Visual Diagnosis: The ABCs of Rashes in Kids

Noon ConferenceP.S.: It’s not all about HIV.

Ann Petru, M.D.

Pediatric Infectious Diseases

October 23, 2009

Alphabetical List of Topics– Bullous dermatosis– Candidiasis, Cellulitis– Coccidioidomycosis– Ecthyma gangrenosa– Eczema herpeticum– Foot ulcer– Granuloma annulare– Henoch-Schoenlein purpura– Impetigo– Job’s syndrome– Kawasaki syndrome– Leishmaniasis– Lupus (Congenital, SLE)

– Meningococcemia, MRSA– Nephrotic syndrome– Neutropenia– Osteomyelitis– Peritonitis, Pott’s puffy tumor– Q-taneous larva migrans– Ritter Disease (SSSS)– STDs, Shingles, SJS, Sweet’s– Tinea,Toxoplasmosis, TB– Ulcerating hemangioma– Varicella– Warts, Yuck (pus)– Zebras……… and more.

Behcet’s disease•14 yo boy referred to ID clinic• 5 year hx of oral ulcers• Intermittent fevers• 6 wks of foot pain, redness, swelling• ESR 93, WBC 9.6• Occasional bloody stools • Weight loss• Presented 6 months later with acute chest pain and ECG changes.• Eventually diagnosed with coronary aneurysm/fistula and subendocardial myocardial infarction with severe MV disease.

Bullous dermatosis• 13 yo Tongan has recurrence of this

rash after 3 years in New Zealand• Rapid evolution• UCSF bx: Bullous pemphigoid or

Linear IgA bullous dermatosis• B.P. is usually men 60-80 yo• Linear IgA dz - rare, autoimmune,

linear deposits of IgA along basement membrane

• Pruritic, annual papules, vesicles and bullae in groups; predilection for extensor surfaces, symmetric

• Collarettes: blisters form as new lesions arise in periph. of old ones

• Subepidermal PMN infiltrates• Rx: Dapsone and steroids

Candidiasis• 3 mo old boy with diaper

rash: Candida albicans• Fussy, hard to console.

Seems to ‘burn’ with every wet diaper

• Characteristic ‘satellite’ lesions at edges

• ‘id’ reaction - severe inflammatory component

• Mgmt: Oral antifungal & topical steroids to control inflammatory reaction

• Response was excellent

Cellulitis of the chest wall• 14 mo with rapidly

progressing swelling

• Seen in ER, T 102

• WBC 18K (72 polys, 12 bands)

• ESR 48, CRP 6

• Blood culture sent

• Needle aspirate: pus obtained --> GS: GPC

• Most likely organism: Staphylococcus aureus

• Big concern in 2005? MRSA

* Empiric antibiotic choices?

Clinda vs Vanco vs TMP-SMX

+/- rifampin

Cellulitis of the neck, premie• Growing premature infant, was 2 weeks old and doing fairly

well, breathing on his own, until…

• He crashed: reintubated, cultured, antibiotics started

• Blood: Group B Streptococcus

• Rx amp/gent x 3 wks

• Recovered fully

• No meningitis!

• Remember: GBS may

recur (GI colonization)

Group B Strep cellulitis-adenitis syndrome

• Abrupt onset of fever, poor feeding, and irritability• Unilateral non-discrete facial or submandibular

swelling - erythematous and tender. • Bacteremia usually present, • GBS isolated from aspirate of cellulitis or LN• Ipsilateral otitis media is common. • Suppurative submandibular lymphadenitis caused by

S. aureus is distinguished by manifestation as a discrete mass and propensity for suppuration.

Coccidioidomycosis

• 12 year old boy from the Central Valley was referred to CHRCO for evaluation of this skin lesion

• Local dermatologist had done a biopsy, which grew Coccidioides immitis !

