[CIS PIDD] [cis-pidd] Puzzling Case

[Cooper, Megan]

Hi - Have you looked at NALP3/cryopyrin?  It looks like an FCAS rash.  We have pts like this without fever.  Are inflammatory markers up?


Megan

Megan Cooper
Washington University

On Jun 18, 2013, at 5:55 PM, "Church, Joseph" <JChurch at chla.usc.edu> 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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