Dermatologic Emergencies Topics Epidemiology Rash Red Flags

4/8/2014
Topics
Dermatologic Emergencies
Jennifer McCarthy, MD James Burhop, DO
PEM 2nd Year Fellows
The Children’s Hospital of the King’s Daughters
Epidemiology
• Dermatologic complaints account for 5‐30% of pediatric ED visits
• Majority are not medical emergencies
• A subset of illness with significant morbidity/mortality will present with prominent skin manifestations
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Stevens‐Johnson Syndrome
Toxic Epidermal Necrolysis
Staphylococcal Scalded Skin Syndrome
Necrotizing Fasciitis
Omphalitis
Kawasaki Disease
Henoch‐Schönlein Purpura
Idiopathic Thrombocytopenic Purpura
Meningococcemia
Rocky Mountain Spotted Fever
Rash Red Flags
• Associated with mucous membrane involvement
• Extensive blisters/peeling of the skin
• Erythroderma with fever
• Pain out of proportion to physical exam
• Rashes associated with AMS
• Petechial/purpuric lesions
ED Case
• 12 y.o. male • 1 week h/o URI symptoms/malaise
• Tx 4 days ago with polytrim drops for bilateral conjunctivitis
• 2 day h/o fever
• Now with cracked erythematous lips, mucosal lesions, decreased po intake
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4/8/2014
Steven‐Johnson Syndrome
(SJS)
• Background:
‐ 1922: Stevens and Johnson noted condition of mucosal changes associated with characteristic target rash of erythema multiforme
‐ EM, SJS, and TEN often thought of as a spectrum of disease
• Epidemiology:
‐ Majority of cases occur in late childhood‐young adulthood (mean age of 12 y.o.)
‐ Slight male predominance
‐ Incidence of both SJS/TEN: 1‐3/million/year
Steven‐Johnson Syndrome
(SJS)
• Pathophysiology: ‐
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Not well understood
Presumed acute hypersensitivity reaction to infection or drug exposure
HSV implicated as possible viral precipitant: based on study of pt’s with recurrent EM
Mycoplasma pneumoniae: most commonly reported infectious trigger
Increased risk seen in HIV patients
Seen associated with immunization administration
30‐50% have symptoms associated with a recent URI
Most common precipitant: drug introduced typically in past 7‐21 days
Notorious Drugs
• Antibiotics: PCN, sulfonamides, cephalosporins
• Anticonvulsants: phenobarbital, phenytoin, carbamazepine • NSAIDs
• Allupurinol
• Anti‐retrovirals
Steven‐Johnson Syndrome
(SJS)
• Presentation:
General: ‐ Majority present with a prodrome of flu‐like symptoms (1‐2 weeks prior to skin findings)
Mucosal Findings: ‐ Mucositis develops shortly after febrile response noted with prodrome symptoms
‐ Typically occur 1‐2 days prior to cutaneous findings
‐ Burning sensation/pain noted at site of mucous membrane involvement early in disease course
‐ Lips erythematous/edematous
‐ Friable bullae/ulcerations often with associated hemorrhagic fluid noted on mucosal surfaces
‐ Hemorrhagic crusts may extend over lips to involve palate, esophagus, larynx
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4/8/2014
Steven‐Johnson Syndrome
(SJS)
Ocular Findings:
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50% of patients have eye involvement: lid edema, injection of bulbar conjunctiva +/‐ conjunctival blisters, corneal blisters, keratitis, uveitis
Severe purulent conjunctivitis with photophobia
Ocular sequelae noted in up to 40% Steven‐Johnson Syndrome
(SJS)
Cutaneous Findings
‐ May precede, follow, or occur simultaneously with mucosal changes
‐ Significant variation in morphology/distribution between patients
‐ Hallmark finding: plaques with dusky centers (target and iris lesions)
‐ Early Lesions often mistaken for hives, occur in crops ‐ Early skin lesions progress to form annular plaques followed by widespread blister formation/desquamation ‐ + Nikolsky’s sign
‐ Non‐erythematous skin remains intact, affected skin often detaches leaving a raw denuded base
‐ Typically face, trunk, and extensor surfaces of UE are involved first, followed by involvement of legs, dorsa of hands, and feet ‐ Perineum and scalp are typically spared
