Photoclinic Diagnosis: Primary Intestinal Lymphangiectasia

A17-year-old boy was referred for evaluation of chronic watery diarrhea. He had a history of large-volume nonbloody diarrhea for four months, about three times a day, not associated with pain or weight loss. There was a history of perioral paresthesia with hand and foot cramps for three months, which was treated by three grams of daily elemental calcium and 8000 IU of calcitriol (Rocaltrol®). On physical examination, the vital signs were normal without fever or any peripheral lymphadenopathy. The body mass index was 20 kg/m2. Mild anemia and bilateral pedal edema were detected, along with iron deficiency anemia, hypokalemia, hypocalcemia, hypoalbuminemia, and panhypogamma- globulinemia. Direct stool examination and culture showed no pathogen and it was negative for occult blood. Fat droplets were detected in stool by Sudan III staining. HIV antibody was negative. Abdominal ultrasonography, chest radiography, echocardiography, and upper gastrointestinal endoscopy were normal. Abdominal and pelvic computed tomography with intravenous and oral contrast showed no ascites or lymphadenopathy.