[PAGID] Newborn with Sweet's syndrome and hypogammaglobulinaemia

[Cowan, Mort]

I may have missed it, but what is the lymphocyte count? You might want
to look for maternal engraftment; sounds a little like GVHD and SCID
kids can have unusual biopsy findings, not typical of GVHD. A bone
marrow exam would certainly be helpful as well as T cell studies.

 

Morton J. Cowan, M.D.

Professor of Pediatrics

Chief, Blood and Marrow Transplant Division

UCSF Children's Hospital, Room M659

505 Parnassus Ave

San Francisco, CA 94143-1278

 

Phone: 415-476-2188

FAX: 415-502-4867

 

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________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of John Ziegler
Sent: Monday, April 13, 2009 4:45 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] Newborn with Sweet's syndrome and
hypogammaglobulinaemia

 

We are looking for ideas about a 1 month old boy with 
  
1) Neonatal Sweet's syndrome 
2) Hypogammaglobulinemia 
3) Hepatosplenomegaly 
4) Thrombocytopenia 

This is the first child of well unrelated Chinese parents, born in
Sydney by vaginal delivery after a pregnancy complicated by hyperemesis.
Mum had no medications during pregnancy, there were no infectious
symptoms and the antenatal infectious screening was negative.   There is
no family history of skin disease or immune deficiency and no deaths in
early childhood.

He presented with a mild vesicular rash a few minutes after birth, and
the rash progressed over the next 3 weeks.  The distribution is head,
arms, legs, scrotum and upper thorax, and the appearance is of elevated
lesions with deep dermal infiltration and necrotic overlying skin.
Biopsy demonstrated panniculitis with extensive neutrophilic
leukocytoclasis throughout the dermis and subcutaneous tissue.  This
neutrophilic dermatosis was thought to be consistent for Sweet's
syndrome, however, the young age and panniculitis are atypical. The rash
was associated with a mild peripheral blood neutrophilia which reached
11,000, a significant monocytosis = 4.1 (0.3- 1.2), CRP =179 and ESR =
90.  Associated with this is ongoing thrombocytopenia with platelet
counts between 41-65,000.  There is moderate splenomegaly 7.3 cm (upper
limit normal 5 cm) but no Howell Jolly bodies on the blood film, and
unfortunately the attempted bone marrow biopsy was an insufficient
sample.  He has hepatomegaly 7.0 cm (normal 4-6) with raised gGT = 400
and low albumin = 22, but with relatively normal AST and ALT. ANA was
-ve in the baby and has not yet been determined in the mother.     

He has had a negative infection screen thus far, including for direct
viral detection of HSV, VZV, CMV and Enterovirus from a variety of
fluids.  No organisms were grown on MC&S, other than a sensitive E. coli
on eye swab from a pussy eye, while gram/ fungal stain from the skin
biopsy were negative (cultures of biopsy were not performed).  

He was treated with steroids and seemed to have some initial response
but despite 1 mg/Kg prednisolone continues to develop new skin lesions.


He was also found to be hypogammaglobulinaemic with no IgA or IgM and
IgG of 2.3, and 1% of B-cells.  He is now being treated with IVIG and we
are awaiting a response.  T cells were normal in number; T cell function
has not yet been assessed.

Has anyone seen a neutrophilic dermatosis at birth in an immunodeficient
child?  Other than congenital infection, what mechanisms might be
involved?  If this is a PID, other than XLA what possibilities should be
considered?

 

Summary of investigations: 

FBC/ CBC 

Hb              100 (Holding up) 
Lymphocytes:    4.1 @ presentation, currently 1.3 on steroids. 
Monocytes:      Highest = 4.1 (0.3 - 1.2), now normal range on steroids.

Neutrophils:    Highest = 11, prior to steroids.    

Immunoglobulins (@1 month of age)  

IgG                             2.30    g/L         1.7 - 5.8  
IgA                             <0.06   g/L        0.00 - 0.50 
IgM                             <0.05   g/L        0.19 - 0.95 
IgE                     <5      IU/ml      0 - 1.5  

Lymphocyte subsets (@1 month of age)  
Lymphocytes                     3.6     X10^9/L     3.8 - 7.6  

CD3                             3.28    x10^9/L     2.3 - 7.0  
CD3                             91      %         

CD4                             1.91    x10^9/L     1.7 - 5.3  
CD4                             53      %         

CD8                             1.37    x10^9/L     0.4 - 1.7  
CD8                             38      %        

CD19                            0.04    x10^9/L     0.6 - 1.9  
CD19                           1       %         

NK CELLS                0.14    x10^9/L     0.2 - 1.4  
NK CELLS                  4       %       

 

Dr Paul Gray and A/Prof. John B. Ziegler 
Department of Immunology & Infectious Diseases 
Sydney Children's Hospital 
High St., Randwick NSW 2031 
Australia 
T: (02) 93821515 
F: + 61 + 2 93821580 
E: j.ziegler at unsw.edu.au 

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