Hospital Dermatology: Pearls and Pitfalls U044 - Bridges - 13089 10938...•Fever, leukocytosis,...
Transcript of Hospital Dermatology: Pearls and Pitfalls U044 - Bridges - 13089 10938...•Fever, leukocytosis,...
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Hospital Dermatology:
Pearls and PitfallsAlina G. Bridges, D.O.
Associate Professor
Program Director, Dermatopathology Fellowship
ASDP Alternate Advisor to the AMA-RUC
Department of Dermatology, Division of Dermatopathology and Cutaneous Immunopathology
Mayo Clinic, Rochester
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Disclosures
• Relevant Financial Relationships
–None
• Off Label Usage
–Yes
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What is Hospital Dermatology?• Long-standing unique tradition at Mayo, a
tertiary care center with a large referral service
• We preserved and improved on the quality of care rendered our patients with difficult skin diseases in our hospital program
• Hospital core group– Clinical Division
• MDP Davis, R El Azhary, D. Wetter, M McEvoy, J Sartori
– Laboratory Division
• AG Bridges, MC Camilleri
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Role of Dermatology Hospitalist
Hospitalist
Inpatient DermatologyLymphoma, Autoimmunebullous disorders, Ulcers,
PG, Calciphylaxis, Vasculitis, CTD, Erythroderma,
Generalized dermatitis, Hypersensitivity
reactions, Cellulitis, Psoriasis
Outpatient - Hospital based
Psoriasis• Goeckerman• Phototherapy
Consult ServicePediatrics/Neonatal
medicine, Drug eruptions, GVHD, Dermatitis, Lesions,
Vasculitis/vasculopathy, Autoimmune diseases,
Infection, Ulcers, Lymphoma
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Pearls and Pitfalls of Hospital Dermatology
Goals of this lecture
• Review clinical and
histopathological
presentations,
differential diagnosis,
evaluation and
management of major
categories of
dermatologic diseases
in hospitalized patients
1. “Help!” Red and Scaly All Over
2. I itch and nothing helps!
3. I think it’s a drug eruption. Now what do I do?
4. Bugs – What “bugs” you?
5. Oh no! The patient has purpura
6. Yikes! The flesh eating ulcer
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“HELP!”
Red and Scaly
All Over
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• 91 year old woman
admitted to Ortho for left
hip replacement
• HTN, hyperlipidemia,
GERD and dermatitis
• Worsening dermatitis for
3 months
• Hip replacement
cancelled
• Admitted to Derm service
©2011 MFMER | slide-7
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What is your diagnosis?
A. Dermatitis
B. Pityriasis Rubra
Pilaris (PRP)
C. Psoriasis
D. Drug reaction
E. Lymphoma
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CD4
CD7
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Sézary syndrome
• Clonal T-cell receptor gene rearrangement detected in skin biopsy
• Lymphadenopathy
• Convoluted lymphocytes on peripheral smear
• Patient declined aggressive management and methotrexate initiated up to 25mg weekly
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Erythroderma• Psoriasis
• Pityriasis rubra pilaris
• Dermatitis
• Drug eruption
• Lymphoma
• Infection
• Autoimmune bullous disorder
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“I itch and
nothing
helps!!”
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• 54 y/o F with severe pruritic eruption x 1.5 years
• Admitted to Dermatology inpatient service for wet dressings
• Outside skin biopsies non-specific dermatitis
• DIF ? DH (negative anti-gliadin and TTG antibodies, duodenum biopsy negative for celiac disease)
• Prior treatments: - Topical corticosteroids (poor response)- Dapsone gel (poor response)- UVB (some benefit)- Dapsone 50 mg QD x 8 months (initially helpful)- Systemic corticosteroids ( 30 day course w/ taper)- Hydroxyzine and doxepin (some benefit)
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©2011 MFMER | slide-20
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What is your diagnosis?
A. Scabies
B. Prurigo Nodularis
C. Dermatitis Herpetiformis
D. Bullous Pemphigoid
E. Lymphoma
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Pruritus Evaluation
• Normal or negative: CBC, peripheral smear,
ESR, SPEP, IgA, IgM, IgG, TSH, ANA, gliadin Ab,
endomysial Ab, TTG, Hep B/C, HIV, CXR
• AST 51 and ALT 111: known steatohepatitis
• Skin swab: Methicillin sensitive Staph aureus
• Biopsies for H&E and DIF obtained
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• Elevated BP180 (90) and BP230 (90)
• Final diagnosis: Bullous pemphigoid (pemphigoid
nodularis)
DIF: Linear deposition of IgG
along BMZIIF: 1:640 BMZ titer on monkey
esophagus with epidermal
pattern on salt-split skin
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Pearl: Atypical presentations of bullous pemphigoid
• 20% non-bullous
• Eczematous, dyshidrosiform, urticarial,
erythrodermic, nodular, lichenoid, or
targetoid
• Include evaluation for autoimmune bullous
disorders in erythroderma and pruritus
work-up
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Pruritus
• Xerosis
• Dermatitis
• Hypersensitivity
reaction
• Dermatophytosis
• Folliculitis
• Scabies
• Autoimmune bullous
disease
• Liver disease
• Renal disease
• Thyroid disease
• Anemia
• Lymphoproliferative
disorders
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I think it’s a drug eruption.
