A Case Report of Paraneoplastic Pemphigus Associated with Esophageal Squamous Cell Carcinoma
A 68-year-old male presented to the emergency room (ER) with progressive bullous and erosive skin lesions involving the whole body. Two weeks earlier, the patient had visited the ambulatory clinic with a painful oral ulcer. At that time, bilateral enlarged supraclavicular lymph nodes were observed on physical examination, and fine needle aspiration of the lymph node revealed metastatic squamous cell carcinoma. The patient was diagnosed with diabetes 20 years ago and had been treated with diabetic nephropathy since eight years ago. He denied any recent changes in medication.\nAt presentation to the ER, he showed a blood pressure of 93/64 mmHg, a heart rate of 91/min, and a respiration rate of 20/min. Flaccid blisters, exfoliated skin, and crusted erosions involving eyelids, buccal mucosa, tongue, trunk, back, and extremities were observed (). Laboratory tests showed anemia and thrombocytopenia: white blood count 5,170/mm3, hemoglobin 7.7 mg/dL, and platelets 426,000/mm3. He showed azotemia with blood urea nitrogen 52.3 mg/dL (normal, <22 mg/dL) and creatinine 2.98 mg/dL (normal, <1.3 mg/dL), which was due to already known diabetic nephropathy. Findings on computed tomography scan of the chest and positron emission tomography scans revealed multiple cervical and mediastinal lymph node enlargement with increased fluorodeoxyglucose uptake.\nTo evaluate the primary site of metastatic squamous cell carcinoma, upper gastrointestinal endoscopy was performed; a deep ulcerative mass covered with yellow exudates was detected on the mid to distal esophagus. The biopsy showed moderately differentiated squamous cell carcinoma (). Skin biopsy was also performed on the abdominal skin lesion and the histology showed suprabasal acantholysis with bullous cleft formation () and C3 deposits in the basal layer of mucosa, which was compatible with PNP. The patient was finally diagnosed with PNP associated with esophageal cancer of clinical stage IVB (American Joint Committee on Cancer, 7th edition).\nThe patient's skin lesion and general condition showed rapid aggravation, therefore, the patient was admitted to the intensive care unit for close observation and supportive care. Palliative combination chemotherapy with fluorouracil and carboplatin was started for control of the underlying esophageal cancer activity. We chose carboplatin instead of cisplatin because of the underlying diabetic nephropathy. Intravenous methylprednisolone 40 mg/day was also started simultaneously for treatment of pemphigus. Despite the concomitant chemotherapy and steroid treatment, the skin lesion worsened, with extensive skin defects, progressive exfoliation, and increased discharge.\nOn the sixth day of chemotherapy, neutropenic fever developed. Although broad spectrum antibiotics were started immediately, he expired due to subsequent overwhelming sepsis on the ninth day of chemotherapy.