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

Thu Nov 30 10:42:20 EST 2017

The suggestions to use IV products subq and to consider complement
deficiency and immune complexes are good ones. You didn't tell us what dose
the patient was receiving when she was getting IGIV or what her trough
level was. I couple of thoughts come to mind:
1. The excipients for Privigen (proline) and Gammagard (glycine) are
different implying that it is the IgG and that it is unlikely that a change
in product will make a difference. One could argue that the rash is being
caused by trace contaminants but I doubt it.
2. Despite my comment above, I have used Carimune NF subq at 16% without a
problem.
3. If the patient does have a complement deficiency, I don't know what
could be done differently.
4. Although I have great respect for Marianna, I don't think it will be
possible to desensitize to IG as though it was a small molecule.
5. If the rash is being caused by deposition of immune complexes, that
could be confirmed by immunofluorescence studies of the biopsy. If there
are immune complexes, then I would consider using high dose IVIG in an
effort to capture the antigenic load. The first infusion is likely to
result in rash but if you keep the level high, the rash may not continue.
Actually, if you are in high enough antibody excess, immune complexes may
not be deposited. If you think that the antigens in the immune complexes
are bacterial, you might treat with a several week course of a broad
spectrum antibiotic before that first dose of IGIV.

Richard Wasserman
Dallas

On Thu, Nov 30, 2017 at 9:23 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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
>
>
>
>
>
> *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
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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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-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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