[CIS PIDD] [cis-pidd] Puzzling Case

[Church, Joseph]

Thank you for your suggestion.  Testing of alternative pathway components is in process.  JC

From: Nacho Gonzalez [mailto:nachgonzalez at gmail.com]
Sent: Tuesday, June 18, 2013 4:36 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Puzzling Case


Dear Dr. Church,
I' ve seen three patients with factor I deficiency. Two of them showed persistent/recurrent schonlein-henoch purpura with CH50 0-5 & AP50 0 (they were 8 and 5 year old,perhaps skin manifs may be somewhat different) so have you tested Factor I, factor h and anti factor h antibodies? Agree that in factor I def usually c3 is low with normal measurement of classical pathway components

Regards
Luis Ignacio Gonzalez-Granado
Immunodeficiencies
Hospital 12 octubre
El 19/06/2013 00:55, "Church, Joseph" <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> escribió:
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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