A 12 Year Old Girl Presents With Abdominal Pain, Bloody Diarrhea And A Purpuric Rash.

A 12 year old girl is admitted to the pediatric ward with a 2 day history of Lower abdominal pain and blood stained diarrhea. Three days later she began to complain of pain in her ankles and the right elbow. She also felt nauseous but there was no history of vomiting.
On examination the patient looks sick, has a periorbital edema and a purpuric rash affecting the arms and the legs.
The picture of her rash on lower extremeness is shown below:

She is afebrile but her BP is 150/95 mmHg.
The lab workup and the results are given below:

• Hb = 10g/dl
• WBC= 12000/cmm
• Platelets= 135,000/cmm
• ESR= 35
• PT = 13 sec (control= 13 sec)
• aPTT= 34 sec ( control= 36 sec)
• Sodium=138 mmol/l
• Potassium= 5.9mmol/l
• Urinalysis= Blood++, Protein++

Based on the above history and investigations what is the most probable diagnosis?

?
?

.

Henoch-Schonlein Purpura ( The combination of lower abdominal pain, bloody diarrhea, purpuric rash, nephritis, and a high blood pressure in a young girls are all suggestive of the disease)

Case Discussion:

Henoch-Schonlein Purpura is a small vessel vasculitis that most commonly occurs in young children aged 4-15 years. This disorder causes inflammation and bleeding in the small blood vessels of the skin, joints, kidneys and intestine. 

Clinical features: The most striking feature of Henoch-Schonlein Purpura is a purpuric rash most commonly seen on the lower legs and the buttocks. Other symptoms include:

• Abdominal pain
• Bloody diarrhea
• Nausea and vomiting
• Fever
• Joint pain especially involving the knees and the ankles
• Anorexia
• Blood in urine
• Periorbital edema

The classic triad of Henoch- Schonlein purpura is purpura, arthritis and abdominal pain. 

Diagnosis: The classic clinical presentation is very helpful in diagnosis, however sometimes all the characteristic symptoms may not appear immediately and so the definite diagnosis is made by a skin or a renal biopsy. 

• Skin biopsy demonstrates a leucoclastic vasculitis with IgA deposition.
• Renal biopsy reveals mesangial IgA deposition associated with glomerulonepritis. 

Treatment: 

1. Analgesics may be needed for abdominal pain and arthritis.

2. It is important to ensure proper hydration

3. Monitor for gastrointestinal or renal complications. 

4. Antibiotics may be needed to treat an underlying infection.

5. Steroids are generally avoided but may be needed in cases where there is persistent nephrotic syndrome, severe abdominal pain, severe soft tissue edema and any signs of neurologic involvement.

5. Immunosuppresive drugs like azathioprine, cyclophosphamide and cyclosporine may be needed in certain patients.

6. Plasmapheresis may be effective in delaying the progression of kidney disease.