Nikolsky Sign
Steven‐Johnson Syndrome
(SJS)
Steven‐Johnson Syndrome
(SJS)
• Diagnosis: • Work‐up:
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Clinical
Presence of target and iris lesions
Inflammation of at least 2 mucous membranes with oral mucosa universally involved
Characterized by skin involvement of < 10% of total body surface area
Recommend cultures of blood, and skin
• Clinical Course:
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Mild SJS 5‐15 days but symptoms often persist up to 6 weeks
• Differential Diagnosis:
‐ Presentation with prominent skin blistering: pemphigus, pemphigoid,
‐ Presentation with prominent mucous membrane involvement: lichen planus, aphthous stomatitis, Kawasaki Dx
‐ Presence of Nikolsky sign: SSSS
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4/8/2014
Steven‐Johnson Syndrome
(SJS)
Steven‐Johnson Syndrome
(SJS)
• Management:
• Corticosteroids: ‐
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‐ Several retrospective reviews show they do not shorten disease course and do produce more medical complications ‐ If used should be started early and limited to < 1 week course
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Hospitalize all for observation/supportive care
Supportive care similar to that for burn patients (IVF, skin care/ nonadherent moist dressings, pain control, nutritional support)
Mouth care
Eye care/Ophtho consult if eye involvement noted
Prophylactic Abx NOT recommended in absence of signs of secondary infection/bacteremia
Antibiotics for suspected mycoplasma: have not been shown to alter outcome
Stopping the suspected inciting drug is recommended but there is no data that it alters the clinical course
Steven‐Johnson Syndrome
(SJS)
• Morbidity/Mortality:
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High dehydration risk with extensive mucosal involvement/epidermal detachment
Bacterial superinfection +/‐ sepsis
Impaired temperature regulation
Electrolyte imbalance
Impaired immune function
Rare development of esophageal, urethral, vaginal, anal strictures
End organ failure 1‐5% mortality with SJS, 10‐15% for SJS‐TEN transition (10‐30% of TBSA involved)
• Sequelae:
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Blindness in up to 10% presenting with eye involvement
Cutaneous Dyschromia: most common sequelae Nail dystrophy
Cutaneous scarring is rare in absence of secondary infection
• IVIG:
‐ Not recommended due to lack of data
‐ Currently being investigated
Toxic Epidermal Necrolysis
(TEN)
• Background:
‐ Often considered a variation/escalation of SJS where > 30% of total body surface area is involved
• Epidemiology:
‐ Majority of cases occur in adults (mean age of 45 y.o.)
‐ Slight female predominance
• Pathophysiology:
‐ Unclear but thought to be similar to that of SJS
‐ Drug exposure (same as listed for SJS) are the most common causes
Toxic Epidermal Necrolysis
(TEN)
• Presentation:
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Characterized by prominent blistering of epidermal sheets (> 30% of TBSA)
Often with extensive painful erythroderma
Less mucosal involvement then noted with SJS
+ Nikolsy’s sign
• Management:
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Similar to SJS: supportive care/burn care
Case reports suggest that IVIG may shorten course and decrease morbidity in patients with signs of organ dysfunction (particularly with eye involvement)
• Mortality:
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30‐35%
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4/8/2014
ED Case
• 6 day old FT male
• 2 day h/o erythematous sun‐burn like lesions over face and spreading to the neck
• Irritable, worse when being held
• No medication exposures
• No mucosal lesions
Staph Scalded Skin Syndrome
(SSSS)
• Epidemiology:
‐ Occurs mostly in younger children (< 5y.o.): due to lack of antibodies against the organism and inability to metabolize and excrete the toxin
• Pathophysiology:
‐ Toxin mediated systemic manifestation of a localized S. aureus infection
