A Young Man with Henoch-Schönlein Purpura

Author: V. Dimov, M.D., Assistant Professor, University of Chicago
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.

The increased frequency of peripheral Th17 cells and serum IL-17 levels are shown in childhood HSP that may in part contribute to vascular inflammation, suggesting cellular immunity is likely to be involved in the process of HSP (Ped Allergy Immunology, 2011).

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.

Henoch - Schönlein Purpura (HSP) Mnemonic

HSP = 10 P

PALPABLE
PURPURA
PLATELETS OK -- DDx from ITP
PRURITUS -- URTICARIA
PAIN -- ABDOMEN and LEGS
POSITIVE +guaiac -- FECES
PRESSURE -- increased blood pressure
PROTEINURIA
PREDNISONE = Tx

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.
HSP. The 5-Minute Clinical Consult, 2007 By Frank J. Domino.
HSP. Differential diagnosis in internal medicine: from symptom to diagnosis By Walter Siegenthaler.
Henoch-Schönlein Purpura - JAMA Patient Page, 2012.

Note: The patient agreed to provide the photos for this publication and to the use of the text and images for online education.

Published: 08/19/2006
Updated: 01/26/2012

7 comments:

  1. Interesting case ..
    and really a great blog ..


    Thanks a lot.

    Mohammad, KSA.

    ReplyDelete
  2. I developed HSP about one month after my 58th birthday.
    I know I am a rare case, but I suggest you never stress too much the age criteria when diagnosing.
    A better criteria would be to check for raised blood pressure levels.
    HSP often causes elevated BP. My BP is normally "perfect" I am told, but was high when I visited the hospital emergency room at the conditions onset.
    I am disappointed BP was omitted from the above list of criteria.
    Not ashamed to be different, I am apparently the perpetual adolescent I have always claimed to be.

    ReplyDelete
  3. That prior comment about my bout of HSP was listed as anonymous because I didn't wish to screw any longer with the "profile" criteria necessary to post a message.
    But if any one has knowledge of any sufferer of HSP who was older than a good whiskey at the onset of symptoms I would appreciate some details as to the lingering affects such as swollen joints and arthritic pains.
    A year after the fading of the rashes, raised welts and open sores my lower legs are discolored, and the stiffness and pains in the joints persist. Thankfully, my kidneys were only slightly and briefly affected, though my good doctor is still vigilant.
    I can be reached at acp.design@yahoo.com

    ReplyDelete
  4. i got mine when i was in 1st grade and have never gotten it again and i am 13

    ReplyDelete
  5. good case to review...

    ReplyDelete
  6. I got mine when I was pregnant and I was 34 years old and have never gotten it again

    ReplyDelete
  7. good case .. real patients commenting ... fantastic ...

    ReplyDelete