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

[michael clayton]

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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