Deadly Derm Case #1 2/3/2014

Deadly Derm
Kathleen Cowling, MS, DO, FACEP
MCEP Winter Symposium
Boyne Mountain 2014
Case #1
• 20 y.o. male college student presents with
rash, fever, and headache. He appears ill.
• PMHx: none
• SocHx: Sexually active, recent travel to NC
• Vitals: BP 110/60, RR 18, HR 110, T 101.8°
Rocky Mountain Spotted Fever
Etiology: Tick vector for Rickettsia rickettsi
Where: Eastern 2/3 of the US
Incidence: varies by State
Clues: maculopapular palmar rash, starts
distally and spreads centrally, progresses to
palpable petechial (vasculitis)
• Caveats: only ~50% remember the tick
• Diagnosis: Clinical, DO NOT WAIT TO TREAT
Rocky Mountain Spotted Fever
• M&M: Death 30% untreated, which drops to
5% with ABX. 15% have permanent deficits
• Treatment: Doxycycline even for PEDS and OB
Case #2
• 18 y.o. woman with confusion and fever, and
evolving rash, college student
• PMHx: asthma
• SocHx: No drugs, non-smoker, rare Etoh
• Etiology: Neisseria meningitidis
• Pathophysiology: Gram – intracellular
diplococci causing fulminate sepsis
• Who: close quarters, adolescents, peds<4
• Clues: ill appearing, fever, shocky, mental
status changes, maculopapular rash begins on
extremities and spreads, evolves into palpable
• Diagnosis: LP, pleocytosis Glucose, Protein
• Treatment: Ceftriaxone DOC, Dexamethasone
0.15/kg before ABX if possible
• Prevention: vaccine
• Prophylaxis: Cipro PO adults, Ceftriaxone IM
• Mortality: 3-50% depending on treatment
• Morbidity: DIC, ARDS, ARF
Case #3
• 60 y.o. male complaining of weakness and
weight loss with fatigue
• Vitals: HR 140, RR 26, BP 98/60 T 101.5°
• PE: Appears toxic, JVD, coarse Breath sounds,
3/6 systolic murmur, generalized palpable
petechial rash
Bacterial Endocarditis
• Symptoms: Fever, weakness, fatigue, weight
• Signs: Murmur, Janeways, Oslers nodes,
splinter hemorrhages, Roth spots, petechial
rash (most common finding)
• Clues: Valvular disorders, IVDU, Ports, Dialysis
poor dental hygiene
• Etiology: Streptococcus viridans, coag negative
Case #4
• 35 y.o. female complaining of fever and rash
to her legs
• PMHx: neg
• SocHx: no IVDU
• Vitals: BP 100/50, RR 24, HR 120, T 101.0°
• PE: non-palpable Petechiae/Purpura
Bacterial Endocarditis
• Diagnosis: Blood Cultures X 3 and TEE
• Treatment: Broad, but cover MRSA with
vanco, or linezolid, gentamicin
• Mortality: Staph 25-40% Strep 19%
• Morbidity: high incidence of valvular surgery
Thrombotic Thrombocytopenic
• Symptoms: Fever, neuro changes, rash
• Diagnosis: Thrombocytopenia, hemolytic
anemia, LDH, renal failure
• Etiology: Deficiency of ADAMTS13, platelet
aggregation  thrombi
• Treatment: Plasmapheresis
• Causes: HIV,SLE, pregnancy, malignancy,
transplant, Quinine, Cyclosporine, Clopidogrel
• Clues: PT, PTT, & fibrin are often normal, will
see helmet cells and schistocytes on smear
• Treatment: Immediate Heme consult, need
plasmapheresis, if not available give FFP and
transfer. Steroids and admit ICU
• Mortality: >90% if not treated,  10% with
exchange transfusion
• AVOID: giving platelets- fuels the fire, causes
more thrombi
“Fifth Disease” Erythema Infectiosum
• Etiology: Parvovirus B19, respiratory spread
• Symptoms: fever, headache, runny nose,
• Signs: “slapped cheek” erythematous cheeks
• Diagnosis: Clinical ( serum to check for
immunity only if necessary)
• Treatment: supportive
• Why a deadly derm? Isolation-protect
Purpura Fulminans/DIC
• Signs: Hemorrhagic bullae, Multi organ failure
• Etiology: Sepsis, Gram neg, & meningococcus
- Massive trauma, hepatic failure, end stage
malignancy, snake envenomations, transfusion
reactions, pregnancy
• Stimulus causes endogenous over activation
of fibrinolytic system, resulting in bleeding,
vessel occlusion and end organ damage
4 y.o. male with rash and fever, rhinorrhea
PMHx: neg
Immun: UTD
Vitals: BP 90/62, HR 105, RR 22, T 100.6°
Case #6
• 49 y.o. female via EMS, lethargic, bleeding
gums, hypotensive and petechial rash with
widespread bullae
• PMHx: HTN, COPD, known lung CA with mets
• Vitals: BP 70/35, HR 134, RR 30, T 102°
• PE: toxic, dry MM,
Purpura Fulminans/DIC
• Diagnosis: Thrombocytopenia, schistocytes from
• fibrinogen, PT,PTT, D-DIMER, FDP’s
• Clue: the lower the fibrinogen the worse the
• Treatment: ICU, Immediate Heme consult, ABX,
FFP, Vit K, platelets, cryoppt, trans rbc’s
• M&M: varies
Case #7
Pemphigus Vulgaris
• 58 y.o. female via EMS complaining of painful
swallowing and rash
• PMHx: HTN, myasthenia gravis
• Vitals: BP 100/70 HR 110, RR 20, T 99.4°
• PE: Toxic, mucocutaneous blistering