Now what do I do?
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Drug Eruptions
Simple
No systemic symptoms
Complex
Systemic involvement
Life threatening
Other
Morbilliform
Urticarial
Drug hypersensitivity reaction
Stevens-Johnson syndrome (SJS)
Toxic epidermal necrolysis (TEN)
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Drug Reactions
3 things you need to know1. Type of drug reaction
2. Statistics
– What drugs are most likely to cause that
type of reaction?
3. Timing
– How long after the drug was started did
the reaction begin?
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Drug TimelinePatient Name:
Clinic Number:
RASH
Reaction Type Drug 2/5
Morbilliform ASA x x x x x x x x x x x x x x x x
Urticarial Colace x x x x x x
Fixed Drug Bactrim x x x x
Erythroderma Tetracycline x x x x x x
EM PCN x x x x x x
SJS Vancomycin x x x x x x
TEN HCTZ x x x x x x x
AGEP Dilantin x x x x x x x x x x
DRESS
Vasculitis
• LCV
• HSP
• Other
Lupus-like
Blistering
• Pemphigus
• LABD
• PseudoPCT
Pseudolymphoma
Cytotoxic
OTHER
MR = Many Reports
R = Reported
NR = Not Reported
UserName = Mayo1
Password = Physicians
DATES
% Rxn
for Drug
http://www.drugeruptiondata.com
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This patient is hospitalized for a pneumonia and has
developed this pruritic eruption. What is the most important
intervention that will lead to resolution of the rash?
A. Topical corticosteroids
B. Systemic Corticosteroids (prednisone 0.5-1 mg/kg/day)
C. Find out what drug was recently started and stop that drug
D. Arrange for an urgent skin biopsy to find out what the rash is due to
E. Oral antihistamines
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Clinical Pearls• Drug eruptions are extremely common
• Tend to be generalized
• Maculopapular/morbilliform most common
• Best intervention: Stop the offending drug
• How to spot the culprit?– Drug started within days to a week prior to rash
– Can be difficult!
– Tip: can generally exclude all drugs started after onset of rash!
• Drug eruptions can continue for 1-2 weeks even after stopping culprit drug
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CASE
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Patient just admitted
BP 80/50 mmHg
Your diagnosis?
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TEN
• epidermal necrosis & dermal
infiltrate compared to SJS
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TEN• Most severe presentation in
spectrum which includes
EM & SJS
• Generalized erythema,
purpura & flaccid bullae
• Widespread epidermal
detachment 10% of BSA
• Extensive mucous
membrane involvement
• No target lesions
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TEN/SJS• Drug-related
• Extracutaneous involvement
– Fever
– Leukopenia
• Poor prognostic factor
– Involvement of GI, renal & respiratory
mucosa
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TEN/SJS• Drug-related
– Sulfonamides
• Slow acetylator phenotype
– Anticonvulsants
• Dilantin, phenobarbital, carbamazepine, lamotrigine
– Penicillin derivatives
– NSAIDS
– Allopurinol
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SJS
• Target lesions
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SJS
• Generalized erythema or purpura
• <10% epidermal detachment
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SJS
• Extensive ulceration
of oral, genital, &/or
ocular mucous
membranes ( 2
surfaces)
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TEN/SJS Bottom Line
(Personal View)• TEN/SJS are both associated with high mortality rates (up to 25%) from
sepsis and multiorgan failure
• Require intensive supportive management in ICU/Burn unit
• Discontinue the causative drug or treat underlying infection
– Mycoplasma in 25% of pediatric patients with SJS
• Bulk of evidence
– Supports use of intravenous immunoglobulin• 0.5-1gm/kg/d X 4 days
• IVIG blocks Fas mediated apoptosis in vitro and arrests development of TEN in vivo
– Does not support use of systemic corticosteroids
– Cyclosporine • 3mg/kg X 10days, then 1 mg/kg X 10 days; go back up if you taper too quickly & the
patient flares
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CASE
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• 33 yo F G1P1, 2 weeks postpartum
• Persistent , painful generalized eruption X 3 months
• Severe head, ear and mouth pain with dysphagia
• Reported NO medications
• Received systemic and topical steroids
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©2011 MFMER | slide-48
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What is your diagnosis?
