[CIS PIDD] [cis-pidd] 12 year old with frequently recurrent episodes of facial HSV-1

[Nacho Gonzalez]

High IgG4? Absent NKT cells?

Luis Ignacio Gonzalez-Granado
Immunodeficiencies. Pediatric Infectious Diseases
Pediatric Hematology & Oncology
Hospital 12 octubre
Madrid. Spain


2014-02-12 22:25 GMT+01:00 Kumar, Ashish <Ashish.Kumar at cchmc.org>:


> We have seen both DOCK8 and STAT3 mutant patients with normal IgE, so you

> can't go just by that. If the eosinophil count is high, DOCK8 becomes a

> prime suspect.

> Ashish

>

> Ashish Kumar

> Cincinnati Children's Hospital Medical Center

>

>

> -----Original Message-----

> From: michael clayton [mailto:mhclayton at msn.com]

> Sent: Wednesday, February 12, 2014 1:48 PM

> To: CIS-PIDD

> Subject: Re: [cis-pidd] 12 year old with frequently recurrent episodes of

> facial HSV-1

>

> Thanks to everyone for your suggestions.  The patient's IgE was 183.  No

> monocytopenia (absolute count is 700).  Normal lymphocyte response to

> Candida (as well as PHA, ConA, PWM).  I wasn't clear on Val dose- she is

> receiving 500 twice daily.  Will try pushing this higher/give more

> frequently.  Could this mild elevation in IgE be consistent with a DOCK 8

> mutation?

> Thanks,  Michael Clayton

>

> On Feb 11, 2014, at 2:54 PM, Michi wrote:

>

> > Strongly consider DOCK8!

> >

> > Von meinem iPhone gesendet

> >

> > Am 11.02.2014 um 21:49 schrieb michael clayton <mhclayton at msn.com>:

> >

> > Dear Colleagues:

> >

> > I wonder if you could offer advice on treatment of a 12 year old girl

> who has had frequently recurrent episodes of facial HSV-1 (culture

> confirmed) over the past 8 months.  She typically has an outbreak over her

> right cheek with a sense of tinglng, pain and warmth preceding the outbreak

> of vessicles.  No systemic symptoms.  No eczema or skin trauma.  Frequency

> is about every 10-14 days, and the longest interval between outbreaks has

> been 3 weeks.  She occasionally has an outbreak over lateral arm on same

> side and more rarely, over the lower arm.  She is developing chronic skin

> changes at these sites with thickening, mild hyperpigmentation and

> persistant stinging between outbreaks.  Stress and menstrual periods

> obviously make the episodes more severe, but starting an OCP has had no

> significant effect, nor did use of a high SPF sunblock daily over these

> areas.    The primary care provider previously had her use a daily regimen

> of acyclovir 400 mg BID with valacyclovir added in at onset of rash (500

> BID).  Topical antivirals were not helpful.  I changed her prophylactic

> regimen to daily valacyclovir, also with no benefit.

> >

> > Other health history:

> > -Very atopic girl with multiple food sensitivities, both type I and II

> as well as environmental sensitivities.

> > -Long standing (earliest childhood) history of potentially autoimmune

> > problems including alopecia, granuloma annulare, recurrent episodes of

> urticaria, intermittent joint swelling and pain with normal ANA, RF

> -Suspected seizure activity with "drop attacks" associated with intense

> exercise, and emotional outbursts that are sustained and suggestive of a

> partial complex sz. disorder, but normal EEG and negative cardiac workup.

> > -Obstructive sleep apnea.

> > -Asthma, step II well-controlled.

> > -Menorrhagia.

> > -Two outbreaks of varicella after receiving her varicella vaccine.

> >

> > Family History:

> > Younger brother with eczema and seizure disorder.  Mother has alopecia

> areata, Two maternal aunts have Crohn's disease, maternal grandmother has

> lupus.  Virtually all adult females on maternal side have hypothyroidism.

> > No known immune deficiencies, or problems specifically with herpes or

> zoster.

> >

> > Labs:

> > Nl CBC with diff

> > Immunoglobulins:  IgG 1230, IgA 138, IgM 229 (30-211) Tetanus IgG= 2.5

> > Pneumococcal serotypes= 8/13 protective range CH50= nl.

> > Immune deficiency panel (CD 16/56= 5%, absolute CD4= 1046, all others

> > normal, as well) Lymphocyte mitogen response panel normal NK

> > cytotoxicity profile at Mayo:  Diminished but not absent natural NK

> > cell cytotoxicity which was comparable to shipping control.  Normal NK

> cell number (86 cells/ul.) Negative Celiac Panel.

> > ANA, RF (-)

> >

> > I plan to repeat the NK cytotoxicity panel, but wonder if anyone has

> > other ideas regarding workup or treatment?  She is scheduled to see a

> > Pediatric ID consultant within the month, and is followed by a

> > Pediatric Rheumatologist without a diagnosis, thus far.  Thanks in

> > advance,

> >

> > Michael H. Clayton, MD, MPH

> > Albuquerque, NM

> > 505-228-7958

> > mhclayton at msn.com

> >

> >

> >

> >

> >

> >

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