• Cutaneous ‘cocci’ had not been seen in our clinic before

• Treated with oral antifungal and had an excellent response

Coccidioidomycosis• Coccidioides immitis… dimorphic soil fungus. Spores are airborne and lead to disease.• Primary infection: respiratory• 60% asymptomatic or self-limited• Sympt.: flu-like, fever, cough, malaise, myalgia, headache and chest pain• Diffuse rash, EM, erythema nodosum, arthralgia • 5% may develop pulmonary scars• Extrapulmonary is rare, follows trauma, soft tissue infection• Dissemination is rare (<1%)• Dx: Culture (warn lab; danger with aerosol- ization of organism in lab); serology (UC Davis c/o Dr. Pappagianis).• Rx: Ampho B (severe) or itraconazole or fluconazole (oral)

Coccidioidomycosis: Erythema nodosum

Cutaneous coccidioidomycosis

• Another teenager • Central Valley• Chronic skin

problems• Biopsied by local

MD, sent to us.• Cx: + for cocci• Rx: Underway

with fluconazole

Ecthyma gangrenosa

14 yo boy with acute myelogenous leukemia (AML) for past 2 years. Prior pneumonia (scar) was due to Rhizopus infection (bx); AML now relapsed and out of control• E.G. => Painful, widespread lesions• His culture from one grew Fusarium species• Gram stain usually positive in cases with EG• Other bugs: Pseudomonas, Vibrio species

Eczema = Atopic Dermatitiswith secondary infection

• 11 yo with 2-3 week hx of itchy rash, started on right leg, spreading rapidly. Nontoxic. Some areas ooze purulent material. No prior dx of atopic dermatitis!

• Cx: Meth-sensitive Staph aureus• Dry, itchy, scratched areas become

darker & thickened (lichenified)• Predisposed to become infected• Commonly Staph aureus and Strep

Gp A• Rx includes aggressive skin care

with moisturizers, antihistamines, and potent antibiotics (IV for this).

Eczema herpeticum (HSV)12 month old boy with severe eczema

Thickened, rough skin despite creams

Itching all the time

Kissed by a loving relative with a cold sore - no thanks!

Rx: IV acyclovir, antihistamines, skin care

Guttate Psoriasis

• Excessive prolif. of keratinocytes

• Thickened, scaly plaques; itching

• Inflammatory changes of epidermis and dermis.

• Guttate psoriasis distinctive acute form in children and young adults

• Closely associated with preceding sore throat, tonsillitis, Strep infxn

Henoch-Schoenlein Purpura Also calledAnaphylactoid Purpura

Is this rash an infection?

5 month old boy awoke with swollen hands, feet and face.

Henoch-Schoenlein Purpura

• HSP is a vasculitis of small blood vessels• Cause is unknown, typically follows URI• Children > adults, males > females, 9/100,000.• Mostly 2-8 yo, winter months• Lab studies are nonspecific and not diagnostic• Acute onset sx, low-grade fever, fatigue, RASH• Main consequences: GI (colicky pain, blood in

stools, diarrhea), kidneys, and arthritis (edema).• Prognosis good; steroids for GI dz only.

Herpes Labialis with MSSA

Job’s syndrome (Hyper IgE syndrome)

• Rare disorder, autosomal dominant inheritance (var)

• Mucocutaneous candidiasis (yeast) early in life

• Recurrent infections (pneumonia, skin, eczema)

• High IgE (>2000 IU/ml)

• Eosinophilia

• Dental problems

• Scoliosis, fractures

Common pathogens (lungs, skin, abscesses): Haemophilus influenzae, Strep pneumoniae, Staph aureus

Job’s syndrome: Eczema and secondary Staphylococcal infections

• 6 yo boy went to Middle East with family for 2 mos• 3 lesions developed on arms, leg, spreading. Biopsy done. • An older man asked, “Could this be an oriental sore?”• Google: “oriental sore” = Leishmaniasis.• It’s all in the history (and modern technology)• Bite of infected sandfly, inoculates parasites (Leishmania)

Leishmaniasis

Leishmaniasis• 3 different clinical syndromes

– Cutaneous: sandfly inoculates parasites into skin, proliferate in local phagocytes -> nodule, ulcer with raised borders, face & extremities in exposed areas, local lymph nodes react. Spontaneous resolution, weeks to years, flat scar

– Mucocutaneous (espundia): from skin parasites disseminate to oral and nasopharyngeal mucosa, sometimes ulcerate, scars, mucosal perforation

– Visceral (kala-azar): Skin through mononuclear macrophages, concentrated in spleen, liver, bone marrow -> fever, anorexia, weight loss, HSM, enlarged lymph nodes, anemia, low WBC, low platelets, low albumin, and high Igs. Untreated: fatal.

