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

[CIS-PIDD]

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

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