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Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder characterized by the loss of intraepithelial adhesion affecting the skin and mucous membranes, predominantly affects females in their fifth and sixth decades of life. Due to its rare occurrence in children and adolescents, there is often a delay in diagnosis and treatment in this age group. PV should always be considered in the differential diagnosis of oral ulcerative and vesiculobullous lesions in both children and adolescents.

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Rituximab in Childhood and Juvenile Pemphigus Vulgaris: A Systematic Review.

Cureus

April 2024

Oral Medicine and Radiology, Faculty of Dentistry, Jamia Millia Islamia, New Delhi, IND.

Article Synopsis
  • - Pemphigus vulgaris (PV) is a rare autoimmune skin disorder that can affect both genders but is more common in females in their 50s and 60s; about 1.4 to 3.7% of cases occur in children and adolescents, leading to delayed diagnosis.
  • - A systematic review was conducted on various studies to assess the effectiveness of rituximab (RTX) in treating childhood and juvenile PV, with 18 studies involving 46 patients analyzed for treatment outcomes.
  • - Most pediatric patients treated with RTX achieved remission, experienced few relapses, and had only minor side effects; however, the limited evidence and lack of controlled studies call for more research to create a standardized treatment approach.
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Pemphigus foliaceus is an autoimmune blistering disease of the skin that is not frequently associated with mucous membrane involvement. It is characterized by immunoglobulin G (IgG) antibodies against desmoglein-1, a component of epidermal intercellular adhesion, in the granular layer of the epidermis. Pemphigus foliaceus consists of scattered, arcuate, crusted erythematous lesions often in a seborrheic distribution that may progress to diffuse skin involvement and exfoliative erythroderma.

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