy is beneficial when the disease is widespread and in the crusty phase. Secondary dressings are required for most areas, and can include surgical or fish-net garments to anchor dressings and allow for easier mobility. Silicone-backed foam dressings without adhesive borders are also recommended for easy removal of loose necrotic tissue without causing painful skin tears.
In summary, pemphigus is a group of autoimmune disorders that causes diffuse and chronic blistering of the epidermis and requires meticulous medical and wound management to prevent infection and to optimize function.
 Kridin K, Patel PM, Jones VA, Cordova A, Amber KT. IgA pemphigus: A systematic review. J Am Acad Dermatol. 2019. Available at https://doi.org/10.1016/j.jaad.2019.11.059. Accessed March 19,2020.
Available at http://www.pemphigus.org/living-with-pemphigus-pemphigoid/all-about-pemphigus-patient-edition. Accessed March 19, 2020.
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