[CIS PIDD] [cis-pidd] severe molluscum/ CMC

[Dr. Carsten Speckmann]

  Bamboo hair? (Netherton?)
Naive T cells, TCR repertoire, g/d T cells? (combined ID? - the 
undetectable IgM raises suspicion)
IgE is normal, but high levels somtimes just gradually evolve over time 
in AD HIES and can be normal in some young children.
Plt are normal and the patient is female (so not classic WAS), but on 
the end of your differential list you might want to add WIP deficiency

Dr. med. Carsten Speckmann
Funktionsoberarzt/Consultant Immunologist
Zentrum fuer Kinderheilkunde und Jugendmedizin
Centrum fuer Chronische Immundefizienz - CCI
Universitaet Freiburg
Mathildenstr. 1
79106 Freiburg
Germany

phone: +49 (0)761-270 43010
mail: carsten.speckmann at uniklinik-freiburg.de
web: www.cci.uniklinik-freiburg.de



Am 24.10.13 13:07, schrieb Blachy Davila-Saldana:

>

> Hello all;

>

> I'd to hear your thoughts regarding a patient. This is a 20 month old 

> female, only child to non-consanguineous parents, who has had severe 

> eczematous dermatitis since around 4 weeks of age, not responsive to 

> any treatment by dermatology. She has required a previous hospital 

> admission for wound consult management due to the severity, also had 

> several episodes of suprainfection with Staph species, mostly MSSA. 

> Additionally, she developed molluscum contagiosum which has become 

> widespread as well. She has a history of reactive airway disease and 

> frequent URI’s, but no documented pneumonias, abscesses or ear 

> infections. No severe thrush or fungal skin issues by history.

>

> On exam, she is 50^th percentile for both height and weight. The 

> dermatitis is severe and covers forearms, axillae, diaper area and 

> legs. Her molluscum covers her upper trunk and all extremities. She 

> has mild to moderate developmental delay. Even with her severe rash, I 

> only felt a couple small nodes on her occipital area, and none 

> elsewhere. She has dysplastic nails, mainly on her lower extremities. 

> Her hair is short and sparse.

>

> Labs:

>

> WBC 19.2, with ANC 5400, ALC 10000 and 1300 eos

>

> Hgb 13.8

>

> Plt 422

>

> CMP normal, including normal albumin (3.6), glucose (90) and calcium 

> (10.3)

>

> IgG 265

>

> IgA 65

>

> IgM <10

>

> IgE 28

>

> Lymphocyte subsets:

>

> - Normal total numbers of T cells and B cells

>

> - Slightly decreased NK cells (177.2 cells/ul)

>

> - Slightly increased polyclonal CD5 positive B cells

>

> - No monoclonal B cell population

>

> - Naïve B cells 89.2%

>

> - Non-switched, marginal zone like memory B cells 9%

>

> - swiched memory B cells 0.7%

>

> Mitogen proliferation studies:

>

> *Absent Lymphocyte responses to Candida ***

>

> Low-normal Lymphocyte responses to Tetanus

>

> Low-normal Lymphocyte responses to PHA.

>

> Normal Lymphocyte responses to Con A.

>

> Normal Lymphocyte responses to Pokeweed Mitogen.

>

> HIV non-reactive

>

> Antibodies:

>

> Tetanus 0.1 (0.1 considered protective in this lab)

>

> Diphteria 0.0

>

> HiB 0.1 (1 or above considered protective)

>

> She has been given another vaccine challenge and we will retest 

> titers. DOCK8 deficiency evaluation is in process.

>

> A fungal culture from her nails grew yeast, only identified as NOT C. 

> Albicans. She was placed on fluconazole as well as aldara by 

> dermatology, after a cantharone trial, but her molluscum continues to 

> spread and is worsening.  As next treatment, and before we knew these 

> results, she had two intralesional candida antigen injections, with no 

> improvement (and no skin reaction, at all).

>

> I know treatment of her skin will be challenging, but I was wondering 

> how others have treated severe molluscum in this setting. 

> Additionally, I wondered of further testing.  Would you proceed with 

> testing for CMC?  Should we consider screening further for 

> endocrinopathies, even though her labs are normal?

>

> Your thoughts are appreciated.

>

> Blachy

>

> Blachy J. Dávila Saldaña

>

> PGY-7

>

> Pediatric Hematology-Oncology Fellow

>

> Mail Code CDRCP

>

> 3181 SW Sam Jackson Park Road

>

> Portland OR 97231

>

> ---

>

> The CIS-PIDD listserv is supported by:

>

>

> /The science & practice of human immunology/

>

> P: +1.414.224.8095

> E: info at clinimmsoc.org

>

> Not a member of CIS? Please visit www.clinimmsoc.org 

> <https://cis.execinc.com/edibo/Signup> to join!

>

> You are currently subscribed to cis-pidd as: 

> carsten.speckmann at uniklinik-freiburg.de 

> <mailto:carsten.speckmann at uniklinik-freiburg.de>.

> To unsubscribe click here: 

> http://lm.clinimmsoc.org/u?id=183824763.5f364cefbfb582ee981d7d49949cd0d8&n=T&l=cis-pidd&o=44009927 

> <http://lm.clinimmsoc.org/u?id=183824763.5f364cefbfb582ee981d7d49949cd0d8&n=T&l=cis-pidd&o=44009927>

>


---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=44009974
or send a blank email to leave-44009974-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20131024/880a4f04/attachment.html>