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SAMATHA MADHAVARAPU PGY-1. Case Conference. 6 m/o with rash on face. HPI. Worsening rash on the face since 3 days Rash initially on R cheek Spread to R eyelids and L side of face x 1 day Baby has been scratching it Rash was associated with some blisters
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SAMATHA MADHAVARAPU PGY-1 Case Conference
HPI • Worsening rash on the face since 3 days • Rash initially on R cheek • Spread to R eyelids and L side of face x 1 day • Baby has been scratching it • Rash was associated with some blisters • assoc with oozing, bleeding and scabbing • H/o fever + Tm 102 F
HPI Contd • Crying a lot • No URI symptoms/ GI symptoms • Good P.O • Normal urine output • Sibling has similar rash around her mouth • No daycare • No recent travel • No pets
PMH : h/o eczema + uses aquaphor • No surgeries, no hospitalizations • BHx: FTNSVD, Birth wt: 5lbs 8 oz . Pregnancy was uneventful • Imm: UTD All: NKDA • DHx : Enfamil 6 oz every 3-4 hrs • Devpt: Appropriate for age • FHx : no eczema, no asthma. • SHx : lives with mother, grandmother and 2y/o and 3y/o siblings
Physical Examination • VS: 98.9 F, HR: 132, RR: 32 , PO2 99% • HEENT: AFOF, PERRLA, conjunctivalerythema+ swollen R eyelidsOP: MMM, b/l TM n • Post occipital nodes + • RRR, S1+, S2+ no murmur • Lungs: CTA b/l • Abd: soft, NT, ND, BS+ • Ext: FROM x4, cap refill < 2 sec, pulses +2
Physical Examination • Skin: Impetiginous lesions, some scabbing, some vesicular and wet lesions over R cheek, R upper and lower eyelids, L upper eyelid. • Labs: • CBC : 11.4, 17/ 36.8/ 347/ N: 33.9/L: 53.4 • 3.9/ 64/ 2.5/ 39/ .
ECZEMA HERPETICUM • Kaposi varicelliform eruption • Refers to herpetic superinfection of pre-existing skin disease. • HSV 1, HSV 2, Coxsackie A 16, Vaccinia • In preexisting dermatoses • Most common: Disseminated HSV infection in pt with Atopic Dermatitis.
Pathophysiology • Disruption of stratum corneum sec to skin disease is most common predisposing factor. • Involves both cell mediated and humoral defects in persons with atopic dermatitis. • T cell mediated immunity to control primary and recurrent HSV • Antibodies against HSV limit the severity of infection • Reduced NK cell number and IL-2 receptors , a marker for lymphocyte activation
Pathophysiology • One study- skin in pts with AD is rich in IL- 4 which inhibits Th-1 cells and supress INF –gamma secretion • Cathelicidin is antimicrobial peptide , component of innate immune response. • Low levels of cathelicidin protein expression seen in eczema herpeticum pts • Inverse correlation between cathelicidin expression and serum Ig E levels in KVE
High levels of IgE increase the risk for EH • Corticosteroid Rx increases risk. Retrospective analysis of 100 cases of KVE -> 75% did not get it in 4 weeks before onset. • Some showed showed topical clacinuerin inhibitors, tacrolimus increased risk. • Immunosuppression • Increased incidence of KVE since 1980, due to increased HSV infections • Mortatilty from KVE decreased from 50% to <10% due to IV Rx
Effects men and women equally • Thought to be disorder of infants, can effect children of any age. • German study : 75 patients, age of onset 5 months to 69 yrs • Mean age of onset of AD was lower (5.6 yr) in pts with KVE compared with AD controls.
History • Begins as clusters of umbilicated vesiculopustules in areas of preexistent dermatitis. • They progress to punched out erosions, pathognomonic of KVE • Usually prediliction for upper body and head. • Vesicles become hemorrhagic and crusted • Erosions coalesce to large denuded areas, bleed and secondarily infected.
Often diagnosis is delayed • Eruption continues to spread over 7 -10 days and assoc with high temperature, malaise, and lymphadenopathy. • Primary episode of KVE runs its course and heals in 2-6 weeks.
Average duration of illness is 16 days • Transmitted by contact with an infected person or by dissemination of primary or recurrent herpes • Recurrent episodes may also occur, but milder and not assoc with systemic symptoms • Some studies showed a high frequency of HSV DNA in the oral cavity of pts with KVE • In severe cases, lesions heal with scarring
Other diseases assoc with KVE • Mycosis fungoides • Pityriasis rubra pilaris • Neurodermatitis • Irritant contact dermatitis • Congenital ecthyosiform erythroderma • Ichtyosis vulgaris • Rosacea • Benign familial pemphigus • Wiscott Aldrich Syndrome • Sezary syndrome • Seborrheic dermatitis • Skin grafts/Burns • Cow pox • Cutaneous T cell lymphomas
DD • Chicken pox • Contact dermatitis, allergic • Impetigo • VZ virus • Eczema vaccinatum
Labs • Viral Cultures of fresh vesicular fluid and DFA stain –most useful and reliable • Swabbing should be done vigorously as HSV is cell assoc and paucity of extracellular virus particles may be present • DFA staining as accurate as viral cx. Available in hrs. • Tzank smear: epithelial multinucleated giant cells and acantholysis • If lesions are atypical, equivocal, or old, consider PCR or biopsy.
Consult Ophthal: If eye involvement is suspect • Herpetic keratitis can cause scarring • Ocular herpetic infection in setting of KVE is rare.
Treatment • Nucleside analogs: Acyclovir • Acts by inhibiting viral DNA polymerase • Systemic / Topical antibiotics for secondary bacterial infections • Dose: 15mg/kg/d IV tid for 5 days or until lesions heal
Foscarnet: For immunocompromised host with HSV and acyclovir resistant HSV • Poor clinical response / persistant viral excretion indicates viral resistance • Vidarabine, Trifluridine , Valacyclovir • F/U care in 2 weeks to evaluate response to treatment
Complications • Systemic Viremia: Liver, lungs, brain, GIT adrenal glands • Septicemia from secondary bacterial infections of skin • Staph aureus, group A beta hemolytic strep, Pseudomonas and Peptostreptococcus • Ocular involvment: blepharitis, conjunctivitis, • Keratitis, uveitis. Blindness due to stromal scarring. • Very few ocular herpetic disease in KVE, even with + conjunctival HSV cx.