A. Scabies
B. Drug reaction with eosinophilia and systemic disease (DRESS)
C. Polymorphic eruption of pregnancy
D. Connective tissue disease (CTD)
E. Psoriasis
F. Autoimmune bullous disorder
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Laboratory evaluation– Oral swab: + HSV-1 &
Candida
– Cutaneous swab: MSSA
– Leukocytosis (18.9) with eosinophilia (4.47)
– ↑ aldolase, LDH, ALT, AST
– PCR HHV-6 negative
• CT chest/ abdomen/ pelvis
– B/L axillary & inguinal lymphadenopathy
• ↓ zinc
• TSH wnl
• EKG normal
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• Latent TB
– + PPD 7 months ago – INH initiated
– Onset of rash 4 months later
– Rash persisted and 1 month later INH
discontinued
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Hospital course
• 3 consecutive days high dose
methylprednisolone (1.5 gm/day)
followed by transition to prednisone
• Mycophenolate mofetil and IVIG
• ↓ aldolase, LDH, LFTs, eosinophils
• Improvement in skin, symptoms
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DRESS Syndrome
Drug
Reaction with
Eosinophilia and
Systemic
Symptoms
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DRESS Syndrome• AKA:
• Anticonvulsant Hypersensitivity Syndrome
• Drug-induced Pseudolymphoma
• Drug-induced Hypersensitivity Syndrome
• Hypersensitivity syndrome
• Drug-induced Delayed Multiorgan
Hypersensitivity Syndrome
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DRESS Syndrome• Severe, idiosyncratic drug reaction
• Commonly implicated drugs: anticonvulsants,
sulfonamides, allopurinol, NSAIDS, azithromycin,
azathioprine, and anti-retrovirals
• Most cases present after delay of 2-8 weeks, but onset has
been reported from 3-105 days after drug initiation
– Time to abnormally metabolize the drug leading to toxic
drug metabolites
• Reactivation of HHV-6 has been observed
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Clinical Features of DRESS• Fever, leukocytosis, eosinophilia
• Huge variability in presentation
– Each class of drug causes a slightly
different clinical picture
• Facial edema mimicking angioedema
• Generalized eruption: erythematous
edematous papules, vesicles, bullae, pustules,
purpura, target lesions and erythroderma
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Clinical Features of DRESS• Lymphadenopathy
• Myositis
• Liver function test abnormalities
• May result in severe hepatocellular or
cholestatic damage, necessitating transplant
– 10% mortality typically from liver failure
• Variable lung, kidney, heart, thyroid
involvement
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Treatment of DRESS• Withdrawal of offending medication
• Avoid cross-reacting medications
• High-dose systemic steroids
– 1.5-2 mg/kg tapering dose over 1-3 months
• LFTs should be followed until resolved
• Supportive care
• ICU/Burn unit care is not required
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Miscellaneous Drug Eruptions You
Should Know About
• Acute generalized exanthematous
pustulosis (AGEP)
• Linear IgA bullous dermatosis (LABD)
• Symmetric drug-related intertriginous
and flexural exanthema (SDRIFE)
• TNF-alpha antagonist-induced lupus-
like drug eruption/syndrome (TAILS)
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CASE
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• 31 year old woman admitted to Medicine
• 1 week history of generalized erythema and edema
• Following uncomplicated Caesarian delivery
• Given Clindamycin
• Tachycardia, leukocytosis, elevated ESR and CRP
• Systemic steroids given
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Acute Generalized Exanthematous
Pustulosis (AGEP)• Also known as a pustular drug eruption
• Fever and leukocytosis with neutrophilia and eosinophilia are often present
• Many implicated drugs: macrolide & beta-lactam antibiotics, cephalosporins, tetracyclines, vancomycin, sulfonamides, carbamazepine, allopurinol, furosemide, antifungals, calcium channel blockers, ACE inhibitors, acetaminophen
• Sudden onset 2- 5 days after drug started
• Begins on the face and intertriginous areas
• Disseminates in a few hours
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Treatment of AGEP
• Discontinuation of drug
• Topical care
• Eruption usually resolves rapidly in 3-5
days with desquamation
• May be due to a specific T-cell reaction
• Neutrophils may be recruited by local
cytokines
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CASE
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• 19-yo man with
pruritic eruption
• Began shortly after
starting
vancomycin
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DIF- Linear deposition of IgA along the
BMZ
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Drug-induced LABD• Various clinical presentations
• Vesiculobullous eruption on trunk/extremities
– Herpetiform or rosette-like bullae
• Morbilliform, urticarial
• Can resemble DH, BP, CP, PV, LP, EM or TEN
• 40% with mucosal involvement
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Drug-induced LABD• Drug-induced
– Vancomycin –most common drug
– Others: Penicillins, cephalosporins, captopril,
trimethoprim/sulfamethoxazole, phenytoin,
furosemide, glyburide, diclofenac
• Onset:3-14 days after exposure
• Target Ag- BPAg2 (BP180)
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Drug-induced LABD
Treatment
• Removal of the offending agent
• Dapsone, Sulfapyridine,
Corticosteroids
• Dialysis
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CASE
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• 84 yo male
• 2 day intertriginous
eruption
• On Levaquin,
Cefepime, and
Hydroxyzine
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©2011 MFMER | slide-82
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©2011 MFMER | slide-83
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What is Your Diagnosis?
