A Young Man with Henoch-Schönlein Purpura

Author: V. Dimov, M.D.
Reviewer: S. Randhawa, M.D.

A 31-year-old Caucasian male is admitted to the hospital with a 10-day history lower extremities rash and hands and feet swelling. He had upper respiratory tract infection before his current symptoms started.

A biopsy of skin lesions at an outside hospital shows leukoclastic vasculitis. All his other laboratory results are normal, including ESR, CRP, UA, CBC, CMP.

He is requesting a second opinion.

Past medical history (PMH)

Negative.

Medications

Celecoxib (Celebrex) 200 mg po daily started 5 days ago.

Physical examination

Afebrile, VSS.
WD/WN in NAD.
Chest: CTA (B).
CVS: Clear S1S2.
Abdomen: Soft, NT, ND, +BS.
Extremities: edema of hands and feet (figure 1), purpura of lower extremities (figures 2, 3).


Figure 1. Edema of hands and feet (click to enlarge the image).


Figures 2, 3. Purpura and edema of lower extremities (click to enlarge the images).

What tests would you order?

CBC, CMP, UA, ESR, CRP are all normal.
X-rays of hands are normal.

What is the likely diagnosis?

Henoch-Schönlein Purpura (HSP).

What test would you order to help confirm the diagnosis?

IgA
ASO
Throat culture
GC/Chlamydia probe
Stool guaiac x 2

What happened?

IgA level was elevated: IgA 516 mg/dL (lower limit 78, upper limit 391). ASO was elevated but the the throat culture was negative. The rest of the tests were normal.

Rheumatology and ID consults confirmed the diagnosis of Henoch-Schönlein purpura.

On hospital day 2, the purpura and swelling started to resolve. The patient felt much better and was discharged home with Celecoxib (Celebrex) 200 mg po qd and follow-up with rheumatology in 3-4 weeks. He was scheduled to have UA and BMP at that time to monitor for development of HSP-related renal disease.

Final diagnosis

Henoch-Schönlein purpura.

What did we learn from this case?

Henoch-Schönlein purpura (HSP) is a self-limited systemic vasculitis. It is characterized by 4 clinical syndromes:

1. Palpable purpura in the absence of thrombocytopenia or coagulopathy. Develops in 100% of patients.
2. Arthritis/arthralgia in 45-75% of patients. Second most common manifestation of HSP.
3. Abdominal pain in 50%, GI bleeding (often occult) in 20-30% of patients.
4. Renal disease in 20-50%.

Our patient fulfilled 2 of the 4 ARA criteria for the classification of HSP:

1. Palpable purpura
2. Age 20 or younger at disease onset
3. Bowel angina
4. Wall granulocytes on biopsy

HSP is an immune-mediated vasculitic disorder that is suspected to be triggered by an IgA-mediated response to an antigen. Serum IgA levels are elevated in 50 to 70% of patients with HSP, which was also observed in our patient.

HSP is uncommon in adults but when it develops, adult patients are at increased risk for renal involvement which may lead to CKD in 10% and end-stage renal disease in additional 10%.

Treatment of HSP generally consists of NSAIDs for arthritis/arthralgia. Glucocorticosteroids may be beneficial in patients with abdominal pain. There is no proven treatment for prevention of progression of renal disease.

References

Henoch-Schönlein Purpura: A Review. American Family Physician, 1998.
Henoch-Schönlein Purpura. eMedicine.
Management of Henoch-Schönlein purpura.
Clinical manifestations and diagnosis of Henoch-Schönlein purpura.

Related Reading

Interesting Case of Adult Henoch-Schonlein Purpura. Renal Fellow Network, 2009.

Henoch-Schönlein Purpura (HSP) in Mini Oxford handbook of clinical medicine By Murray Longmore, Ian Wilkinson, Supraj R. Rajagopalan:



HSP. The 5-Minute Clinical Consult, 2007 By Frank J. Domino.

HSP. Differential diagnosis in internal medicine: from symptom to diagnosis By Walter Siegenthaler.

Published: 08/19/2006
Updated: 08/26/2009