A 20 years old female patient, the sixth of a family of 8 children is treated since 2010 for edematous-ascitic syndrome related to a primary intestinal lymphangiectasia (or Wildmann's disease) suspected by hypoalbuminemia, lymphopenia and hypogammmaglobulinemia. The disease was confirmed by an intestinal biopsy indicating dilated lymphatic ducts Figure 1, Figure 2. The patient followed a low-fat diet associated with medium-chain triglycerides, vit A and K supplementations.
The evolution was good with effusions resorption. However, 6 months ago, the patient presented MRC grade 3 dyspnea with alternated fever and apyrexia. It appeared after she chose to stop, 8 months ago, the low fat diet that was prescribed by her pediatrician. The physical exam revealed a cachectic, apyretic patient with BMI of 17kg / m2, WHO performance index of 1, a minor Conjunctivial discoloration and a RR of 22 B/min. The lung exam showed a bilateral fluid effusion syndrome. The cardiovascular exam indicated a pericardial rub with a systolic murmur at the mitral area. The abdominal exam found a shifting dullness with a smoothed umbilicus. The skin exam showed a thin skin with no dehydration signs, skin lesions or edema. Suspected diagnoses were: Tuberculosis, type B Lymphoma, Disseminated Lupus erythematosus. Paraclinical tests showed a moderate inflammatory syndrome. The AFB sputum examination by direct microscopy, the tuberculin skin test, the 24h proteinuria and the immunological tests were negative. The thoracentesis revealed a lymphocytic exudative fluid with no MTB in the pleural fluid (direct microscopy) and a negative ADA level. The abdominal ultrasound showed medium-abundance ascites associated with an abundant pleurisy and a renal cyst. Heart ultrasound reveals a pericardial effusion with a normal ejection fraction. However, no causal factor has been found in either thoracoabdominal CT scan, bronchoscopy or thoracoscopy.