‐ Exfoliative toxins A&B
‐ Typical staph infection sites: nasopharynx, umbilicus, urinary tract, blood
‐ Cleavage plane for the associated skin sloughing is epidermal vs dermal involvement in TEN
Staph Scalded Skin Syndrome
(SSSS)
• Presentation:
General:
‐ Often don’t want to be held/comforted: irritable when touched
‐ Fever/malaise
Skin:
‐ Sun‐burn appearance worst around neck, intertriginous areas, and periorificially, followed by bullae formation and desquamation
‐ Tender blistering/denudation of skin (within 2 days)
‐ + Nikolsky Sign
Ocular:
‐ Purulent eye discharge: no conjunctival injection
Mucosal:
‐ No mucous membrane involvement
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4/8/2014
Staph Scalded Skin Syndrome
(SSSS)
• Diagnosis:
Clinical: ‐ Pt with sudden onset of fever, irritability, cutaneous tenderness, and diffuse erythematous rash ‐ And + Nikolsky sign ‐ Without mucosal findings
Biopsy:
‐ Cleavage of stratum granulosum layer of epidermis vs dermal/epidermal separation seen in TEN
Staph Scalded Skin Syndrome
(SSSS)
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Differential Diagnosis:
TEN
Graft vs host reaction
Radiation
Aspergillosis
Lymphomas
Differentiating TEN and SSSS
TEN
SSSS
• Occurs in both kids and adults
• Rapid progression
• Intraoral involvement
• More toxic appearing
• Dermal‐epidermal separation on biopsy
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Staph Scalded Skin Syndrome
(SSSS)
• Work‐up:
‐ Blood Cx: typically negative
‐ Wound/vesicle/ bullae Cx: typically negative
‐ Cx of exfoliating skin, umbilicus, throat, eyes, ears nose, or rectum: occasionally + for staph
‐ BMP: monitor electrolytes
• Clinical Course:
‐ Typically self‐limited
‐ Resolution of skin findings within 2 weeks
Occurs mostly in kids
Slower progression
Mucosa not involved
Less toxic
Cleavage of epidermis (stratum granulosum) on biopsy
Staph Scalded Skin Syndrome
(SSSS)
• Management:
Abx: ‐ Probably shorten the course
‐ Recommend IV Anti‐staph coverage with cefazolin or naficillin/oxacillin for pts < 12mos old or toxic appearing
‐ Substitute Vancomycin if MRSA is a strong consideration
‐ Add IV clindamycin to decrease exfoliative toxin production in ill‐
appearing pts
‐ Consider oral Abx in older children depending on severity of presentation (dicloxacillin/1st /2nd gen cephalo vs bactrim/doxy/clinda if MRSA prevalence is high)
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4/8/2014
Staph Scalded Skin Syndrome
(SSSS)
Steroids: ‐ No proven benefit/may exacerbate dermatitis and increase ability of organism to proliferate
Skin Care: ‐ Silvadene to denuded areas
Staph Scalded Skin Syndrome
(SSSS)
• Morbidity/Mortality:
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Superinfection
Dehydration/electrolyte imbalance
Bacteremia is rare
4% mortality in kids, adults 60%
Supportive: • Sequelae:
‐ IVF, electrolyte management
‐ Admission: those < 12 mos old, toxic appearing, or with significant skin involvement
‐ None: skin heals without scarring
ED Case
• 9 y.o. female transferred from OSH with CC of 5% TBSA burns that occurred 3 days ago, now with signs of infection
• Pt with some malaise, N/V
• ? Myalgias/stiffness
• Fever
• Upon arrival pt is tachycardic with widened pulse pressures Staphylococcal Toxic Shock Syndrome
(TSS)
Staphylococcal Toxic Shock Syndrome
(TSS)
• Pathophysiology:
• Pathophysiology Cont. :
‐ Caused by toxin producing strains of staph aureus
‐ Clinical manifestations secondary to TSST‐1 and staph enterotoxins (SEA,SEB,SEC) ‐ Toxins act as superantigens
cytokine release capillary leak hypotension and organ failure
‐ 50% now non‐menstrual type
‐ Can occur in males and females without an obvious source of infection
‐ Risk factors: surgical post‐partum wound, mastitis, sinusitis, osteomyelitis, burns, peritonsillar abscess, empyema, cellulitis, upper airway infection (bacterial tracheitis)
‐ Pts with TSS (especially menstrual type) are at risk of recurrence
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4/8/2014