A. Acute generalized exanthematous pustulosis (AGEP)
B. Intertrigo
C. Hailey-Hailey disease
D. Cellulitis
E. Symmetric drug-related intertriginous and flexural exanthema (SDRIFE)
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©2011 MFMER | slide-85
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©2011 MFMER | slide-86
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Symmetric Drug-Related Intertriginous and
Flexural Exanthema (SDRIFE)• Diagnostic criteria
– Exposure to systemic drug predominantly
aminopenicillins and B-lactams at first or repeated
dose (contact allergens excluded)
– Erythema of the gluteal/perineal area
– Involvement of at least one other
intertriginous/flexural localization
– Symmetry of the affected areas
– Absence of systemic symptoms and signs
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CASE
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• 18 year old female with juvenile RA in the MICU
• Fever, chills, hypotension, headache, N/V, fatigue,
weakness
• Etanercept 0.98mL of 50 mg/mL SC weekly
• Started 1 year ago and last injection 4 wks ago
• Methotrexate 2.5 mg weekly
• Discontinued 8 wks ago
• Prednisone 5 mg qAM
• Increased to 20 mg
• Started on amoxicillin 2 days after rash
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©2011 MFMER | slide-90
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©2011 MFMER | slide-91
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©2011 MFMER | slide-92
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©2011 MFMER | slide-94
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The most likely diagnosis for this patient’s
skin eruption is:
A. Stevens Johnson syndrome (SJS)
B. Infectious mononucleosis from EBV
C. Lupus erythematosus or lupus-like reaction secondary to etanercept
D. Secondary syphilis
E. DRESS (drug reaction with eosinophilia and systemic symptoms)
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Laboratory Evaluation
• Pancytopenia
• Anemia and thrombocytopenia (3 months prior);
leukopenia; normal peripheral smear; no
eosinophilia
• Elevated ANA (>12)
• Elevated ds-DNA (>1000)
• ENA: Elevated Scl70 Ab (1.8)
• Decreased Complement
• Total C (8), C4 (9)
• SPEP: Polyclonal hypergammaglobulinemia
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Vacuolar interface dermatitis consistent with lupus-like
reaction secondary to etanercept or LE
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TNF-alpha Inhibitors• Using more of these agents to treat chronic inflammatory
diseases
• RA, AS, psoriasis, PsA, and IBD
• Infliximab
• Chimeric monoclonal Ab (IV)
• Etanercept
• Recombinant TNF-alpha soluble receptor fused to the Fc fragment of IgG2 (SC)
• Adalimumab
• Recombinant human IgG1 monoclonal Ab (SC)
• Golimumab
• Human IgG1 monoclonal Ab (SC)
• Certolizumab
• Recombinant humanized monoclonal Ab Fab fragment (SC)
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TAILS
• Incidence 0.2-0.4% • Difficult to establish
• Based on post-marketing studies
• As use of these agents increases, incidence of cutaneous reactions will increase
• Most cases caused by the agents that have been more widely used
• Onset: <1 month – 4 years
• Diagnostic and therapeutic challenge
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TAILS: Pathogenesis
• TNF-alpha inhibitor leads to production of autoantibodies
• Disruption in cytokine balance• Suppression in production of Th1 cytokines,
driving the immune response to Th2 cytokine production
• Interference with apoptosis by decreasing CD44 expression
• Inhibition of cytotoxic T-cells
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Drug-induced autoimmunity
• Patients treated with TNF-alpha
inhibitors develop antibodies found in
patients with SLE (ANA, ds-DNA, ENA)
in the absence of clinical features of
SLE
• Not an indication to stop drug
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Drug-induced autoimmunity
• Use of these agents may trigger or unmask SLE in some patients
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TNF-alpha Inhibitor Induced SLE: Diagnosis
• In the setting of ongoing treatment with TNF-alpha inhibitor
• No prior history of SLE
• Cutaneous findings of SLE • Malar rash, photosensitive rash, mucosal ulcers, alopecia
• Systemic findings of SLE• Constitutional symptoms – fever, malaise, weight loss
• Arthralgias, arthritis
• + Serology – ANA, ds-DNA
• Low complement
• Negative anti-histone antibodies
• Resolution of symptoms when drug is discontinued
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TNF-alpha Inhibitor Induced SLE:
Management
• Stop the drug
• Resolution of symptoms in 3 weeks to 6 months
• Patients may require systemic therapy
• Corticosteroids and steroid sparing immunosuppressive agents (methotrexate, azathioprine, mycophenolate mofetil)
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Regarding TNF-alpha Inhibitors
which statement is INCORRECT:A. It is contraindicated to use these drugs in patients with
SLE
B. Re-challenging patients who develop anti-TNF alpha induced SLE with alternative TNF-alpha inhibitor agents is safe.
C. It is recommended to perform baseline serologic testing for CTD prior to starting therapy
D. It is recommended to perform baseline CXR and TB testing prior to starting therapy.
E. Patients should be followed closely after initiation of therapy to assess for development of lupus-like reactions and other cutaneous reactions
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TNF-alpha inhibitors can cause the
following cutaneous reaction: A. Psoriasiform dermatitis
B. Granulomatous dermatitis
C. Vasculitis
D. Alopecia areata
E. All of the above
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Psoriasiform dermatitis and palmoplantar pustulosis indistinguishable from psoriasis
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Cutaneous Reactions to TNF-alpha
Inhibitors• Injection site reaction
• Urticaria
• Cellulitis
• Psoriasiform dermatitis
• Granulomatous dermatitis
• LE-like syndrome
• Vasculitis
• Alopecia areata
• Erythema nodosum
• SJS
• Morphea
• Erythroderma
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CASE
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• 70 yo F, 2 month h/o recurrent follicular lymphoma, treated 2 yrs ago
• Transferred from outside hospital for worsening desquamating eruption and severe mucositis for 1 month
• Skin and mucosal biopsies showed lichenoid dermatitis and mucositis c/w SJS/TEN
• 1 month prior to the eruption developing, she received 1 dose of a cefazolin as well a course of trimethoprim/sulfa and levofloxacin for URI
• She has not responded to systemic steroids and IVIG
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What is the likely diagnosis?
