[PAGID] Newborn with Sweet's syndrome and hypogammaglobulinaemia

[fabrício prado monteiro]

Despite the initial examinations discard LES initially, I think that would
be important to confirm this and evaluation of a rheumatologist.

Importantly, theses findings further support the hypothesis that lupus
erythematosus should be considered in the differential diagnosis of a
nonbullous neutrophilic dermatosis, as it may represent the initial
manifestation of the disease.
Fabricio Monteiro.




2009/4/13 John Ziegler <John.Ziegler at sesiahs.health.nsw.gov.au>


>  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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-- 
Dr. Fabrício Prado Monteiro.
  Imunologia - Pediatria.

CRM-DF12270 CRM-GO11928

          HMIB/HRAS.

Centro Clínico Sudoeste SALA 248
          ENDOPED

Fone 3361 1601
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