Staphylococcal Toxic Shock Syndrome
(TSS)
• Presentation:
General:
‐ Acute febrile illness characterized by fever (typically > 39), rash, hypotension (< 5th %), and multisystem organ involvement
‐ Often have prodrome: malaise, myalgias, chills that progresses to fever, lethargy, diarrhea, AMS
‐ Edema of hands/feet/face
Cutaneous:
‐ Diffuse macular erythroderma with eventual desquamation 1‐2 weeks later
‐ Rash accentuations in skin folds
‐ Rash typically begins on trunk and spreads to UE and LE
Diagnosis of Staph TSS
Staphylococcal Toxic Shock Syndrome
(TSS)
HEENT:
‐ Hyperemia of mucous membranes
‐ Pharyngitis
‐ Strawberry tongue
‐ Palatal petechiae
Staphylococcal Toxic Shock Syndrome
(TSS)
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Differential Diagnosis:
Kawasaki Dx
Scarlet fever
Drug reaction
RMSF
Staphylococcal Toxic Shock Syndrome
(TSS)
• Work‐up
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CBC: leukocytosis with left shift, platelets < 100
CMP: LFTs and Cr: > 2x normal
CPK: > 2x normal
Coags: increased
Blood Cx: will be negative
Wound Cx: in 80‐90% of cases staph will be isolated from wound site
Urine, stool, throat cultures:, +/‐ LP to ID source
U/A: sterile pyuria
• Clinical Course
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Get early onset of oliguria
Hypotensive shock w/in 48 hours
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4/8/2014
Staphylococcal Toxic Shock Syndrome
(TSS)
• Management:
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Supportive care: extensive IVF (10‐20 liters/day), +/‐ pressors
‐ Abx: 2‐fold goal:
1. Kill the offending organism: Beta‐lactamase resistant anti‐staph agent (oxacillin/naficillin) or Vancomycin (in communities with high prevalence of MRSA)
2. Stop toxin production: Clindamycin
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IVIG: may be considered for infection refractory to several hours of aggressive therapy, or in presence of undrainable focus, or persistent oliguria with pulmonary edema
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Surgical: Aggressive drainage/irrigation of sites of purulent infection as soon as possible
Streptococcal TSS
Staphylococcal Toxic Shock Syndrome
(TSS)
• Morbidity/Mortality:
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Refractory shock
DIC
ARDS
Renal Failure
Mortality: kids 3% vs 30‐80% in adults
Outcome depends on prompt initiation of therapy
Diagnosis of Strep TSS
• Epidemiology:
‐ Association with recent varicella infection
• Pathophysiology:
‐ Differs from Staph TSS in that effects are mediated by strep pyrogenic exotoxins • Presentation:
‐ Most patients present with localized pain to an extremity out of proportion to findings on PE
‐ Influenza‐like prodrome in 20%
Streptococcal TSS
• W/U:
‐ Same as with Staph TSS: blood Cx should turn positive for strep • Management:
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Supportive: IVF, +/‐ pressors
IV Abx: initially same as Staph TSS Once strep source is identified, switch to PCN and clindamycin
Continue IV Abx until pt afebrile, hemodynamically stable, and blood Cx negative
‐ Early surgical drainage/irrigation of sites of infection
‐ Early surgical debridement if necrotizing fasciitis present
‐ IVIG: same indications as with staph TSS
ED Case
• 16 y.o. male
• H/O minor trauma to right knee 2 days ago resulting in a small superficial abrasion
• Pt now with 1 day of worsening right knee pain and limited ROM
• Febrile/tachycardic
• PE: Knee warm, small effusion, limited ROM
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4/8/2014
Necrotizing Fasciitis
• Background: ‐ Most cases prior to varicella vaccine were secondary to complications of varicella
• Pathophysiology:
‐ Deep soft tissue infection ‐ Differs from cellulitis in that it involves the fascia/muscle tissues in addition to the skin/subcutaneous tissues
‐ It does not always extend to the bones/joints
‐ Pt develops a local inflammatory response at site of infection (similar to that with cellulitis)
