[CIS PIDD] [cis-pidd] Hyper-IgD?

[Verbsky, James]

I agree with MVK analysis.  His platelet count and WBC are high which would represent hyper-IgD or other autoinflammatory syndrome.

Can you tell us more about the rash??  Evanescent?  Arthritis? Inflammatory markers?  Systemic JIA is possible..should have fever but child is fairly young.  The skin rash is unusual...shouldn't be constant in fever syndromes or SOJIA.  If it occurs off the face a biopsy might help.

I wouldn't give thalidomide.  If you are thinking a fever syndrome steroids, anakinra, colchicine are better empiric therapy...but I would still want to know more about symptoms and the rash

Ive seen high IgD (up to 900) in kids that are hypergam...not sure what this means.  Your patient IgG looks pretty good so not sure how to interpret that.

Best

James


James W. Verbsky M.D./Ph.D.
Associate Professor of Pediatrics and Microbiology
Medical College of Wisconsin
Milwaukee, WI
414-266-6701



From: Aristoteles Alvarez Cardona [mailto:dr.aristoteles at yahoo.com.mx]
Sent: Monday, April 29, 2013 2:09 PM
To: CIS-PIDD
Subject: [cis-pidd] Hyper-IgD?


Dear All

I would like to know your opinion on the following patient.

Male 10 months old, only child from non consaguineous parents.

Born from the first pregnancy at term LBW, with breastfeeding until now.

At 9 months of age he had non complicated viral wheezing and from 10 months of age he developed persistant erythematous papules on face initially treated as chickenpox, without recurrent fever episodes.

He was sent to me because persistant upper respiratory tract symptoms (runny nose and wheeze)

At examination the only finding were the aforementioned papules.

Initial lab: Hb 12.7 Leukocytes 14600 cells/mm3 Neutrophils 4150 Lymphocytes 8650 Eosinophils 630 (4.6%) Platelets 707,000
IgG 739mg/dl IgM 43 IgA 23 IgD 739mg/dl IgE 1022 IU/ml

We indicated milk, egg and soy free diet along with breastfeeding, ICS/SABA, asked for mevalonate kinase deficiency test and immunocap.

Now, is this IgD level somehow related to the high IgE levels or is truly a HIDs that has not yet developed other symptoms but skin ones?

It is indicated to order a skin biopsy?

Should I initiate thalidomide?

Thanks on advance


Aristóteles Álvarez Cardona MD.
Clínica de Inmunología. Alergia. Pediatría
Inmunodeficiencias Primarias

Universidad Autónoma de Aguascalientes
Alumno. Maestría en Investigación Biomédica.
Avenida Universidad 940
Ciudad Universitaria
Edificio 202 Laboratorio 2
Aguascalientes, Ags. México CP 20131.
www.uaa.mx<http://www.uaa.mx>





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