Congenital (Neonatal) LupusBaby whose mother had SLE:• Due to placental transfer of IgG

autoantibodies between 12-16th week of gestation to baby

• Manifestations: congenital heart block, cutaneous lesions, liver disease, low platelets, low WBC, pulmonary and neurologic disease.

• Most manifestations resolve but congenital heart block requires pacemaker.

• This baby recovered gradually over a few months.

Rash mimics serious infections, such as meningococcemia.

Systemic lupus erythematosus• SLE is a rheumatic disease of unknown cause• Autoantibodies directed against self-antigens

Inflammatory damage to target organs– Kidneys, blood cells, central nervous system

• Diff Dx: FUO, arthralgias, anemia, nephritis• Dx: autoantibodies: antibodies to DNA, ribosomes,

platelets, coagulation factors, Ig’s, RBCs and WBCs• ANA often present but not specific. Anti-Smith + in SLE• Anti-double-stranded DNA more specific than ANA.• CH50, C3 and C4 are decreased in active disease• Dx: 4 of 11 criteria, usually ANA positive

Systemic lupus erythematosus

• 18 yo woman with multisystem disease and these painless symmetric vasculitic changes on fingers

Systemic lupus erythematosus

• Older teen with recent diagnosis of SLE

• Characteristic ‘malar’ rash associated with lupus

• Also had severe renal involvement

• Getting high doses of IV steroids

Meningococcemia

• Overwhelming bacterial infection caused by Neisseria meningitidis

• Sepsis, meningitis, or both• Onset abrupt with fever,

chills, malaise, prostration, and typical rash: “purpura” or “petechiae”

• Fulminant cases: purpura, “DIC”, shock, coma, and death within several hours.

Meningococcemia

Neisseria meningitidis• Can cause meningitis, pneumonia, occult bacteremia,

conjunctivitis, chronic meningococcemia.

• Invasive disease complications: arthritis, myocarditis, pericarditis, endophthalmitis

• 5 groups (A,B,C,Y,W135)

• Short incubation period (1-10 days, mostly < 4 d)• Gram stain, cultures from blood/CSF/skin scraping or joint,

buffy coat smear; antigen in CSF.• Detectable in CSF by PCR (30-50% + in UK)• Rx: Penicillin, cefotaxime, ceftriaxone, ampicillin• Short courses of treatment are adequate (5-7d)

Neisseria meningitidis meningitis and meningococcemia

• Isolation: droplet precautions until 24 hours Rx. • 4-valent vaccines available, not including group B.• Chemoproph with rifampin, ceftriaxone or cipro

– Prophylaxis for household contacts, childcare, nursery school contacts within 7 days, direct exposure to patient’s secretions, mouth-to-mouth resuscitation, sleeping/eating close to patient within 7 days of onset of symptoms, and in outbreaks.

Here’s an Easter Bunny in ICU

• This bunny was left on the bed of the patient with meningo-coccemia

• The boy recovered…

• The bunny didn’t!

Nephrotic syndrome: spontaneous bacterial peritonitis

• 10 yo girl with long hx of

nephrotic syndrome

(proteinuria, low albumin,

edema, hyperlipidemia)• Sunburn over bikini line ?• Came in with fever, abdominal pain, distension, redness• Each time her paracentesis cultures grew Strep

pneumoniae or E. coli; responded well to antibiotics.

Omphalitis: MRSA• 6 wo boy, 3-4 days of

fussiness, ‘colic’, then fever 102, pan-cultured

• WBC 2.7K… to CHO• Pale, mottled, lethargic• IV fluids, antibx for

‘compensated shock’.

