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

Wed Oct 22 18:29:30 EDT 2014

I would suggest ordering 16S PCR on the joint fluid to cast as wide a net as possible for prokaryotes or perhaps unbiased NextGen sequencing as reported by Wilson et al in June (PubMed PMID: 24896819).  We are involved in the case of a patient with CVID currently being treated for Mycoplasma salivarium septic arthritis involving multiple joints.  It grew out after a couple of weeks in culture but 16S PCR provided the answer more quickly.

Prescott

T. Prescott Atkinson, MD PhD, Director
Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel 205-638-9072
Fax 205-975-7080

From: Boyce, Thomas G., M.D. [mailto:Boyce.Thomas at mayo.edu]
Sent: Wednesday, October 22, 2014 5:06 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] Patient with NEMO rash and joint inflammation

PCR for MAC on skin biopsy?
Could be pyoderma gangrenosum.
Strongly doubt Lyme.

From: Cunningham-Rundles, Charlotte [mailto:charlotte.cunningham-rundles at mssm.edu]
Sent: Wednesday, October 22, 2014 3:07 PM
To: CIS-PIDD
Cc: Welch, Kate; Gulko, Percio
Subject: [cis-pidd] Patient with NEMO rash and joint inflammation

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<mailto:Charlotte.Cunningham-Rundles at mssm.edu>

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