[CIS PIDD] [cis-pidd] Puzzling Case

[Kirkpatrick, Charles]

Joe:

Maybe measurement of cleavage fragments (C4a) or membrane attack complexes could shed some light on consumption vs. primary.

Chuck Kirkpatrick

-----Original Message-----
From: Church, Joseph [mailto:JChurch at chla.usc.edu] 
Sent: Wednesday, June 19, 2013 8:04 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] Puzzling Case

Kate, Mikko:

Thank you for your thoughts.  Since all measured classical component levels are low it is difficult to tell what is primary and what is consumption.  Sequencing is being pursued.

JC

-----Original Message-----
From: Sullivan, Kathleen [mailto:sullivak at mail.med.upenn.edu]
Sent: Wednesday, June 19, 2013 3:26 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Puzzling Case

C3 deficiency

Almost assuredly

Kate
On Jun 18, 2013, at 6:53 PM, Church, Joseph wrote:


> Colleagues:

> 

> Rheumatology referred a now 6 month infant girl who since 5 weeks of age has had a persistent, shifting, generalized rash characterized by irregular reddish-brown plaques (see attached picture) that are completely non-pruritic.  A biopsy showed "urticarial vasculitis."  A brief course of prednisone resulted in transient complete clearing of the rash.  The infant is growing and developing normally; she has had no serious infections; her exam is normal except for the rash; there are no joint findings.  Labs:

> 

> CH50 and AH50 were '0' (repeatedly).

> All classical pathway component tested were low.

> RF, CIC, anti-C1q all positive

> ANA, anti-dsDNA, SSA and SSB all negative (mom also).

> UA normal

> Extensive search for infectious agents has been unrevealing.

> CBC shows neutropenia with positive anti-neutrophil antibody, and anemia-Coombs pending.

> 

> Other than neonatal lupus any thoughts regarding diagnostic interventions and possible therapy (?IVIG).

> 

> Joe Church

> Children's Hospital Los Angeles

> 

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Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



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