Less than 10 year old male with no significant medical history admitted for fevers and weight loss.

The patient has had relapsing and recurring fevers since the age of two. The mother endorses that the
current episodes, the patient would present with multiple pustules on the skin, that would have pus,
drainage, and ulcerate causing wounds that heal with scarring. The areas that pustules occurred included
the scalp, bilateral cheeks, lips, and overlying joints including hands, elbows, and knees.

The active wound on the right knee was present for ~20 days, that began as a pustules and since grown
and worsened.

The patient also gets recurrent rhinorrhea around the nose for several years, usually resolving with
topical or oral antibiotics that completely goes away.

There is no intellectual disability per mother.

He is from Honduras.

Infectious Disease, Rheumatology, and Dermatology consulted

- ++ Moraxella cataharralis
- ++ MSSA on biopsy, + EBV
- CRP 4.2, ESR 47
- IgE elevated to 1,900, IgD to 29.6 (IgA, IgM, IgG wnl)
- NADPH oxidase activity
- SCID profilfe CD8 positive T cells elevated 1,400 (vs 1,100)
- Interleukin 18 serum elevated to 955
- Negative for HLA B51, blast, coccidio, leishmania, pneumococcal, ANCAS
- Normal granulocyte dihydrorhodamine fluorescence suggesting normal

Diagnosis: Hydroa Vacciniforme Lymphoproliferative disease

Hydroa vacciniforme is a rare photodermatosis of childhood, characterized by vesicular

lesions on sun-exposed skin that eventually heal, leaving depressed scars. The presence of
latent Epstein-Barr virus (EBV) in typical hydroa vacciniforme has been reported and may
be a contributing factor in the etiology.

Hydroa vacciniforme occurs primarily in the summer. Sunlight induces the lesions on
exposed areas of skin, most commonly the face and dorsal hands, within hours of sun
exposure. The eruption is symmetric, exhibiting erythematous macules that progress to
tender papules, vesicles, and crusts (picture 2A-B). Lesions are associated with pruritus or
a burning sensation.

In a case series of five patients, three were found to respond to photodesensitization with
NBUVB [32]. Other treatments include antimalarials, intermittent
corticosteroids, azathioprine, cyclosporine, and dietary fish oil [41-44]. There is a single
case report of a patient who responded to acyclovir and valacyclovir [45]. There is a report
of two adult cases with EBV-associated hydroa vacciniforme-like lym

PLAN:

EBV DNA from blood

Start Acyclovir

Oncology referral placed

ENT referral placed for nasal scope and potential biopsy

Photoprotection

NIH Referral

Immunology referral