‐ Most commonly secondary to GAS
Necrotizing Fasciitis
• Presentation:
General:
‐ Fever (often > 39 C)
‐ Tachycardic/toxic appearing
‐ Pain out of proportion to exam
Skin:
‐ Erythema, edema, pain, warmth, limited ROM at site of infection ‐ Rapidly progressing induration/erythema
‐ +/‐ varicelliform lesions
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4/8/2014
Necrotizing Fasciitis
• Work‐up:
‐ CBC: marked leukocytosis
‐ Blood Cx: often grows responsible organism
‐ Surgical biopsy
• Management:
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Supportive Care: IVF
Aggressive irrigation/drainage of accessible sites of infection
IV Abx: PCN G and clindamycin +/‐ vancomycin in areas with high MRSA prevalence
Emergent surgical consult for debridement and biopsy
+/‐ repeated resections of necrotic tissue
+/‐ IVIG if toxic appearing
ED Case
• 2 y.o. male with 5 day h/o fever
• Irritable/Inconsolable
• PE: bilateral conjunctivitis (no drainage), cracked lips, strawberry tongue
• Erythematous macular rash, worse in groin
• Swelling of hands/feet
Kawasaki Disease
(KD)
• Background:
‐ First described in Japan: endemic occurrence with superimposed epidemic outbreaks
‐ Similar pattern of disease recognized in North America
‐ Missed diagnoses of KD among the most common malpractice verdicts against providers in US
• Epidemiology:
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Kawasaki Disease
(KD)
• Pathophysiology:
Typically presents in < 5y.o. (almost all < 12y.o.) with peak at 2y.o.
Younger patients are more likely to have atypical presentations
Higher incidence in boys
More cases in winter/early spring
Siblings of infected pt have increased risk of KD from that of general population
Kawasaki Disease
(KD)
• Presentation:
‐ Vasculitis of small‐medium sized arteries with predilection for the coronary vessels
‐ Etiology unclear: no evidence for person to person spread or common source spread
‐ Incubation period unknown
‐ Acute phase: 7‐14 days, subacute phase 2‐4 weeks
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4/8/2014
Kawasaki Disease
(KD)
Conjunctival Injection with Perilimbal Sparing
Mucosal Changes
Extremity Changes
Desquamation: Late Finding
Perineal Accentuation of Rash
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4/8/2014
Kawasaki Disease
(KD)
• Differential Diagnosis:
‐ Adenovirus
‐ Other Viruses: parvovirus, enterovirus, measles, EBV
‐ Bacterial Dx: TSS, RMSF
‐ SJS/Drug reaction
‐ Leptospoirosis
‐ JIA
Kawasaki Disease
(KD)
• W/U:
‐ No confirmatory diagnostic testing
Lab Trends:
‐ Increased ESR > 40/CRP > 3
‐ Platelets > 450,000 in 1st 2 weeks
‐ Sterile pyuria due to urethritis in 70%
‐ Mild hepatic dysfunction in 40%
‐ CSF pleocytosis: 25%
‐ GB hydrops < 10%
‐ Elevated GGT in 70%
Kawasaki Disease
(KD)
Kawasaki Disease
(KD)
• Clinical Course:
• Management:
‐ If no treatment: fever for 2 weeks, irritability for additional 2‐3 weeks after fever resolution
‐ Desquamation occurs after the fever resolves
‐ Recurrence of disease in 2%
Treatment goals: • Decrease inflammation • Antiplatelet therapy ‐ IVIG (2g/kg x 1) , can repeat dose in 24‐48 hours if fever persists
‐ High dose ASA: 80‐100mg/kg/d until fever resolves (TYPICALLY < 2 weeks)
‐ Low dose ASA: 3‐5mg/kg/d until labs normalize and no aneurysms noted on echo (typically 2 mos)
‐ Initiate treatment within 10 days of presentation to best prevent cardiac sequelae
‐ Echocardiogram: at time of diagnosis, and then 1‐2 and 6‐8 weeks after onset
Kawasaki Disease
(KD)
• Morbidity/Mortality:
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Most deaths occur suddenly between 3‐8 wks of onset: typically from MI due to coronary artery occlusion
Mortality in US and Japan: < 0.2%
• Sequelae:
Cardiac: ‐ Pericardial effusions (< 5%), myocarditis with CHF (< 5%), arrhythmias, valve regurgitation
‐ Coronary artery aneurysms (20%‐25% of untreated within 1st 10 days vs 5% of treated pts)
‐ Aneurysms typically occur between 1‐4 weeks after onset of illness and typically no later than 6 weeks
