Intestinal lipodystrophy

Alternative names

Whipple's disease

Definition

Whipple's disease is a rare condition that causes malabsorption (inadequate absorption of nutrients from the intestinal tract) due to infection of the intestine.

Causes, incidence, and risk factors

Whipple's disease is most likely caused by infection with Tropheryma whippelii. The disorder primarily affects middle-aged white men, and the onset of symptoms is usually slow. Without treatment, it may be fatal.

Whipple's disease is extremely rare, and risk factors are unknown.

Symptoms

• Abdominal pain
  
• Fatty stools
  
• Fever
  
• Diarrhea
  
• Weight loss
  
• Gastrointestinal bleeding
  
• Enlarged lymph glands
  
• Joint pain
  
• Edema
  
• Gray to brown skin coloration
  
• Mental status changes
  
• Memory loss
  

Signs and tests
Primary tests:

• Small bowel biopsy showing infection-fighting cells containing bacteria (other body parts can also be biopsied)
  
• Complete blood count (CBC) showing anemia
  

This disease may also alter the results of the following tests:

• Fecal fat
  
• D-xylose absorption
  
• Albumin
  

Treatment

Patients should receive prolonged antibiotic therapy with a drug that can treat infections of the brain and central nervous system. If symptoms reappear during therapy, patients may require a change in their antibiotic regimen.

Relapses can occur after therapy has been completed, requiring close patient monitoring.

Patients who experience nutritional deficiencies caused by the malabsorption will receive treatment with appropriate dietary supplements.

Expectations (prognosis)

Without treatment, the condition is usually fatal. Treatment improves the chance of a good outcome.

Complications