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

[Freeman, Alexandra (NIH/NIAID) [E]]

I agree this could be DOCK8 deficiency, as well as some of the other
diseases mentioned. In the setting of DOCK8 deficiency,  when molluscum
are too widespread to curettage, I find them really difficult to control.
The topical steroids used for the eczema lead to worsening molluscum, and
then the molluscum lead to more itching and worsened eczema/spread of
molluscum, so it is a bad cycle.  I have used IFNa in a few individuals
with DOCK8 deficiency, and have had more success with HPV than with
Molluscum, but I have also had tolerance issues limiting this therapy. In
2 patients, the IFNa seemed to worsen the eczema making us stop therapy
and in another in whom I used for molluscum, cytopenias and depression led
to a stop. I have also used topical cidofovir for molluscum, but that can
cause burning and skin sloughing, and I would be really cautious in this
age group.  When it it was available for compassionate use, I used the
oral formulation of cidofovir once for severe molluscum and HPV, but this
was limited by increasing LFTs and seemed to improve the HPV more than the
molluscum.  At least in the setting of DOCK8 deficiency, I have found
transplant to be the best method to improve molluscum.
Alexandra
-----
Alexandra Freeman MD
Laboratory of Clinical Infectious Diseases
NIAID, NIH
NIH, Bldg 10,Room 12C103
9000 Rockville Pike
Bethesda, MD  20892
Phone 301-594-9045
Fax 301-496-0773
freemaal at mail.nih.gov

Notice: This communication may contain privileged or other confidential
information.
If you are not the intended recipient or believe that you have received
this communication in error,
please do not print, copy, retransmit, disseminate or otherwise use the
information.
Please indicate to the sender that you have this e-mail in error and
delete the copy you received.





On 10/24/13 10:28 AM, "Sokolic, Robert (NIH/NHGRI) [E]"
<sokolicr at mail.nih.gov> wrote:


>My best luck with molluscum in WAS has been with destructive methods ­

>curretage or silver nitrate. Might be difficult depending on extent of

>lesions.

>

>

>--

>

>This e-mail and any attachments thereto may be of a personal,

>confidential,

>or medical nature.  Under the Privacy Act of 1974, all data of a private

>nature must be protected from unauthorized disclosure. If you are not the

>intended recipient(s) of this message, or an employee or agent of the

>intended recipient(s), please note that any dissemination, distribution

>or copying of this message is strictly prohibited.  If you have received

>this e-mail in error, please delete the document and notify the sender of

>this error.  This e-mail, any attachments, and any response thereto

>should not be considered a secure, confidential or private means of

>communication.  The opinions and statements herein are those of the

>author, and do not necessarily represent official policy of the National

>Institutes of Health or any other part of the Government of the United

>States of America.

>

>

>

>

>From: Blachy Davila-Saldana <davilasa at ohsu.edu<mailto:davilasa at ohsu.edu>>

>Reply-To: CIS-PIDD

><cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>

>Date: Thursday, October 24, 2013 7:07 AM

>To: CIS-PIDD 

><cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>

>Subject: [cis-pidd] severe molluscum/ CMC

>

>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 URI¹s, but no documented pneumonias, abscesses or ear

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

>

>On exam, she is 50th 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:

>

>[http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]

>The science & practice of human immunology

>

>P: +1.414.224.8095

>E: info at clinimmsoc.org<mailto: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:

>sokolicr at mail.nih.gov<mailto:sokolicr at mail.nih.gov>.

>To unsubscribe click here:

>http://lm.clinimmsoc.org/u?id=183824760.3ff55aacd8c930275f65c3a15acc4c67&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: freemaal at mail.nih.gov.

>To unsubscribe click here:

>http://lm.clinimmsoc.org/u?id=183824471.c8fc49bf85f51eb5151a5682ab3d4de9&n

>=T&l=cis-pidd&o=44010404

>or send a blank email to

>leave-44010404-183824471.c8fc49bf85f51eb5151a5682ab3d4de9 at lists.clinimmsoc

>.org



---
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=44010760
or send a blank email to leave-44010760-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org