Non‐cardiac:
‐ Aseptic meningitis, gall bladder dilation, pancreatitis, facial nerve palsies
ED Case
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12 mo old male with fever > 39 C x 5 days
Non‐specific diffuse erythematous rash
Cracked lips
Subjective swelling of hands, no peeling
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4/8/2014
Atypical/Incomplete Kawasaki Dx
ED Case
• 1 week old term female
• Erythema around umbilical cord stump x 1 day
• Scant associated drainage
Newburger et al Pediatrics 2004
Omphalitis
• Epidemiology:
‐ At risk in first 2 weeks of life
‐ Rare in developed countries
‐ Risk factors: home delivery, decreased birth weight
• Pathophysiology:
‐ Bacterial colonization of umbilicus occurs soon after birth
‐ In rare cases the bacteria can invade the umbilical cord stump and surrounding tissues
‐ Produces an initial cellulitis with potential for direct spread of bacteria to the liver/peritoneum causing peritonitis, liver abscess, sepsis
Omphalitis
Omphalitis
• Presentation:
• Diagnosis:
‐ Drainage/erythema of varying degrees around the umbilical cord ‐ Clinical: 1. Purulent foul smelling discharge with any erythema of the anterior abdomen
2. Scant drainage with erythema that completely encircles the umbilicus
• Work‐up:
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Full septic w/u
Send any drainage for culture and GS
• Management:
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IV Abx: oxacillin and gentamicin
Consider vancomycin for MRSA coverage
+/‐ surgical debridement Admit all cases
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4/8/2014
Case
Petechial Rashes
• 8 year‐old
• 2 day history of rash
• lower extremities
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4/8/2014
Henoch‐Schönlein Purpura • Background
– Acute vasculitis
• Resulting in localized or systemic vascular damage
– 80% will have systemic involvement
– Commonly occurs in children 2‐10 years of age
– Precipitating factors
• Bacterial or viral infections
• Drugs, chemical toxins, food, immunizations
– Exact pathology not understood
Henoch‐Schönlein Purpura
• Clinical Manifestations
– Gastrointestinal
• Colicky abdominal pain
• Edema and hemorrhage of bowel wall
• Intussusception, obstruction, perforation
– Swollen and painful joints
– Renal
• Hematuria with/without proteinuria
• Acute nephritis
– CNS (rare)
Henoch‐Schönlein Purpura
• Skin Changes
– 2‐10 mm papules, macules, or urticaria
– Symmetrical distribution
– Occur in crops
– Initial lesions fade – Purpura develop
Henoch‐Schönlein Purpura
• Laboratory Values and Diagnosis
– No specific lab test
– CBC
• anemia
• platelet and coagulation studies normal
– Urinalysis: hematuria, proteinuria
– Skin biopsy: vasculitis and IgA deposits
– Radiology studies: suspected intussusception
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4/8/2014
Henoch‐Schönlein Purpura
• Management
– Rest and supportive care
– Steroids
– Hospitalization for systemic involvement
• Prognosis
Case
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9 year‐old
Otherwise healthy child
Rash
Bruising
Oral mucus membrane petechia
– Skin changes last 1 to 4 weeks
– Mortality < 1%
– Renal Disease 1%
– 50% have one recurrence
Idiopathic Thrombocytopenic Purpura
• Background
– An autoimmune thrombocytopenia
– Destruction of IgG coated platelets
– Acute ITP occurs mostly in children
• Peak incidence 18 months to 6 years
– Chronic ITP occurs in children > 10 years
• More common in females
– 4 to 5 cases per 100,000 children per year
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4/8/2014
Idiopathic Thrombocytopenic Purpura
• Clinical Manifestations
– Healthy child
• Skin Changes
– Petechiae, purpura, bruising
• Mucosal Changes
– Petechiae, purpura, bleeding
• Hepatosplenomegaly absent
• CNS (rare)
Idiopathic Thrombocytopenic Purpura
• Laboratory Values and Diagnosis
– CBC
• Thrombocytopenia
• Anemia associated with blood loss
– Type and Screen
– Bone Marrow Aspirate
• Usually not needed
• May be done prior to administration of steroid
