[CIS PIDD] [cis-pidd] Puzzling Case

[Nacho Gonzalez]

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> 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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