A. Stevens Johnson syndrome/ toxic epidermal necrolysis (SJS/TEN)
B. Drug reaction with eosinophilia and systemic symptoms (DRESS)
C. Lymphoma
D. Infection
E. Paraneoplastic pemphigus
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Paraneoplastic pemphigusDIF- Cell surface staining along with linear to granular deposition along the BMZ with IgG and C3
• IIF - + ICS titer 1:80
• Dsg 3 +
• Dsg 1 -
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Pitfall
• Not considering other diseases
associated with mucosal lesions,
blisters, and sloughing when
evaluating a patient for possible
SJS/TEN
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BUGS: What
“bugs” you?
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• 77 yo male with CLL most recently treated with ibrutinib (EGFR inhibitor)
• Abdominal pain secondary to acute pancreatitis
• Worsening skin eruption involving head, neck, chest, and back 2-3 weeks after ibrutinib started
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What is your diagnosis?
A. Ibrutinib drug eruption
B. Scabies
C. Varicella-Zoster infection
D. Deep fungal infection
E. Impetigo
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Disseminated VZV• Lesion swab
- Bacterial culture: Staph aureus 4+
- HSV1/HSV2 PCR: negative
- PCR VZV: Positive
• Reactivation of VZV infection: started on IV Acyclovir
10mg/kg Q8H until lesions have crusted. Negative
pressure room. Mupirocin 2% oint TID
• Ophthalmology: no ocular involvement
• Pancreatitis due to visceral involvement
• Cholecystectomy deferred
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Disseminated Zoster Pearls
• Definition
– > 20 lesions outside of 2 contiguous dermatomes
• At risk group
– Elderly, immunosuppressed
• Viscera can be affected
• Hutchinson’s sign – Call ophthalmology
• Contact and airborne precautions
• Treatment
– IV Acyclovir until lesions healed over or clear
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Cutaneous adverse effects of targeted
therapies• Epidermal growth factor (EGFR) inhibitors
– Papulopustular eruption in a seborrheic distribution
• Most common cutaneous side effect
• Dose-dependent
• 1-2 weeks after therapy
– Pruritus, xerosis
– Paronychia
– Mucositis
– Pattern or cicatricial alopecia
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What is the best management for papulopustular
eruption from an EGFR inhibitor?
A. Systemic steroids
B. Topical retinoids
C. Low potent topical steroids and topical clindamycin
D. Systemic antihistamines for pruritus and systemic antibiotics (tetracyclines) for severe eruptions
E. C and D
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Pearl
• Dermatologists need to be familiar with
the skin-related toxicities associated
with targeted therapies
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CASE
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• 72 yo F with psoriasis and
psoriatic arthritis
– Worsening skin eruption on
extremities x 1 month
• Infliximab
• Methotrexate
• Prednisone
Methylprednisolone
– Fever, tachypnea,
tachycardia
– Admitted for possible sepsis
• Started on Vancomycin
and Cefepime
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©2011 MFMER | slide-131
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What is your diagnosis?A. Occlusive
vasculopathy
B. Infection
C. Lupus-like reaction to TNF inhibitors
D. Psoriasis
E. Lymphoma
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©2011 MFMER | slide-135
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©2011 MFMER | slide-136
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©2011 MFMER | slide-137
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Which organism is this ?
A. Blastomycosis
B. Coccidiomycosis
C. Cryptococcus
D. Histoplasmosis
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Disseminated Histoplasmosis• Cutaneous, pulmonary, and intestinal involvement
• Histoplasma antibody and urine antigen positive
• Fungal blood cultures positive
• All immunosuppressants discontinued
• Responded to treatment with amphotericin X 2 weeks, followed by itraconazole
• Monitor itraconazole levels and Histoplasma urine antigen
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Disseminated Histoplasmosis
• Histoplasma capsulatum
– Grows in soil which contains bird and bat feces
– Endemic in Mississippi and Ohio river basins; Central and South America; Southern Europe; Africa; South and Southeast Asia
• Hematogenous dissemination in patients with suppressed immune systems
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Disseminated Histoplasmosis• Patients may present with ill-defined symptoms
– Fevers, weight loss
• Up to 20% may present in septic shock and multisystem organ failure
• Variable clinical presentation
– Papules & nodules with necrosis & hyperkeratosis; ulcers; bullous EM-like; erysipelas-like; petechiae/purpura; acneiform & folliculopustular papules; vesicles, herpetiform; exfoliative, nummular or psoriasiform dermatitis; morbilliform eruption
• Gold standard for diagnosis
– Tissue cultures
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Pearls• Infection MUST be excluded in an
immunocompromised patient!!!
• Recently described phenomenon of immune reconstitution syndrome– In the setting of disseminated histoplasmosis in
patients who have been on a biologic agent or had reduced cellular immunity
– Patients do well clinically and have therapeutic itraconazole levels with decrease in histoplasma urine antigens
– Develop new skin or LN lesions with negative cultures but organisms still present on biopsy
– Tx with prednisone taper and continue itraconazole
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CASE
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• 3 month boy with
fever, irritability &
rapidly progressing
generalized blisters
over past 24 hours
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©2011 MFMER | slide-145
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©2011 MFMER | slide-146
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What is your diagnosis?
