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Paediatric Immunobullous Disorders constitute a diverse group of rare autoimmune blistering diseases affecting the skin and mucous membranes in children, presenting a complex and challenging spectrum of clinical manifestations. Characterized by the formation of blisters due to the body's immune system mistakenly attacking proteins within the skin, these disorders encompass a variety of conditions, with Pemphigus and Pemphigoid being prominent examples. Pemphigus vulgaris and Pemphigus foliaceus, among others, involve the production of autoantibodies targeting desmogleins, crucial components of intercellular adhesion in the epidermis, leading to intraepidermal blister formation. Conversely, Pemphigoid disorders, such as Bullous Pemphigoid and Epidermolysis Bullosa Acquisita, typically feature subepidermal blistering, triggered by autoantibodies against basement membrane zone proteins like BP180 and BP230. These conditions often present with a range of cutaneous and mucosal lesions, creating diagnostic dilemmas for clinicians. The intricate interplay between genetic predisposition, environmental factors, and dysregulated immune responses contributes to the pathogenesis, further complicating the understanding of these disorders in the paediatric population. Timely and accurate diagnosis is critical for effective management, involving a multidisciplinary approach that integrates clinical evaluation, histopathological examination, immunofluorescence studies, and serological testing. Treatment strategies include systemic corticosteroids, immunosuppressive agents, and emerging biologic therapies, aiming to modulate the immune response and control disease progression. The rarity of these disorders in children necessitates specialized expertise and collaboration between dermatologists, paediatricians, and immunologists to ensure optimal patient care, highlighting the importance of ongoing research efforts to enhance our understanding of the underlying mechanisms and improve therapeutic options for this vulnerable patient population.
