Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus

Abstract

Paraneoplastic pemphigus (PNP) is a rare, life-threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus-like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological-related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5-148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2-year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.

Untitled

“…The Nonhematologic neoplasms associated with PNP include follicular dendritic cell sarcoma [5,42,46,47,72,84,85], inflammatory myofibroblastic tumor [86], leiomyosarcoma [87], liposarcoma [88], malignant nerve sheath tumor [89], inflammatory fibrosarcoma [90], reticulum cell sarcoma [91], and malignant melanoma [92], squamous cell carcinoma of the vagina [93], and adenocarcinoma of the colon [55], prostate [94,95], pancreas [88,93], breast [36], and lung [96,97] (Table 1). Follicular Dendritic Cell Sarcoma (FDCS) is a rare tumor associated with paraneoplastic pemphigus.…”

“…More than 260 case reports of PNP have been documented since that time [1,4,5] and about 20 of the reported patients were children or adolescents. Yet PNP may be more common than is generally believed given that serum detection of autoantibodies is only performed by a handful of laboratories.…”

Peri-anal Paraneoplastic Pemphigus Heralding the Relapse of Follicular Lymphoma and Its Successful Management by Rituximab: A Short Correspondence

“…Dear Editor, Paraneoplastic pemphigus (PNP) is a rare autoimmune muco-cutaneous blistering disorder seen most commonly in the setting of an underlying lymphoreticular malignancy [1]. Given its resemblance to host of dermatological conditions, PNP is often misdiagnosed and culminate in death of patients.…”

“…Although virtually any mucosa may be involved, painful oral erosions are the earliest and a hallmark feature and its absence argues against a diagnosis of PNP [4]. Polymorphous cutaneous lesions follows mucosal involvement (although isolated mucosal disease has been described) and may resemble PV, Pemphigus foliaceus (PF), Bullous Pemphigoid (BP), lichen planus (LP), Steven-Johnson's syndrome (SJS), Graft-versus-host disease (GVHD) and herpes stomatitis [1,5]. Bronchiolitis obliterans (20 %) is the most dreaded complication of PNP due to sloughing of respiratory epithelium from immune complex deposition in the basement membrane and imparts a poor prognosis [6].…”

“…Bronchiolitis obliterans (20 %) is the most dreaded complication of PNP due to sloughing of respiratory epithelium from immune complex deposition in the basement membrane and imparts a poor prognosis [6]. PNP may precede (1/3rd) or develop following the diagnosis (2/ 3rd) of the underlying neoplasm [1]. Haematological neoplasms account for the majority of cases (84 %) including NHL (38 %), Chronic lymphocytic leukemia (CLL, 18 %), castleman disease (18 %), thymoma (5.5 %), waldenstrom's macroglobulinemia (1.2 %) and Hodgkin's lymphoma (0.6 %).…”