A. Erythema multiforme (EM)
B. Herpes infection
C. Stevens Johnson syndrome/ Toxic epidermal necrolysis (SJS/TEN)
D. Staphylococcal scalded skin syndrome (SSSS)
E. Toxic shock syndrome (TSS)
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Staphylococcal Scalded Skin
Syndrome (SSSS)• Common in infants and children
• Secondary to extracutaneous S. aureus, phage grp II, infection
– Produce exfoliative toxins, bind to dsg 1 leading to acantholysis of the upper epidermis
• Fever, irritability, purulent rhinorrhea and conjunctivitis, painful, tender skin– Periorificial edema and scale crusts
– Confluent erythema, superficial erosions and flaccid bullae, esp. in intertriginous sites
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Treatment
• Throat culture positive for MSSA
• IV antibiotics
– Vancomycin (per ID) – stopped after 5 days, Clindamycin 80 mg IV q8h and Oxacillin 300 mg IV q6h
• Plastibase ointment
• Zinc oxide ointment to groin and neck
• Mupirocin ointment to nares and severe areas
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CASE
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• 7 month male
– Atopic dermatitis
– 24 hours of
explosive
worsening of AD
• VSS
• Non-toxic, happy,
no change in
temperament
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©2011 MFMER | slide-152
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©2011 MFMER | slide-153
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©2011 MFMER | slide-155
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What is your diagnosis ?
A. Eczema Coxsackium
B. Eczema Herpeticum
C. Gianotti Crosti syndrome
D. Secondary bacterial
infection in setting of
atopic dermatitis
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Eczema Coxsackium
• Enterovirus PCR swabs from both tongue and skin positive
• HSV/VZV PCR swab negative
• Bacterial swab culture grew 2+ Staph aureus
• After 30 hrs of wet dressings, he had 75% improvement
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Eczema Coxsackium
• Coxsackievirus A6 infection
• Widespread vesiculobullous exanthem favoring the perioral area, trunk and areas of previous dermatitis or injury as well as classic sites of hand, foot and mouth disease
• Young children
• Summer and Fall
• Spread via fecal-oral and respiratory routes
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• 66 y/o male with no prior medical history
• Blisters of mouth and skin for 1.5 months
• Seen by outside derm: biopsies pemphigus vulgaris
• Prednisone 60 mg x 2 wks, worsening
• Prednisone 80 mg
• Mayo referral
• Admitted for wet dressings/wound care
• Plan for therapy with Rituximab
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©2011 MFMER | slide-161
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DIF: Cell surface staining with IgG and C3;
IIF:1:1280 ICS; DSG1 131 DSG3 203
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©2011 MFMER | slide-165
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Evaluation• CBC, chem 20 panel
• Negative CXR, Hepatitis screen, HIV, syphilis
serologies
• QuantiFERON-TB POSITIVE!!!
• Vaccination status– Influenza and Pneumococcal vaccine: past fall
– Herpes zoster: no
– DTaP: unknown
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What would you do next?A. Continue steroids alone
B. Continue steroids, vaccinate, and wait 4 weeks
to give Rituximab
C. Continue steroids, vaccinate and wait 2 weeks
to give Rituximab
D. Continue steroids, hold vaccination, and give
Rituximab
E. Continue steroids, start mycophenolate mofetil
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Infectious Disease Consult• No signs/symptoms of active disease
latent TB
• Started on isoniazid and pyridoxine x 9 months
• QFT +: likely because of systemic steroids
• Delay Rituximab for at least 2 wks (ideally 4wks)
• Give DTaP
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• Inactivated vaccines are safe; effectiveness might be
lower.
• If vaccination within 2 wks of therapy or while on therapy,
should revaccinate (at least 3 months post-therapy if
immunity restored).
• Live vaccines should be administered ≥ 4 wks prior to
therapy otherwise need wait at least 3 months after therapy.
YES MAYBE NO
Influenza (inactivated) yearly Meningococcal Varicella
Tdap once, Td booster every 10 years Hepatitis A Zoster
Pneumococcal (PCV13 or PPSV23) Hepatitis B MMR
HPV, if < 26 y/o
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The Patient has Purpura!
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Purpura DefinitionsClinical Histologic
Erythema Inflammation of blood vessel walls
Purpura RBC extravasation
Subepidermal vesicles/bullae, ulcers Necrosis, occlusion or destruction of blood vessel walls
• Nonpalpable and palpable purpura– Petechiae – purpuric macules
– Ecchymoses – purpuric patches
• Retiform purpura – livedo reticularis (lace-like) or racemosa (net-like)
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Purpura – Etiopathogenesis
guides the differential diagnosis
Purpura
Immunologic or Allergic
Infectious or Septic Occlusive Bleeding diathesis thrombocytopenia
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CASE
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22 year old male developed leg ulcers
associated with a rash. Intensive wound care
has been initiated. Which of the following
would you recommend in addition?