ID consulted: “mass” in upper abdomen

Pressure over mass: volcanic eruption through umbilicus, caught with dad’s cell phone camera.

Further studies: extended into porta hepatis ! I&D done and patient recovered fully with 3 weeks IV abx.

Osteomyelitis, MRSA(Methicillin-resistant Staph aureus)

• 13 yo boy had infected toenail, spread up leg to shin, eventually to tibia: cultures grew MRSA

• Treated x 6 months with various IV antibiotics

• Infection flared every time we tried to stop antibiotics

• Boy faced amputation• UCSF Dr. Dyab saved his

leg!

Pseudoverrucous papules

Boy with suprapubic vesicostomyand recurrent UTIs

Girl with HIVand severediarrhea forseveral weeks

Pott’s Puffy Tumor

• 14 yo boy with swollen, tender forehead

• Typical of teenage boys

• Often no prior symptoms

• CT scan essential to dx

• Unrecognized sinus dz

• Extension into forehead

• CT: epidural abscess !

• Needs drainage!Common organisms: 50% are Strep pneumoniae or H. influenzae; others: viridans Strep, group A Strep, or Staph aureus

Cutaneous larva migrans• 12 month old child from Sri Lanka, nursed x 4 months• Then came to US on 12/15/04 with

adoptive parents• Poor weight gain x 3-4 mos• Possible abdom discomfort• 2 weeks prior parents noted red, raised bump near anus, now expanding in linear fashion laterally, then curled in loop• No visible ‘worms’ in stool

Cutaneous Larva Migrans

• Nematode larvae produce pruritic, reddish papules at site of skin entry “creeping eruption”

• As larvae move, leave intensely itchy serpinigous tracks (~pathognomonic)

• Eventually self-limiting (wks-months)

• Rare complic: severe pneumonia (Loeffler syndrome), myositis, eosinophilic enteritis.

Cutaneous Larva Migrans

• Cat and dog hookworms: Ancylostoma braziliense and Ancylostoma caninum.

• Contact with soil contaminated with dog, cat feces• US: Southeast.• Dx: Clinical; +/- eosinophilia; larvae in sputum

(pneu); serology not readily available• Rx: No Rx (self-limited) or albendazole,

ivermectin or topical thiabendazole

Staphylococcal Scalded Skin Syndrome (SSSS) - Ritter disease

• 20 month old, East Indian girl admitted 12 hours ago

• High fevers, irritable, intense erythroderma

• + Nikulsky, peeling around mouth, red eyes

• NP and skin from near rectum both: S. aureus

• Gradually recovered on Burow’s soaks, eucerin, vaseline or aquaphor, and pain meds, IV antibiotics

Staphylococcal scalded skin syndrome

• Toxin-mediated complication of a Staph infection (phage gp 2, strains 71, 55): exfoliative toxins A or B.• Mostly < 3 yo• First malaise, fever, irritability, exquisite tenderness of skin• Diffuse redness diffusely, worse in creases and around mouth• Eyes red, purulent, sticky• Skin wrinkles, blisters, erodes. Sheets peel off (+ Nikulsky sign).• Peels 2-5 days later, heals without scars. Quick recovery.

Sexually transmitted disease(s)?• 17 yo teen came to ER• Had 10 recent partners and

didn’t know names of any of them

• Ulcer is painless now• Had swollen, painful, red

nodes in groin• Refused HIV testing• GS: gram-neg diplococci• Cx: Group B Strep,• RPR: positive (syphilis)• Suspected: H. ducreyi too

H. ducreyi = chancroid, painful ulcer & painful nodes; rx: Azithromycin or cipro

GND: Ns. gonorrhea?

Shingles: “Herpes zoster”, caused by Varicella zoster virus (VZV)

• Previously well 6 yo came in with low-grade fevers and this blistering rash on L leg

• Often no prior history of primary chickenpox (typically unrecognized, kids had it in 1st 12 months)

• Typical dermatomal distribution (this is S1)

• Usually contagious only with contact with blister fluid.

