Paraneoplastic pemphigus

Abstract

Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering skin disease. Clinically, it is characterized by severe mucosal erosions and various cutaneous lesions associated with lymphoproliferative neoplasmas. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family that includes desmoplakin, bullous pemphigoid antigen I (BPAG1), envoplakin and periplakin, and desmogleins 1 and 3. Castleman's tumor, non-Hodgkin's lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP. We have also demonstrated that the autoantibodies reacting to epidermal proteins are directly produced by the cells in the associated tumors. Bronchiolitis obliterans is frequently found in PNP and may cause respiratory failure and death. In our experience, the early detection and removal of the tumor and i.v. administration of immunoglobulin are critical for the treatment of PNP.

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“…Both patterns were seen in our patients. In patients 1 and 3, the results of DIF are negative and thus it is not a necessary diagnostic criterium for PNP [6][7][8]. similar to normal follicular dendritic cells.…”

“…This is the case with Castleman's disease and thymoma [7,46]. Early detection and resection of the tumor are essential for the treatment of the patients with castleman's disease.…”

“…Using i.v. immunoglobulin before, during and after the operation in a total dose of 1-2 g/kg is recommended to block the circulating autoantibodies released from the tumor, which could cause pulmonary damages [7]. However, except in cases associated to Castleman's disease, treatment of neoplasia is not associated with improvement of PNP in general.…”

“…Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. Autoantibody activity in PNP is complex, but usually highly characteristic [2,[6][7][8]. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family that includes desmoplakin, bullous pemphigoid antigen I, envoplakin and periplakin, and desmogleins 1 and 3.…”

Paraneoplastic pemphigus associated with dendritic cell neoplasm and Castleman’s disease: report of a new case and review of the literature

“…Paraneoplastic pemphigus is an uncommon life-threatening mucocutaneous disease, generally presenting in patients with lymphoproliferative disorders [1]. We report on a new case of the disease, which occurred in a woman previously diagnosed with follicular dendritic cell sarcoma and multicentric Castleman's disease hyalinevascular type.…”