A. Observation
B. Topical corticosteroids
C. Systemic corticosteroids
D. ACE wraps
E. Surgical consultation for possible revascularization procedure
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LCVImmunofluorescence Findings
• IgM > IgG, C3, & fibrinogen in blood vessels in the superficial dermis
IgM IgG
C3
F
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Immunologic or Allergic
Palpable purpura LCV and its
Variants
HSP or IgA vasculitis
Mixed cryoglobulinemia
Types II and III
Urticarial vasculitis
ANCA-associated vasculitis
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LCV- Etiology
Vasculitis is a clinical sign
Ask what underlies the diagnosis
• Idiopathic (45-55%)
• Infection (15-20%)
• Inflammatory diseases/Systemic diseases (15-20%)– Connective tissue diseases: Lupus erythematosus,
rheumatoid arthritis, Sjögren's syndrome
• Drugs ( 10-15%)– PCN, sulfas, PTU, allopurinol, thiazides
• Malignancy (<5%)
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Evaluation• Possible causes & extent of involvement
– H & P, hemoccult, CBC, UA, ESR, CRP, ANA, ENA, ANCA, complement studies, RF, SPEP, TSH, cryoproteins, hepatitis serologies, thrombophilia workup, pan cx, CXR
• Skin biopsies
– 1 – 2 day old lesion for routine histology
– < 24 hour old macule for DIF
– Do not biopsy ulcers
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HSP, Types II & III cryoglobulinemia, ACNA-
associated vasculitis
Clinical Features
• Similar to LCV
• Involvement above waist
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HSP, Types II & III cryoglobulinemia, ACNA-
associated vasculitis
Clinical Features
• Larger lesions with figurate, retiform, or
stellate shapes
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HSP
• Histologic Findings
– Similar to LCV
• Immunofluorescence Findings
– IgA in blood vessel walls in the superficial dermis in >90%
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Clinical Pearl
When to treat?
• If mild and nonprogressive
– Many do not treat
– Supportive tx, compression
• If painful, severe or rapidly progressive
– Treat!
• Associated with systemic disease
• Associated with ulcerations
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Pitfall
• Treating the ulcer as something other
than vasculitis
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CASE
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The college student was well earlier today.
6 hours ago she developed a bad sore throat and
muscle pains ‘I feel awful’
Now prostrate, febrile, hypotensive, & tachycardic
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The most important & lifesaving
measure initiated was
A. Supportive management alone
B. Intravenous ceftriaxone
C. Systemic corticosteroids
(dexamethasone)
D. Activated Protein C
E. Lumbar puncture
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Clinical Pearl
• Purpura fulminans
– Due to DIC
– Induced by meningococcemia
• Emergency!
– No more than 30 minutes should elapse before the administration of appropriate antibiotics
– Treat empirically, don’t wait for the results of tests
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Pearl• Purpura fulminans? Identify cause of DIC, manage cause
– Think meningococcus or other underlying infection
• Medical emergency requiring intensive supportive management in an ICU
• Dermatologists can be valuable in guiding appropriate diagnosis and management
• Pitfall: Thinking this is LCV and starting systemic corticosteroids
• Purpura fulminans with symmetrical peripheral gangreneis an ominous clinical presentation affecting all age groups
– Outcome: Death/Amputation
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Occlusive
Intraluminal
Thrombosis Embolism
Coagulation disorders Fibrin
Cryoprotein- Type I cryoglobulinemia
Vessel Wall
Degos’ disease or malignant atrophic
papulosis
Livedoid vasculopathy
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Occlusive Vasculitis
Intraluminal• Thrombosis-
Embolism
– Atherosclerosis
– Cholesterol emboli
– Thrombotic
thrombocytopenic
purpura (TTP)
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Occlusive Vasculitis
Intraluminal
Coagulation Disorder - Fibrin• DIC/purpura
fulminans
• Coumadin necrosis
• Heparin necrosis
• Protein C deficiency
• Protein S deficiency
• Antithrombin III
deficiency
• Antiphospholipid
antibody syndrome
• Mutation in factor V
Leiden
• G20210A mutation in
prothrombin gene
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Occlusive Vasculitis
Intraluminal
Coagulation Disorder - Fibrin
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Occlusive Vasculitis
Intraluminal
Coagulation Disorder - Fibrin
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Occlusive Vasculitis
Intraluminal• Cryoproteins
–Cryoglobulins
–Cryofibrinogens
• Type I cryoglobulinemia
– Monoclonal Ig, most commonly IgM
– Associated with lymphoproliferative disorders
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Type I Cryoglobulinemia
Clinical Features
• Purpuric macules & papules
• Small, punched-out ulcers on acral sites
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Type I Cryoglobulinemia
Histologic Features
• Deeply eosinophilic, homogeneous, hyalin, amorphous, PAS+ material in lumina of vessels
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Infectious or Septic Vasculitis
Pathogenesis• Direct infection of blood vessel
wall
• Interaction of the host immune
response with microorganism in
the blood vessel wall
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Infectious Vasculitis
Clinical Variants• Ecthyma gangrenosum (Pseudomonas)
• Gonococcemia (Neisseria gonorrhoeae)
• Bacterial endocarditis
• Rocky Mountain Spotted Fever
(Rickettsia rickettsii)
• Fungal sepsis (Candida, Aspergillus)
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Infectious Vasculitis
Histologic Features • Involvement of deeper vessels
• Prominent thrombosis
• Usually cell-poor infiltrate
• Organisms may be visualized in &
around blood vessel walls in acute but
not chronic septic vasculitis
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Infectious Vasculitis
Histologic Features
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CASE
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A patient on hemodialysis
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A patient on dialysis for chronic renal failure suddenly
develops extraordinarily tender ulcerations involving
legs, thighs and abdominal pannus
Which of the following statements are most accurate
about this condition?