• NOT Bullous pemphigoid because of the oral
lesions. BP occurs in older patients and has a
better prognosis than PV
Pemphigus Vulgaris
Case #8
• Clue:+Nikolsky’s, Bullae can coalesce resulting in
• Diagnosis: Clinical and biopsy
• Etiology: chronic autoimmune disorder,
antibodies directed against keratinocytes
causing the separation of the epidermis,
triggers- stress, drugs (captopril, rifampin)
40 y.o. male with leg pain, weakness, vomiting
PMHx: HTN, IDDM, arthritis
Vitals: BP 95/55, HR 120, RR 24, T 102°
PE: Toxic, dry MM, hemorrhagic bullae
• Treatment: wound care prevent infection and
steroids, immunosuppressant Rx by Rheum
• Mortality: 90%without steroids, 5-15% with
Necrotizing Fasciitis
• Etiology: Group A β-hemolytic Strep most common,
however can be polymicrobial and need to cover
anaerobes, Vibrio vulnificus
• Risk patients: IVDA, DM, AIDS, Alcoholics, any
• Clue: pain out of proportion for cellulitis, crepitance,
rapidly spreading
Necrotizing Fasciitis
• Treatment: Surgical debridement, Broad
spectrum ABX, possibly HBO
• Mortality: depends on the organism and
patient population, upwards of 75%, but
improved with HBO used after surgery
• Diagnosis: plain film may show SubQ air, blood cultures
Case #9
• 22 y.o. male with skin peeling for 2 days and
complaint of weakness
• PMHx: recent cellulitis tx with TMP-SMX
• SocHx: no travel
• Vitals: BP 96/50 HR 120 RR 20 T 99.2°
• PE: Toxic, dry MM, shedding skin
TEN-Scorten Score
Toxic Epidermal Necrolysis
• Definition: >30% BSA detachment, extreme of
Steven Johnson’s
• Symptoms: fever, eye irritation, sore throat
• Signs: mucosal lesions, +Nikolsky’s
• Etiology: Drug reaction from Sulfa
• Severity rated via Scorten score done on
presentation and then 3 days later
• RISK patients: HIV poly pharm
Toxic Epidermal Necrolysis
• Treatment: ICU-Burn unit, wound care
• AVOID Sulfadiazine burn cream
• Ophthalmology consult-adhesions
• Careful Fluid/Electrolyte management
• IVIG-not FDA approved
• Controversy over steroids- successful in India
Case #10
65 y.o. male with oral lesions and rash
PMHx: recent sinus infection
Vitals: BP 115/79 HR 124
RR 24 T °101.7
Stevens Johnson Syndrome
• Etiology Drug Reaction is most common-SULFA
50% idiopathic
• Clue: Coalescing target lesions, mucosal lesions,
<10% skin slough
• Treatment: STOP the precipitant, and treat any
other underlying cause, Fluid resuscitation and
electrolyte monitoring, comfort measures
Case #11
• 10 month old male with fever, decreased oral
intake and peeling rash
• PMHx: neg
• Immun: UTD
• Vitals: HR 130, RR 18 T 102.4°
• PE: diffuse erythematous rash, which keeps
Case #12
25 y.o. female with fever and red rash
PMHx: neg
Meds: none
LMP: currently
Vitals: BP 90/60 HR 120 RR 20 T 101.4°
Toxic Shock Syndrome
• Treatment: Remove the source, and give
Clindamycin plus another anti-staph ABX, fluid
resuscitation, admit to ICU and possible IVIG
• Morbidity: Multi-organ failure
Staphylococcal Scalded Skin Syndrome
• Children <5 yr
• Symptoms: Starts as ‘scarlatiniform’
erythematous rash and proceeds to blister
and sloughs + Nikolsky’s
• Treatment: ABX against staph, wound care,
fluid management
• Mortality: <5%
• Clue: no oral lesions
Toxic Shock Syndrome
• Classic association with tampons-staph
• Etiology: Toxin mediated
• Change in susceptibility antigenic shift in the
population now most have developed
• Clue: Not always tampons, any source of staph
or strep can do it
Acknowledgement to
Heather M. Murphy-Lavoie, MD
• Petechiae and fever should get your attention
• Palpable petechiae means vasculitis
• Non-palpable petechiae most often due to
• Hemorrhagic bullae are BAD!
• Diffuse erythema with associated fever and
+Nikolsky’s is ominous
• Bullous rash and mucosal lesions steroids are
likely indicated
Image Challenge
Image Challenge
Q What is the diagnosis in this patient who had abdominal pain?
2. Henoch-Schönlein purpura
Q What is the diagnosis in this patient who had abdominal pain?
1. Familial Mediterranean fever
2. Henoch-Schönlein purpura
3. Meningococcemia
4. Polyarteritis nodosa
5. Rocky Mountain spotted fever
Skin biopsies revealed leukocytoclastic vasculitis with IgA deposition, which is consistent
with IgA vasculitis (Henoch-Schönlein purpura). The patient was treated with high-dose
glucocorticoids including pulse therapy, which were tapered successfully after
cyclosporine was added to the treatment. Henoch-Schönlein purpura, a systemic smallvessel vasculitis, causes palpable purpura in the lower extremities, abdominal pain,
arthralgia, and glomerulonephritis.