[CIS PIDD] [cis-pidd] 40 year old woman with Behcet's and adult onset recurrent rapidly progressive cellulitis/sepsis

Tue Oct 6 23:13:11 EDT 2015

The recurrent bacteremia and cellulitis seem most suggestive of a complement or phagocyte disorder - would definitely suggest checking out her alternate pathway and continuing to look at her phagocyte function.  Granule structure by EM and platelet aggregation normal? No signs of any unusual birthmarks that might be incontinentia pigmenti or premature graying of her hair?
Prescott

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, October 06, 2015 9:52 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] 40 year old woman with Behcet's and adult onset recurrent rapidly progressive cellulitis/sepsis

Her IgE is 19.  An AH50 has not been checked.

Her nutritional status seems adequate.  Her vitamin D level hasn't been checked in awhile and that should be repeated in the near future.

Chris

From: "cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Tuesday, October 6, 2015 9:42 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: RE:[cis-pidd] 40 year old woman with Behcet's and adult onset recurrent rapidly progressive cellulitis/sepsis

Hi Chris:  do you have AH50 and IgE test results?
Prescott

T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel 205-996-9582
Fax 205-975-7080

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, October 06, 2015 9:36 PM
To: CIS-PIDD
Subject: [cis-pidd] 40 year old woman with Behcet's and adult onset recurrent rapidly progressive cellulitis/sepsis

Dear colleagues,

I am seeking input on a complex adult patient who was well until 25 years of age. She was referred by an infectious disease specialist for evaluation of her neutrophil function.  Her last two life-threatening documented bacterial infections required the addition of WBC transfusions on top of directed parental antibiotics for clinical improvement.

  1.  25 years of age presented with recurrent fevers and found to have enterococcal endocarditis
  2.  30 years Arthritis with diagnosis of spondyloarthropathy. Started on humira
  3.  Shortly after starting humira experienced first episode of rapidly progressive polymicrobial cellulitis on lower extremity.  This required skin grafting. The graft still hasn't healed completely.
  4.  Shortly afterwards noted to have severe pathergy to the extent that venipuncture could not be done and a central line was placed.  She was also experiencing recurrent oral ulcers.  Slit lamp exam showed mild anterior chamber uveitis and a diagnosis of Behcet's was made.  She was started on prednisone and rotated through a series of immunomodulatory meds. Currently she is on low dose daily prednisone and apremilast.
  5.  She has experienced 5 central line infections, most cultures positive for enterococcus. She has recurrent skin infections. The last two episodes in the past 6 months presented with sepsis-like syndrome. One episode was preceded by image confirmed preseptal cellulitis.  This progressed rapidly necessitating enucleation and the WBC transfusions despite good antibiotic coverage.
Her WBC counts have been slightly elevated with these episodes and normalize in-between. The tissue biopsies have demonstrated chronic inflammation with detectable neutrophils. DHR was normal. HIV negative. MHC Class I and II expression reported as normal. Normal CD11b expression. Normal in vitro inflammatory response to panel of TLR agonists (done commercially). Normal chemotaxis 2D and 3D (done on a research protocol). Normal CH50, normal humoral function, normal T cell function.

This woman is very disabled by her recurrent infections since she has prolonged hospitalizations, end-organ damage, and long recoveries.  These infections seem out of proportion to her low dose daily steroids and her immunosuppressive therapy. I couldn't find a Behcet's case in the literature with this degree of life-threatening infections.

Have any of you seen a similar case in your practice?
Any other diagnositic tests that should be pursued?
Any thoughts on additional therapies?

Thank you

Chris

Christine M. Seroogy MD,  FAAAAI
Associate Professor
University of Wisconsin School of Medicine and Public Health
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
1111 Highland Avenue
4139 WIMR
Madison, WI  53705-2275
phone: 608-263-2652
fax: 608-265-0164

Confidentiality Notice: The information in this message (and the documents attached to it, if any) is confidential and may be legally privileged. It is intended solely for the addressee. Access to this message by anyone else is unauthorized. If you are not the intended recipient, any disclosure, copying, distribution or any action taken, or omitted to be taken in reliance on it is prohibited and may be unlawful. If you have received this message in error, please delete all electronic copies of this message (and the documents attached to it, if any), destroy any hard copies you may have created and notify me immediately by replying to this email. Thank you.

---

You are currently subscribed to cis-pidd as: patkinson at peds.uab.edu<mailto:patkinson at peds.uab.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396838.952f64cd9dda04997031bb8889426d86&n=T&l=cis-pidd&o=3187381

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3187381-96396838.952f64cd9dda04997031bb8889426d86 at lyris.dundee.net<mailto:leave-3187381-96396838.952f64cd9dda04997031bb8889426d86 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: cmseroogy at wisc.edu<mailto:cmseroogy at wisc.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396468.6e28a86ce1555cf89cd74c4f18d7650b&n=T&l=cis-pidd&o=3187402

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3187402-96396468.6e28a86ce1555cf89cd74c4f18d7650b at lyris.dundee.net<mailto:leave-3187402-96396468.6e28a86ce1555cf89cd74c4f18d7650b at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: patkinson at peds.uab.edu<mailto:patkinson at peds.uab.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396838.952f64cd9dda04997031bb8889426d86&n=T&l=cis-pidd&o=3187423

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to leave-3187423-96396838.952f64cd9dda04997031bb8889426d86 at lyris.dundee.net<mailto:leave-3187423-96396838.952f64cd9dda04997031bb8889426d86 at lyris.dundee.net>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=3187450
or send a blank email to leave-3187450-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20151007/9dfb4a74/attachment-0001.html>