Emergency Dermatology: Case 1, Question 1 The Most Likely Diagnosis Is: Case 1, Question 1 The Most Likely Diagnosis Is
Emergency Dermatology: Case 1, Question 1 The Most Likely Diagnosis Is: Case 1, Question 1 The Most Likely Diagnosis Is
Emergency Dermatology: Case 1, Question 1 The Most Likely Diagnosis Is: Case 1, Question 1 The Most Likely Diagnosis Is
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Skin biopsy Case 1, Question 2
What is the most important consult besides
• subepidermal blister
dermatology to get in a patient with
• epidermal necrosis SJS/TEN?
A. Renal
• sparse dermal
inflammatory infiltrate B. Ophthalmology
C. Allergy/immunology
• Diagnosis: severe
bullous drug eruption D. Wound care
E. GI/liver
Case 1, Question 2
What is the most important consult besides
dermatology to get in a patient with
SJS/TEN?
Emergency Dermatology:
A. Renal Bullae
B. Ophthalmology
1. SJS/TEN
C. Allergy/immunology
2. Pemphigus vulgaris
D. Wound care
3. Bullous pemphigoid
E. GI/liver
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Drug Eruptions:
Urticarial Drug Eruption
Degrees of Severity
• Treatment
– Antihistamines for simple urticaria Simple Complex
– Anaphylaxis
• H1 blocker (diphenhydramine) Morbilliform drug eruption Drug hypersensitivity reaction
• H2 blocker (ranitidine, famotidine) Stevens-Johnson syndrome
(SJS)
• Epinephrine (IM or IV)
Toxic epidermal necrolysis
• Methylprednisolone or dexamethasone
(TEN)
• Cardiovascular support
Minimal systemic symptoms
– MedAlert bracelet Systemic involvement
Potentially life threatening
vancomycin x x x vancomycin x x x
metronidazole x x x metronidazole x x x
ceftriaxone x x x ceftriaxone x x x
norepinephrine x x x norepinephrine x x x
omeprazole x x x omeprazole x x x
SQ heparin x x x SQ heparin x x x
docusate x x x docusate x x x
septra x x x x x x x x
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Hypersensitivity Reactions
Hypersensitivity Reactions
Clinical features (General)
• Skin eruption associated with systemic • Rash (morbilliform initially)
symptoms and alteration of internal organ
function • Fever (precedes eruption by day or more)
• Begins 2- 6 weeks after medication started • Pharyngitis
– time to abnormally metabolize the medication
• Hepatitis
• Classic culprits
– Aromatic anticonvulsants THESE CROSS- • Hematologic abnormalities
REACT – eosinophilia
• phenobarbital, carbamazepine, phenytoin
– atypical lymphocytosis
– Allopurinol
– Dapsone • Lymphadenopathy
– NSAIDs • Facial edema
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Stevens-Johnson (SJS) versus
Stevens-Johnson Syndrome (SJS)
Toxic Epidermal Necrolysis (TEN)
SJS TEN • Prodrome
– Fever, respiratory symptoms,
Atypical targets Erythema, bullae headache, vomiting, diarrhea
Mucosal Skin pain • Clinical morphology:
membranes ≥ 2 Mucosal – Round macules and papules,
membranes ≥ 2 red on the periphery and purple
in the center (like a target)
– Two or more mucous
Causes: Causes: membranes (eyes, mouth,
Drugs Drugs genitalia) involved
– Can progress to resemble toxic
Mycoplasma epidermal necrolysis (TEN)
HSV
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IVIG (intravenous immunoglobulin) as a IVIG for TEN
treatment for TEN Dose and Response
• Recommended dose: 0.5-1.0g/kg/d over 3-5 days
Human IVIG has antibodies against Fas L
• Arrest in disease progression in 24-48 hours
• Complete re-epithelialization within 4-10 days
• Decreases mortality?*
– Decreases to 6-12% in some studies
– Other studies report increased mortality
• 7 of 9 studies (non-controlled clinical studies with ≥ 10 pts)
– Overall mortality benefit of IVIG in doses > 2g/kg^
• Risk factors for failing to respond to IVIG**
IVIG blocks Fas mediated apoptosis in vitro – Delayed use of IVIG (≥ day 10), lower dose (2g/kg total), underlying
