[CIS PIDD] [cis-pidd] IgG rash in CVID patient

Thu Nov 30 12:23:53 EST 2017

thanks Alex.  I will keep this in mind.
-Shayna

      From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
 To: CIS-PIDD <cis-pidd at lyris.dundee.net> 
 Sent: Thursday, November 30, 2017 11:23 AM
 Subject: Re: [cis-pidd] IgG rash in CVID patient

Hi Shayna,
We have one patient with CVID and an erythrodermic eczema that responded to steroids and ciclosporin. She entered onto remission and continued her IVIG w/o further skin lesions. Best regards,
Alex MalbranUAAICBuenos Aires, Argentina

From: cis-pidd at lyris.dundee.net <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Sent: Thursday, November 30, 2017 12:23 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] IgG rash in CVID patient Hi Shayna, You may consider checking complement levels. Sometimes patients with Complement deficiency form immune complexes (IVIG and circulating antigens) and fail to clear those due to complement deficiency. A nice abstract last year during CIS meeting described a similar case, and smaller IVIG dose seemed to help. https://cis.confex.com/cis/2017/webprogram/Paper5106.html regards,Joud Joud Hajjar MD, MS.Assistant Professor of MedicineService Chief, Adult Allergy and ImmunologyBaylor College of Medicine and Texas Children's HospitalSection of Immunology, Allergy & Rheumatologyjoud.hajjar at bcm.edu  From: <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Date: Thursday, November 30, 2017 at 7:18 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: Re: [cis-pidd] IgG rash in CVID patient ***CAUTION:*** This email is not from a BCM Source. Only click links or open attachments you know are safe.Hi All, I am treating a 39 you female with ITP and CVID previously diagnosed by oncology.  Prior to starting IVIG, she had nondetectable immunoglobulins (ie. IgA <10, IgM <20, IgG <75).   I was asked to see her after she had been off of IVIG for >1 year and required multiple admissions to the hospital for sinopulmonary infections (~every 2-3 months) while being off of IVIG.  Her IVIG was stopped since Nov 2015 due to development of worsening rashes while on both Privigen and Gammagard.  Her rashes were diffuse popular extremely pruritic rashes that eventually became painful as well.  She required steroids for treatment and the rashes eventually resolved but have left permanent hyperpigmentation that the patient is not happy about.  When I saw her recently, I suggested a trial of Hizentra weekly, as I hoped that smaller doses SQ weekly might prevent the rashes from occurring and allowing her to received her needed IgG replacement.  The patient tolerated the first 2-3 infusions without any problems, but over the past 2 weeks has now again developed extremely pruritic papules/patches diffusely on her legs, arms, abd, back, and neck.  However, she has not gotten sick nor been hospitalized since starting Hizentra, which is very good for her.   Biopsy of one of the lesions showed likely drug reaction (see below for official path results). Skin with Chronic Interface Dermatitis and Chronic Perivascular Dermatitis
(Superficial and Deep). 
Comment: The surface of the skin shows hyperkeratosis. There is a chronic 
interface lymphocytic dermatitis with spongiosis. Superficially and also in the 
deeper dermis, there is a pronounced chronic inflammatory perivascular 
infiltrate composed of lymphocytes. The findings favor an allergic/ 
hypersensitivity response or possible drug reaction.    So, I'm wondering if anyone has any recommendations on how to proceed?  She clearly ideally needs IgG replacement, but I'm not sure how to get it to her.Thanks!  -Shayna Burke, MDAllergy and Asthma Family Care/St Francis Hospital and Medical CenterConnecticut   ---You are currently subscribed to cis-pidd as:shaynaburke at yahoo.com.To unsubscribe click here:http://cts.dundee.net/u?id=96396884.0c351f975bf8c3cf588a002ca4251489&n=T&l=cis-pidd&o=4681117(It may be necessary to cut and paste the above URL if the line is broken)or send a blank email toleave-4681117-96396884.0c351f975bf8c3cf588a002ca4251489 at lyris.dundee.net ---You are currently subscribed to cis-pidd as: joud.hajjar at bcm.edu.To unsubscribe click here: http://cts.dundee.net/u?id=96396690.01670be70beac432bf57127b486e9165&n=T&l=cis-pidd&o=4681976(It may be necessary to cut and paste the above URL if the line is broken)or send a blank email to leave-4681976-96396690.01670be70beac432bf57127b486e9165 at lyris.dundee.net---You are currently subscribed to cis-pidd as: amalbran31 at hotmail.com.To unsubscribe click here: http://cts.dundee.net/u?id=96396385.0e0ce4b28f5c58f0e2bcb783c8a2ad86&n=T&l=cis-pidd&o=4682168(It may be necessary to cut and paste the above URL if the line is broken)or send a blank email to leave-4682168-96396385.0e0ce4b28f5c58f0e2bcb783c8a2ad86 at lyris.dundee.net ---  You are currently subscribed to cis-pidd as: shaynaburke at yahoo.com.  To unsubscribe click here: http://cts.dundee.net/u?id=96396884.0c351f975bf8c3cf588a002ca4251489&n=T&l=cis-pidd&o=4682243  (It may be necessary to cut and paste the above URL if the line is broken)  or send a blank email to leave-4682243-96396884.0c351f975bf8c3cf588a002ca4251489 at lyris.dundee.net

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