[CIS-PAGID] a newborn with an extensive skin lesions

[Church, Joseph]

Consider STAT3 Hyper IgE syndrome as well.  Has a bone marrow been done?


 

Joe Church

Children's Hospital Los Angeles

 

________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of YaeJean Kim
Sent: Thursday, July 07, 2011 5:56 AM
To: pagid
Subject: [CIS-PAGID] a newborn with an extensive skin lesions

 

Dear All,

 

I have a question about a neonate with severe skin lesions. 

 

40 days old female full-term baby who presented with whole body rash
since day 3 after birth.

She has been treated multiple rounds of antibiotics for r/o sepsis
(leukocytosis and high CRP, no pathogen, skin lesion) and was
transferred to our NICU. 

 

No significant birth hx (full-term, 2.8 kg, vaginal delivery), or family
hx of PID.

 

On arrivail, extensive skin lesions and striking leukocytosis continued 

6/14/2011 WBC 39.4 (Myelocyte 10, metamyelo 8, band 4, seg53, lymph 18,
mono 6, atypical lymph 1, eos 0), Hb 10, plt 99

7/2/2011   WBC 56.5 (myelo 9, metamyelo 9, ban 7, seg 59, eos 1, lympho
12, mono 11),  Hb 8.9, Plt 55K

HIV-, VDRL-

 

-> recently fever continued, developed mild hepatosplenomegaly 

 

DHR normal

 

IgG 1090 mg/dL (<- IVIG was given at other place)

IgA 5 mg/dL 

IgM 8 mg/dL

IgE 161.5 IU/mL

CH50 85 U/mL

 

lymphocyte subset 

=========================
 Parameter             Test value            reference   for her age 

  (MoAb)              %  Count(/ul)                            %
count

--------------------------------------------    
 T  (CD3)                 88   5,974                  72% (60-85%) ,
4,600 (2,300-7,000)
 T4 (CD4)                72   4,867                  55% (41-68%),
3,500 (1,700-5,300)

 T8 (CD8)                14     959                    16% (9-23%),
1,000    (400-1,700)    
 T4/T8 ratio           5.08                       

 B  (CD19)                  1      74                     15% (4-26%)
1,000 (600-1,000)

 NK (CD16+56+3-)  10     664                   8% (3-23%)        500
(200-1,400)
 NKT(CD16+56+3+)  1      74
---------------------------------------------

 

Bone marrow, non-diagnostic, RF (-)

 

skin bx showed lymphocyte infiltraion in vascular wall and dermis ->
vasculitis, no organisms (fungus -, bacteria -, mycobacteria -, HSV -,
adenovirus -, CMV -, EBV -)

 

At first, I thought of hyper IgE then I was suspecting SCID. B cell is
very low but T cells are within normal. I was also thinking the
possibility of maternal engraftment, but there is no eosinophilia
although she has IgE already 161. Should check for chimerism? 

 

Her condition is waxing and waning and deteriorating gradually.  Skin
lesions are now quite nodular.. we are quite concerned about this baby
and I hope to get some help from you for further work-up.

I would appreciate any suggestion. 

 

YaeJean 

 

 

 



-- 

Yae-Jean Kim, MD 

Assistant Professor 

Division of Infectious Diseases 

Department of Pediatrics 

Sungkyunkwan University School of Medicine 

Samsung Medical Center 

50 Irwon-dong Gangnam-gu 

Seoul, Korea
Tel)  +82-2-3410-0987  Fax) +82-2-3410-0043

yaejeankim at skku.edu

 



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