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

Thu Nov 30 11:23:22 EST 2017

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 Malbran

UAAIC

Buenos 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 Medicine

Service Chief, Adult Allergy and Immunology

Baylor College of Medicine and Texas Children's Hospital

Section of Immunology, Allergy & Rheumatology

joud.hajjar at bcm.edu<mailto:joud.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

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________________________________

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, MD

Allergy and Asthma Family Care/St Francis Hospital and Medical Center

Connecticut

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