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The diagnostic utility of immunohistochemistry on paraffin-embedded sections in bullous disorders is useful when frozen tissue is not available. In pemphigus vulgaris and pemphigus foliaceus, an intercellular lace-like staining pattern of IgG4 on lesional tissue by immunohistochemistry has been described, with a comparable sensitivity and specificity to direct immunofluorescence on perilesional tissue. This study aimed to evaluate the staining pattern of IgG4 in non-immunobullous disorders to highlight the potential pitfalls when using this stain.

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Background/objectives: Pemphigus comprises a diverse group of disorders within the autoimmune bullous dermatoses (AIBDs) spectrum. Among these, pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the most commonly encountered variants. Despite its rarity, this condition can pose a life-threatening risk.

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This case report highlights the efficacy of rituximab (RTX), a monoclonal antibody that targets B-lymphocytes, in the treatment of severe pemphigus vulgaris (PV) that was unresponsive to multiple conventional therapies. A 44-year-old female presented with mucocutaneous lesions that had been progressing for 12 months, with a Pemphigus Disease and Area Index (PDAI) total activity score of 66, indicating severe disease. The patient received two infusions of RTX two weeks apart, without complications.

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Pemphigus foliaceus (PF) is one of the most common autoimmune skin diseases of dogs and cats and is characterized by the development of pustules, crusts, erosions, scales, and alopecia. Albeit poorly understood, the pathophysiology of canine and feline PF appears to involve immune dysregulation and immunoglobulin G autoantibodies that are directed against the keratinocyte cell surface. At present, the management of canine and feline PF relies on the long-term to lifelong prescription of immunosuppressive medications.

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Pemphigus vulgaris (PV) is a rare autoimmune disorder characterized by mucocutaneous blistering due to autoantibodies targeting desmoglein proteins, leading to acantholysis. This case report presents a 60-year-old Hispanic male patient with a history of hypertension who developed PV, initially presenting with pruritic scalp lesions that progressively spread to the nares, mouth, chest, neck, and inguinal region. Despite initial management with topical treatments, the lesions persisted, prompting hospitalization.

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