![]() Dyskeratosis with suprabasal acantholysis is a distinctive feature of PNP. Histologic features of paraneoplastic pemphigus (PNP) include epidermal acantholysis and clefting, necrotic keratinocytes, vacuolization of the basal layer, and lymphocyte exocytosis. In contrast, HLA-DR4 and HLA-DR1-14 increase susceptibility to pemphigus vulgaris and pemphigus foliaceus, but have not shown an association with the development of PNP. These alleles are more common in Caucasian and Chinese populations, respectively. Recently, researchers have identified increased susceptibility to paraneoplastic pemphigus development in patients with HLA Class II Drb1*03 allele and HLA-Cw*14. Paraneoplastic pemphigus in children and adolescents is often the presenting sign of Castleman disease. Non-hematological neoplasms account for 16% of cases. In adults, non-Hodgkin lymphoma is the most frequent underlying neoplasm (38.6%), followed by chronic lymphocytic leukemia (18.4%), and Castleman disease (18.4%). However, other neoplasms are associated with PNP development, such as sarcomas, particularly follicular dendritic cell sarcomas, thymomas, squamous cell carcinomas of the skin, tongue, vagina, and carcinomas of the lung, stomach, and colon. Approximately two-thirds of PNP cases are due to non-Hodgkin lymphoma and chronic lymphocytic lymphoma. Lymphoproliferative neoplasms are the most commonly identified underlying diseases in paraneoplastic pemphigus development, accounting for up to 84% of cases. The term “paraneoplastic autoimmune multiorgan syndrome” (PAMS) has since been coined to account for the variable non-bullous cutaneous manifestation and additional systemic findings, such as bronchiolitis obliterans. ![]() It was first identified in 1990 by Anhalt, who proposed the initial set of diagnostic criteria. Adults ages 45 to 70 years are most commonly affected, though children can develop PNP. Patients often require intensive care and should be managed, similar to burn patients. ![]() Extensive epidermal loss can result in extreme dehydration, protein depletion, and an increased risk of infection. However, shared by all patients is the presence of widespread, and often severe, mucosal lesions that can be the earliest presenting symptom of the disease. A variety of lesions with differing morphologies, ranging from flaccid blisters to widespread lichenoid eruptions, have been seen in paraneoplastic pemphigus patients. Paraneoplastic pemphigus typically presents with painful mucosal erosions and dusky patches on the skin that later desquamate. Researchers have identified three significant forms of pemphigus: pemphigus foliaceus, pemphigus vulgaris (most common type), and paraneoplastic pemphigus. ![]() Pemphigus comprises a group of IgG-mediated autoimmune bullous diseases in which autoantibodies react with desmosomes, the cell-to-cell adhesion structures between keratinocytes, resulting in painful skin erosions and blisters. Paraneoplastic pemphigus (PNP) is a rare, autoimmune-mediated mucocutaneous disease that is almost always associated with an underlying neoplasm.
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