Idiopathic Thrombocytopenic Purpura
Idiopathic Thrombocytopenic Purpura
• Management – Reserve drug therapy for platelet count < 20,000
– Drug therapy options
• Steroid
• IVIG
• Anti‐D immunoglobulin (WinRho) – Rh ‐ positive patients only
– Splenectomy • Reserved for some chronic ITP patients – ITP with life‐threatening bleed
• Splenectomy
• Medication therapy and platelet transfusion
• Management
– Avoid aspirin and ibuprofen drugs
– Limit vigorous activity
– Hospital admission • Unreliable social situation
• Active bleed
– Monitor clinical status and platelet levels
• Prognosis
– Self limited, full recovery usually 8 weeks
– Risk of intracranial hemorrhage .1% ‐ .9%
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4/8/2014
Case
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8 year‐old
Fever
Headache
stiff neck
Diffuse rash
Toxic appearance
Meningococcemia
• Background
– Caused by Neisseria meningitidis – Prevalence higher in winter/spring
– Transmission via droplets or respiratory secretions
– Most common in children < 5 years
– Peak incidence 3 to 5 months of age
Meningococcemia
• Clinical Manifestations
– Fever, headache
– Stiff neck, photophobia
– Vomiting
• Skin Changes
– Petechiae
– Purpura
• Fulminant Disease
– Shock, DIC
– Multi‐system failure
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4/8/2014
Meningococcemia
• Laboratory Values and Diagnosis
– Neurological exam
– CBC
• Leukocytosis with elevated band count
• Normal WBC and marked left shift
• Low WBC – Coagulation Studies
– CSF
• Elevated WBC and protein, decreased glucose
• Gram stain, culture
• Latex agglutination
Case
Meningococcemia
• Management
– Antibiotic (Cefotaxime, Ceftriaxone)
– Supportive care
• Airway management
• Venous access
• Hemodynamic support
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6 year‐old
Severe headache
Myalgia
Rash • Prognosis
– Untreated, coma and death can occur in 12 to 48 hours
– Long term • Neurological deficits
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4/8/2014
Rocky Mountain Spotted Fever
• Background
– Multisystem disease caused by Rickettsia rickettsii
• Multiply in the endothelial lining of the vascular system, resulting in vasculitis
– Transmitted via tic bite
• Attachment >6 hours is long enough for transmission
– Affects all ages
• Most frequently occurs in children 5 to 9 years of age
• Approximately 600 cases annually
– Peak incidence April to September
Rocky Mountain Spotted Fever
• Clinical Manifestations
– Tic bite (no tenderness)
– Incubation period 2‐12 days
– Prodrome
• Severe headache
• Malaise, myalgia
• High fever
Rocky Mountain Spotted Fever
• Skin Changes (3‐5 days)
– Erythematous macular rash (wrist/ankles)
– Rash moves centrally toward trunk
– Evolves into petechial, hemorrhagic lesions
– 10% of cases will not develop a rash
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4/8/2014
Rocky Mountain Spotted Fever
• Laboratory Values and Diagnosis
– Serological assays specific for rickettsia are positive 6 to 10
days into illness
– Immunofluorescent staining of skin biopsy
• Not readily available and need expert to interpret
– History of tic bite and clinical exam Rocky Mountain Spotted Fever
• Management
– Early diagnosis and empiric antibiotics
• Delay in treatment can result in death by second week
• Treatment before day 5 of illness, associated with good outcome
– Doxycycline 2 mg/kg/day PO BID 7‐10 days
• Prognosis
– Survival dependent on early diagnosis and treatment
– Mortality 25% in untreated cases
To Recap…..
Rash Findings to take Seriously:
Conclusion
• Rashes associated with mucous membrane involvement
• Extensive blisters/peeling of the skin
• Erythroderma with fever
• Severe pain out of proportion to PE
• Rashes associated with AMS
• Petechial/purpuric lesions
Take Away Points
1. Don’t miss the “Rashes that will kill you.”
2. Don’t sweat the “Rashes that just itch.”
3. “I’ve never seen a Kawasaki patient who looked well.”
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