[CIS PIDD] [cis-pidd] recurrent/chronic HSV-2

[CIS-PIDD]

Hi all,

I wanted to get your opinion on a difficult case of recurrent/chronic HSV-2.

HPI:  4yo girl who was the product of a di-di twin gestation complicated by intrauterine HSV-2 infection resulting in chronic HSV-2 infection and profound neurologic sequelae in both twins.  The twins were born to a 30-year-old G6P2 female at 30 weeks gestation. Prenatal course was notable for a fetal ultrasound at 3 months revealing anencephaly in twin B.  At day 3 of life, twin A developed vesicular skin lesions on the chest and thigh.  PCR testing of skin lesions, CSF, conjunctiva, and oral mucosa were positive for HSV-2.  Twin A was treated with parenteral acyclovir for 21 days and oral acyclovir prophylaxis for 6 months.  Subsequent trials off acyclovir on three separate occasions resulted in recurrent vesicular skin lesions within 1-2 days.  Twin A has epilepsy, mental retardation and lost an eye due to herpes keratitis.

Labs:  Increased NK cells (abs 739) and markedly increased production of TNF-α and IL-6 following stimulation with poly (I:C) (TLR-3 ligand) (We sent the TLR panel through ARUP twice).  Genetic testing for mutations in STAT1, TBK1, TICAM1, TLR3, TRAF3, TYK2, and UNC93B1 were negative.

We were surprised, in particular, by the TLR3 pathway stimulation results.  Contrary to what you would expect see in patients with TLR3 pathway defects resulting in recurrent herpes encephalitis caused by HSV-1, she seems to have exaggerated innate immune responses following stimulation poly I:C.  I found an interesting article that posits that these cytokines alter cellular barrier function, resulting in increased viral penetration and dissemination of chronic HSV-2.

https://www.ncbi.nlm.nih.gov/pubmed/21325490

Question:
- Any ideas on how to definitively treat the HSV-2 and get our patient off lifelong acyclovir?
- Steroids are used in some instances to reduce edema in encephalitis, while timing likely matters, could restoration of barrier function explain the improvement seen in those treated with both steroids and acyclovir?

Ben

Benjamin L. Wright, MD | Assistant Professor | Allergy, Asthma & Clinical Immunology
Office Tel: 480.301.4284<tel:480.301.4284> | Fax: 480.301.9066<tel:480.301.9066>| Pager 127 or (79)1-5302 | wright.benjamin at mayo.edu<mailto:wright.benjamin at mayo.edu>
Mayo Clinic | 13400 East Shea Boulevard | Scottsdale, AZ 85259



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