chronic diseases, higher BSA involved (>65%), older age
& • Also batch-to-batch variation in anti-Fas activity
Arrests development of TEN in vivo *Semin Cutan Med Surg 2006. 25:91-3
^ Allergology Int 2006. 55: 9-16
Bolognia et al. Dermatology 2003. **Arch Derm 2003. 139:26-32
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Pemphigus Vulgaris
• Elderly
• Widespread, larger friable blisters,
erosions
• 50% present with oral erosions and
100% develop oral lesions at some
time
• Flaccid blisters anywhere on the skin
• Blisters do not heal, but leave very
painful erosions up to 10 cm in
diameter
• Gradually worsening, progressive
course in most patients
– Until prednisone became available,
considered a fatal disease
• Treated with systemic
immunosuppressants
Images courtesy of Siegrid Yu, MD
Case 2, Question 1
• In this patient, the test most likely to be
abnormal is:
Emergency Dermatology:
A. Antinuclear antibody
B. Rheumatoid factor Purpura
C. Cryoglobulins
1. Vasculitis
D. Urinalysis
E. Stool guaiac
2. Rocky Mountain Spotted Fever
3. Meningococcemia
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Leukocytoclastic Vasculitis
Leukocytoclastic Vasculitis Etiology
• Conditions associated with LCV
• The causes produce foreign antigen to
– Idiopathic (45-55%)
which the host makes antibodies
– Infection (15-20%)
• Pathogenesis: Circulating immune
complexes (antigen-antibody usually IgG) – Inflammatory diseases (15-20%)
of the right size lodge in small vessels – Medications (10-15%)
• Complement is activated, calling in – Malignancy (<5%)
neutrophils (C5a), degranulating mast – Other
cells, causing vessel damage and swelling • Hypergammaglobulinemic purpura of Waldenström
at the site • HIV
• Cocaine use (p-ANCA +)
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Meningococcemia Meningococcemia
• Organism: N. meningitidis
• Skin lesions typically • Higher risk
associated with acute
sepsis
– Military recruits
• Acutely ill – Close contact with an index case
• Widespread eruption – Travel to an endemic area
– petechiae – Asplenia
– palpable purpura
• stellate, gunmetal gray – College students living in dormitory
• Can progress to
DIC/purpura fulminans
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Case 3
• 55 yr old male
• COPD, HTN, non-small
cell lung cancer and mild
psoriasis
• Presents with low grade
fever, shaking chills, and
diffuse erythema
(erythroderma)
• Meds:
– ACE inhibitor x 3 months
– 1 week of pulsed prednisone
with rapid taper for COPD
flare
Pustular Psoriasis
• Often occurs when known
psoriatics are given
Emergency Dermatology: systemic steroids
• When the steroids are
Erythroderma tapered, the psoriasis
flares, often with pustules
1. Pustular psoriasis
2. Toxin mediated erythemas • Can be life threatening
3. Kawasaki disease – High cardiac output state
4. Drug eruptions (hypersensitivity, TEN) – Electrolyte imbalance
– Respiratory distress
– Temperature dysregulation
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Psoriasis Aggravators Treatment for Psoriasis
• Medications • Sunburn • Topical therapy
– Steroid ointment (start mid-potency)
– Systemic steroids • Severe life stress – Calcipotriene (Dovonex)
– Beta blockers – Tar
• HIV • Phototherapy- refer to dermatologist
– Lithium – Up to 6% of AIDS – Broadband UVB or Narrowband UVB
– Hydroxychloroquine patients develop – PUVA: psoralens + UVA
psoriasis
• Systemic therapy- refer to dermatologist
• Strep infections • Alcohol for some – Acitretin (oral retinoid)
– Guttate psoriasis in – Methotrexate, cyclosporine
children • Smoking for some – Biologics
• etanercept, infliximab, adalimumab, alefacept, efalizumab
• Trauma **Systemic steroids are NOT on this list!