Shingles: Varicella zoster

• 14 yo with perinatally acquired HIV/AIDS

• CD4 250 range and HIV viral load 60,000, on “HAART” Rx

• Hx 10 varicella 1997

• 2 days left neck pain

• Rash emerged over 12-24 hours

• C3 dermatome only

• Slowly responsive to acyclovir and gabapentin (Neurontin).

• Better with higher dose ACV

Stevens-Johnson Syndrome (SJS)• 12 yo with 3 prior episodes of

oral erythema multiforme (bx +), last one ‘aborted’ with steroids. No prior skin dz.

• Evidence of HSV, mycoplasma with high IgG and +++ IgM

• Now flu-like illness, fevers x 1 week, oral swelling, rash over chest, rapidly spreading

• Rash worsened, mouth swollen, progressed rapidly overnight

• Ended up in ICU

Erythema multiforme vs SJS?• EM: Numerous skin manifestations: macules, papules,

vesicles, bullae, urticaria (hives), plaques, confluent erythema (redness)

• Most common 10-30 yo, asymtomatic or with some burning, itching; extremitis more than torso, face.

• Characteristic lesion is doughnut-shaped, target-like papule with red border and inner pale ring and dusky-to-purple center (like an iris or bull’s eye).

• Abrupt onset.• Underlying causes, triggers may vary.• EM minor vs EM major (Stevens-Johnson syndrome)

Stevens-Johnson

Syndrome

Stevens-Johnson Syndrome (1)

• Red areas that rapidly for vesicles and bullae (blisters), and then denude

• Also involve 2 or more mucosal surfaces (eyes, oral cavity, upper airway or esophagus, GI tract or anogenital mucosa)

• Burning, edema, erythema of lips and mouth are often the presenting sign (and very painful).

• Rapid skin progression to bullae, ulcers, and hemorrhagic crusting (not so painful)

Stevens-Johnson Syndrome (2)

• Complications: corneal ulcers, uveitis, panophthalmitis, bronchitis, pneumonitis, myocarditis, hepatitis, enterocolitis, arthritis, hematuria, renal failure.

• Blood loss, fluid loss, bacterial superinfection• Crops of new lesions, scarring.• Labs: nonspecific: high WBC, ESR, LFTs, and

low albumin.• Causes: Mycoplasma, drugs (sulfas and NSAIDs).

Boy with SJS

Group A Streptococcus

• 14 yo boy with acute onset of fever, vomiting, diarrhea; better x 2 days, then fever, swelling left knee, right foot, left arm, wrists.

• BCx negative• ASO +++ (Gp A Strep)• Xrays: tenosynovitis of right

extensor digitorum tendon; erysipelas of left forearm, and prob osteo of right thumb!

• Rx: Clinda/oxacillin -> Keflex

Sweet’s syndrome:Febrile neutrophilic dermatosis

Sweet’s Syndrome

• Unusual hypersensitivity• Tender, red, edematous,

urticarial plaques or large papules

• Irregular edges (?vesicles)• Mimics HSV or strep

cellulitis, erysipelas if solo; mimics bites

• Fever, high WBC, malaise, arthralgias, myalgias, conjunctivitis, episcleritis

• Rare: airway, meningitis

• Pathology: marked infiltration of skin with neutrophils• Treatment: Steroids• Prognosis: Good with support

Tinea corporis (inflammatory ringworm)

• 8 yo East Indian child had been treated with Keflex and Clinda for suspected Staph impetigo

• Cx: Trichophyton tonsurans

• Rx: Griseofulvin• Outcome: CURED!

Tinea corporis (ringworm) Healing phase

Tinea capitis: Ringworm or Kerion?

• 3 year old boy with HIV…..• Teenage girl with scalp pain and severe hairloss

Toxoplasmosis (Toxoplasma gondii)

• Newborn had apnea, neuroultrasound and CT with Ca++ deposits

• Had small left eye (blind)• Developed progressive

hydrocephalus -> shunt • Toxoplasma gondii proven

by serology of baby, mom• Kitty litter changed by

mom thru pregnancy!• Bye, bye kitty!

