Rare and Unusual: Henoch–Schönlein purpura

One of my friends (who shall remain nameless) is queen of the unusual. Things happen to her that have less than 1% chance of happening…and these things happen to her in multiples. The calculated combined chance of all these things happening to one person in one lifetime is roughly…nevermind, it’s been too long since stats class.

My nameless friend has graciously allowed me to share briefly about her rare illness on this blog to raise awareness, in hopes that some other patients may get appropriate care. Here is her rough history/timeline at time of illness:

• Asian/Korean female, age 24, married
• Recently had vaccination protocol for travel to Africa
• Stomach/abdominal pain—saw doc twice for this complaint, doc treated with prescription antacid
• Stomach pain, third visit—accompanied by joint pain, arthritic hands, and fine rash on a few areas of body; diagnosed with virus, no treatment
• Stomach pain, fourth visit—accompanied by joint pain, bloody rash that had spread to multiple areas of body, and malodorous diarrhea; treated for gonorrhea (did not have gonorrhea)
• Received correct diagnosis via dermatological evaluation of rash/lesion biopsy after two days in quarantined hospital bed
• Received steroid treatments in hospital for additional three days and for three weeks post-discharge
• Complications: kidney problems that resolved with treatment (IgA nephropathy)

Henoch–Schönlein purpura (HSP) is a rare form of vasculitis with relatively unknown etiology, although immune system reactions, such as those that occur with viral infections, vaccinations, or insect bites may trigger the condition. Children and young adults experience HSP more often than other demographic groups, with over half of the cases linked to recent upper respiratory illnesses.

All cases of HSP exhibit the large, dark-red splotchy rash that may look like severe bruising. The rash is usually found on the limbs. Other common symptoms include joint pain and swelling, truncal rash, abdominal pain (80% of cases), nausea, vomiting, diarrhea, and acute kidney involvement (manifests as protein and/or blood in urine). Joint pain may arise before the notable rash manifests.

Risk factors include being Caucasian or Asian, early elementary age, recent infections or vaccinations (especially for typhoid, measles, yellow fever, or cholera), and male.

Diagnosis is largely based on combination of symptoms. Other nonspecific findings include elevated platelets (not common to other purpuras), elevated IgA levels, elevated creatinine and urea, or elevated ESR/CRP levels. Diagnosis may also be made via skin biopsy, which may show a distinct stippled pattern of vascular fluorescence (found in 87% of lesion biopsies, per one study).

Treatments focus on managing pain and kidney complications. Medications other than painkillers are rarely necessary. Occasionally, anticoagulants such as heparin or warfarin may be used along with steroids to reduce kidney or other organ complications, but these are not considered routine courses of treatment.

Patients may have recurrences and rare lasting kidney complications, but most are expected to make a full recovery. I am most happy to say that my dear friend has fully recovered, other than some residual scarring from the lesions. Have you cared for any patients with this rare condition? Let us know in the comments!

Resources:

http://www.ncbi.nlm.nih.gov/pubmed/13680332

http://www.ncbi.nlm.nih.gov/pubmed/3534012

http://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura

http://www.mayoclinic.com/health/henoch-schonlein-purpura/DS00838/DSECTION=symptoms