[CIS PIDD] [cis-pidd] Patient with NEMO rash and joint inflammation

Wed Oct 22 16:07:09 EDT 2014

Hello all,

We are hoping to get some insight and suggestions on an interesting but confusing case of a patient with NEMO syndrome and now a rash and arthritis.

Patient is a 28yo man diagnosed with immunodeficiency at a young age and with NEMO at age 12y at the NIH.  His course has been complicated by Neiserria meningitis and disseminated MAC infection but overall he has been doing well.  We switched from IVIGto Hizentra earlier this year, and his IgG levels are good.

Roughly three months ago (after a camping trip to Lake Placid) he developed skin lesions which were not painful or itchy but had  a crusted, granulomatous appearance with a few odd satellites.   He developed about 5 in total (3 on his arms and 2 on his legs) and they are slow to heal.   Does not recall being bitten; no accompanying fever.  Saw a local dermatologist who put him on a 2-3 week course of doxycycline but no biopsy.

In addition, for the past 2 months, he has noted a migrating polyarticularl arthritis, predominantly involving his left MCPs, left elbow and left great toe.  Acutely worsened this week and increased swelling to the point of difficulty opening his hand.

Labs notable for CRP only slightly elevated at 5.9.

We have sent him to derm and rheum this week.  Derm biopsy so far with granulomatous dermatitis; and fluid aspiration with no crystals.  All cultures pending.

We are concerned for a reactive arthritis.  Possible mycobacterial infection.  Possible Lyme, although he was treated and cannot adequately test him, given Ig replacement.

He is on daily azithromycin/moxifloxacin/ethambutol for the MAC history.

We are considering adding back doxycycline and would like to avoid immunosuppression.

Any thoughts  and advice  on workup and treatment are welcome.

Charlotte

Charlotte Cunningham-Rundles, MD, PhD

Departments of Medicine and Pediatrics

The David S Gottesman Professor

The Immunology Institute

Mount Sinai School of Medicine

1425 Madison Avenue

New York, NY 10029

Phone: 212 659 9268

Fax: 212 987 5593

Email: Charlotte.Cunningham-Rundles at mssm.edu

---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=45652150
or send a blank email to leave-45652150-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20141022/0919697d/attachment-0001.html>