Case of pneumatosis cystoides intestinalis in adult dermatomyositis

Dear Editor, A 30-year-old Chinese woman presented in June 1995 with progressive proximal muscle weakness of the limb girdles and the neck, dysphagia and skin rash. Periorbital edema with heliotrope rash of the upper eyelids, Gottron papules on the hands were noted. The level of creatine phosphokinase (CK) was 14 180 U/L. Antinuclear antibody, dsDNA, Sm, ribonucleoprotein, SS-A, SS-B, Scl-70 and Jo-1 were negative. X-ray with barium swallow showed weak pharyngeal muscles and a collection of barium in the pyriform sinuses and valleculae. Electromyography showed myopathic changes. Muscle biopsy of the left deltoids showed characteristic myositis. Chest X-ray, abdominal ultrasonography and mammography was normal. Dermatomyositis was diagnosed. Corticosteroids (0.6 mg/kg/day; bodyweight, 50 kg) was instituted with significant improvement of muscle weakness and dysphagia. After 6 weeks, the skin rash on the face disappeared, and she was able to walk and swallow. CK was near normal. After 6 months, she discontinued corticosteroids by herself. She presented in August 1996 with a recurrence of the muscle weakness and pain of the limb girdles. CK was 406 U/L. Corticosteroids (1.5 mg/kg/day) was resumed. After 3 weeks, pain of the muscles relieved, CK was normal. She took a maintenance dose of 10 mg/d prednisolone for 8 years without recurrence. In May 2003, she presented again with progressive abdominal distention, diarrhea, constipation, weight loss and macies (bodyweight, 36 kg). Her abdomen was soft and distended with no spontaneous pain, tenderness or rebound tenderness in the whole abdomen. Gottron papules were seen on both hands (Fig. 1). CK was normal. Serum albumin level was 27.4 g/L (35–55 g/L). All cultures for infectious agents were negative. Ultrasonography of the abdomen showed seroperitoneum. Ascite routine tests revealed a light-yellow color, 25 g/L protein, and a positive Rivalta reaction. Chest X-ray showed free air under diaphragm, and an obviously dilated small intestine and colon (Fig. 2). Plain abdomen radiograph showed pneumoperitoneum and seroperitoneum (Fig. 3). Computed tomography (CT) of the abdomen showed extensive intramural gas-filled cysts involving the ascending colon and small intestine, thickness of the wall of small intestine, thickness of mesentery vessels, multiple calcinosis in the left pelvic cavity, as well as pneumoperitoneum and seroperitoneum (Fig. 4). Diagnosis of pneumatosis cystoides intestinalis and partial intestinal obstruction were made. Diet was discontinued. She was treated with oral liquid paraffin and 10% potassium chloride solution for a week. The patient’s symptoms gradually improved within 1 week. She was discharged after 1 more week. She suffered occasional abdominal distention, diarrhea and constipation but spontaneously remitted during the 3 years of follow up. Pneumatosis cystoides intestinalis (PCI) is an uncommon condition characterized by multilocular