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Streptococcal Toxic Shock Syndrome
Toxic Shock Syndromes
Criteria
• Streptococcal Toxic Shock Syndrome • Isolation of group A streptococci from normally sterile
• Staphylococcal Toxic Shock Syndrome site OR
• Isolation of group A streptococci from non-sterile site
• AND
• Hypotension (SBP<90mmHg for adults)
• AND
• Two or more
– Renal impairment
– Coagulopathy (platelets < 100000/mm3 or DIC)
– Elevated LFTs
– ARDS
– Generalized erythematous macular rash +/- desquamation
– Soft tissue necrosis (necrotizing fasciitis, myositis, gangrene)
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Staphylococcal Toxic Shock Syndrome Staphylococcal Toxic Shock Syndrome
• Sudden onset high fever, myalgias, vomiting, • Diagnosis
diarrhea, headache, pharyngitis – High index of suspicion
• Rapid progression to shock – Criteria
• Diffuse scarlatiniform exanthem • Treatment
– Starts on trunk and spreads to extremities – Admit
– Erythema and edema of palms and soles – Supportive care (IV fluid, pressors)
– Erythema of mucous membranes – Remove packing, etc
• Strawberry tongue, conjunctival erythema
– IV antibiotics
– 1-3 weeks later, desquamation of hands and feet
– Clindamycin
– IVIG
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Kawasaki Disease-Like Syndrome
Kawasaki Disease
(KDLS) in HIV
• Treatment • Reported in 13 patients
– IVIG
• 2g/kg single infusion – 11 adults
– Aspirin – 2 children
• Must be given within 10d of fever onset
• 80-100mg/kg/d during acute febrile phase, then decrease to
3-5mg/kg/d after fever subsides
• Moderate-to-severe immune dysfunction
• Prognosis (untreated) (CD4 10-298 cells/mm2)
– 75% resolution without sequelae
– 25% abnormal coronary arteries with 1-2% mortality
in acute phase
– Leading cause of acquired heart disease in children
– Risk factor for adult ischemic heart disease and
sudden death in young adults
Case 4
• 37 yo woman with inflammatory bowel disease
• Rapidly progressive, painful ulceration of lower
leg appears 3 days after bumping her leg on a
chair
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Case 4, Question 1 Case 4, Question 1
• The most appropriate treatment for this • The most appropriate treatment for this
disorder is disorder is
A. Systemic steroids A. Systemic steroids
B. Intravenous antibiotics B. Intravenous antibiotics
C. Surgical debridement C. Surgical debridement
D. Compression dressing D. Compression dressing
E. Wet to dry dressings E. Wet to dry dressings
Pyoderma Gangrenosum
• Rapidly progressive (days) ulcerative process
• Begins as a small pustule which breaks down
Emergency Dermatology: forming an ulcer
Ulcers • Undermined violaceous border
• Expands by small peripheral satellite ulcerations
Pyoderma Gangrenosum which merge with the central larger ulcer
• Occur anywhere on body
• Triggered by trauma (pathergy) (surgical
debridement, attempts to graft)
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Pyoderma Gangrenosum
Pyoderma Gangrenosum
Treatment
• Most cases have no • AVOID DEBRIDEMENT
underlying cause • Refer to dermatology
• Associations: • Treatment of underlying disease may not help PG
– Inflammatory bowel – Topical therapy:
disease (1.5%-5% of • Superpotent steroids
IBD patients get PG) • Topical tacrolimus (up to .3%)
– Rheumatoid arthritis – Systemic therapy:
• Systemic steroids
– Seronegative arthritis
• Cyclosporine or Tacrolimus
– Hematologic • Cellcept
abnormalities • Thalidomide
• TNF-blockers (Remicade)
The end.
(whew!)
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