Congenital toxoplasmosis• Mother acquires toxo during pregnancy or it reactivates

due to immunodeficiency (in HIV)• 70-90% of infants are asymptomatic at birth, but visual

impairment, learning disabilities, mental retardation often appear later

• Signs: Rash, enlarged lymph nodes, big liver, spleen, jaundice, low platelets

• Brain infection leads to abnormal CSF, hydrocephalus, small head, chorioretinitis (which leads to blindness and small eye), seizures, deafness.

• Calcifications can be seen on NUS and CT of head

Congenital toxoplasmosis

Congenital toxoplasmosis and perinatal HIV infection

Congenital toxoplasmosis

• Bug: Toxoplasma gondii, protozoan parasite• Diagnosis: Serology

– Special labs: IgM or IgA + in first 6 months• Toxo-specific IgM by double sandwich EIA or ISA is 75-80%

sensitive; Don’t trust IFA for IgM.

– IgG + beyond 12 months of age is confirmative– Test peripheral blood WBCs, CSF and amniotic fluid

for T. gondii by PCR in reference lab– Can attempt to isolate T gondii from placenta,

umbilical cord or blood by mouse inoculation

Tuberculosis of the skin ? Erythema induratum

Lower, inner left leg

PPD scarNewerlesion on arm

Original areas involved

15 yo Afghani girl with painful skin lesions x 12 mos

Tuberculosis of the hand bones & more• 16 yo East Indian girl presented with

massively swollen, tender left thumb, painful left foot and some left eye vision problems

• Left wrist draining pus intermittently• CXR: pleural thickening and infiltrate vs

old disease (scar); bone scan + @ 3 sites• PPD > 30mm induration!• Cx: thumb drainage, sputum + for

Mycobacterium tuberculosis sensitive to all TB meds

• Now doing very well x 9 mos on multidrug regimen

• Brother with TB, too.

Ulcerating Capillary Hemangioma• Treated by PMD with

hydrocortisone and bacitracin and cultured

• Grew mix of stool flora• Treated with mix of oral

antibiotics, telfa gauze, and referred to UCSF dermatology.

• Challenge: mechanical!• Eventually treated with

lasers and gradually improved.

• 6-8 week old born with ‘birthmark’ on cheek of her right buttock•Area ‘broke down’ over time

Varicella zoster virus (chickenpox)

• 3-4 week old infant whose mother had chickenpox when baby was 1-2 weeks old. Baby has sl. fever.

• Biggest risk is if mom has chickenpox in 5 days prior to or up to 2 days after birth (baby gets over-whelming disease, has no antibodies to VZV). One study

• This baby did well.

Varicella in boy on Remicade

• I’m taking Remicade for my Crohn’s dis.

Warts: Verruca vulgaris

• 6 yo girl with HIV • Stable on same regimen

since birth• Father sent this photo• Occlusive dressing and it

vanished!• If immune system is

strong, response is good

YUCK ! From Ortho, “Pus is us”

• 12-13 yo boy with 2 yr chronic leg pain and limp, increasing bullae on anterior thigh• Rx in Samoa with various antibiotics serially; came to CHRCO’s ortho clinic• Had ‘gross’ pus on exam• GS: gram-positive cocci• Cx: Staphylococcus aureus, resistant to oxacillin• Xray: Destroyed underlying femur• Rx: Debridement and VERY longterm course of antibiotics

Zebras

• Famous quote in medicine: “When you hear hoofbeats, think of horses, not zebras”

• ID Perspective: “If you don’t think of zebras, you’ll miss some very interesting diagnoses!”

References

• Bickle KM, Roark TR & Hsu S: Autoimmune Bullous Dermatosis: A Review, Am Fam Physician 2002:65:1861-70.

• Long’s Principles and Practice of Pediatric Infectious Diseases, 2nd Edit, 2003, Group B streptococcus cellulitis-adenitis syndrome, p 168.