Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour

Abstract

A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.

Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature

“…Histologically these tumors can be classified into 3 types: a) Hyaline-vascular type (80–90%), b) plasma cell type (10–20 %), and c) intermediate types [4]. The most common location of the tumor is the mediastinum (60 – 70 %).…”

Treatment of castleman’s disease

“…Hematologic-Anemia of chronic disease; Pure red cell aplasia [70]; Autoimmune hemolytic anemia [71], Autoimmune thrombocytopenia [72]; Acquired hemophilia [69]; Osteosclerotic myeloma (POEMS syndrome) [2,73]71 Oncologic-Non-Hodgkin's lymphoma [22•]; Hodgkin's disease [22]; Follicular dendritic cell sarcoma [23]; Kaposi's sarcoma [74]; Mesenchymal spindle-cell neoplasm [75] Dermatologic-Pemphigus [8,9]; Bechet's disease [10] Renal-Membroproliferative glomerulonephritis [69]; Fibrillary glomerulonephritis [19]; Secondary (SAA) amyloidosis Neurologic-Demyelinating polyneuropathy (POEMS syndrome) [2,73] Pulmonary-Bronchiolitis obliterans [15][16][17]; Lymphoid interstitial pneumonia [14]; Pulmonary fibrosis [16] Rheumatologic-Systemic lupus erythematosus [76] Endocrine-Growth failure, delayed puberty [77]; Adrenal insufficiency [78] POEMS-peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes.…”

“…• Castleman's disease is associated with a number of autoimmune phenomena ( Table 2) including paraneoplastic mucocutaneous disease (paraneoplastic pemphigus [8,9] and Bechet's disease [10], secondary amyloidosis [11][12][13], lymphoid interstitial pneumonitis [14], bronchiolitis obliterans [15][16][17], and a multitude of renal disorders [18][19][20]. Though CD can occur in the context of POEMS syndrome, it can also exist without the hallmark features of POEMS but with a number of other associated "paraneoplastic" symptoms.…”

Castleman’s disease imitating adrenal mass in the retroperitoneal area

“…Plasma-cell variant type is the most rare type and occurs with a rate of 10% to 20%, the third one is intermediate type. 4 While hyaline vascular type is generally seen in unicentric cases, plasma-cell variant type is usually seen in multicentric cases. Clinically the most common case is unicentric hyaline vascular type.…”