A. Corticosteroids are the treatment of choice.
B. Surgical excision of the affected areas should be immediately performed
C. The prognosis of this condition is dismal, and there is no treatment that consistently works.
D. Sodium thiosulfate leads to a predictable improvement in this condition.
E. Parathyroidectomy will usually be curative.
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Occlusive
Intraluminal
Thrombosis Embolism
Coagulation disorders Fibrin
Cryoprotein- Type I
cryoglobulinemiaCalciphylaxis
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Soft tissue X-ray
• Net-like pattern of
calcification
• 90% specificity
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Clinical Pearls
• Calciphylaxis is multifactorial and usually
fatal
• The prognosis is dismal
• 1-year survival: 46%
• 2-year survival: 20%
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‘One of the worst ways to die’
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Clinical Pearls• Under-recognized syndrome
– Occurs in 4% of hemodialysis patients
– Non uremic cases associated with
• Warfarin therapy, CTD, hematologic
malignancies, DM, primary
hyperparathyroidism, vitamin D deficiency,
protein C and S deficiency, factor V Leiden
deficiency, Crohn disease, and liver disease
• No clearly effective treatments
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Calciphylaxis treatment strategies
Correct calcium-phosphate balance
Sodium thiosulfate
Cinacalcet
Low calcium dialysate
Improve tissue perfusion & oxygenation
TPA
Hyperbaric oxygen
Avoid warfarin for anticoagulation
Wound Care Debridement
Surgical
Whirlpool
Maggot
Pain control
Palliative care
• Multidisciplinary approach
• Mechanism - Thrombotic tissue ischemia; Must address the clot & prevent more
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Before low
dose TPA X 14
days
One month later
6 months later
Calciphylaxis
ESRD from focal segmental GN on HD
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CASE
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• 70 yo M with DM, HTN, hyperlipidemia, peripheral neuropathy, pancytopenia, CAD s/p CABG and MI
• Transfemoral, endovascular aortic valve insertion for CHF secondary to aortic stenosis
– Complicated intraoperatively by cardiac arrest, requiring transcatheter placement of another aortic valve
– Postoperatively, he developed fever, leukocytosis, anemia, and thrombocytopenia (43) followed 4 days later by cerebral infarction
• Consulted for LLE nonpalpable purpura that developed 9 days after the procedure
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What is your diagnosis?
A. Cholesterol emboli
B. Thrombocytopenia
C. Infectious emboli
D. Polymer gel emboli
E. Vasculitis
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©2011 MFMER | slide-229
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What is this material?• Colloidal iron stain established this was
hydrophilic material
• X-ray probe microanalysis of the material• Phosphorus, sulfur and iron
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Hydrophilic Polymer Coating• Purpose:
– Decrease friction
– Reduce arterial spasm & pain
– Reduce thrombosis
• Must be meticulously cleaned and moistened in order to avoid drying out and becoming tacky
• Not to be used for initial vascular access, because passage through entry needles can shear off the hydrophilic coating
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©2011 MFMER | slide-233
“Clinical sequelae ranged from
undetectable (no symptoms) to
renal failure, myocardial infarction,
pulmonary infarction, stroke,
ongoing gangrene, and/or death
occurring within days to weeks of
suspected embolization of foreign
material.”
2010
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https://www.sciencedirect.com/science/journal/10548807
Review Article
Polymer coating embolism from intravascular medical devices — a clinical literature reviewAuthor links open overlay panelAmitabh M.ChopraaMonikMehtabJeanBismuthcMaksimShapirodMichael C.FishbeineAlina G.BridgesfHarry V.Vintersg
https://doi.org/10.1016/j.carpath.2017.06.004Get rights and content
Under a Creative Commons license
open access
Highlights•
Recent literature associates polymer coating embolism with a range of adverse clinical sequelae.
•
The elusive, microscopic phenomenon has been difficult to detect for almost three decades.
•
Major polymer-emboli-related conditions include obstructions of blood flow in small vessels.
•
Polymer emboli incidence is dependent on device coating integrity measured by particulate release.
•
Regulators may default to controlling particulate release from intravascular devices.
•
Research highlights the potential of using low-particulate-release coatings on intravascular devices.
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Hydrophilic Polymer Gel Emboli:
An Epidemic
©2011 MFMER | slide-235
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Occlusive
Intraluminal
Thrombosis Embolism
Coagulation disorders Fibrin
Cryoprotein-Type I
cryoglobulinemiaCalciphylaxis
Hydrophilic gel coating
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Pearls:
Hydrophilic Polymer Gel Emboli• Can embolize to the skin and cause
microvascular occlusion presenting as purpura, livedo racemosa, livedo reticularis, hemorrhagic panniculitis, or ulceration
• Recent interventional procedure
• Treatment is supportive
• Lesions gradually improve
• Be aware of internal organ involvement of emboli
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Yikes!
The flesh eating ulcer
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58-year-old woman with multiple ulcerations
and abscesses involving both breasts x 5
months• Started after breast reduction surgery for
pendulous breasts
• Ulcerations began at the incision sites 3 weeks postoperatively
• Multiple admissions to plastic surgery for incision and drainage of breast abscesses
• Several admissions to infectious diseases service for intravenous antibiotics
• Ulcerations have progressed
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The treatment that led to resolution of
these abscesses was: A. Readmission of patient for incision and
drainage of abscesses
B. Bilateral mastectomy
C. Long-term suppressive oral antibiotic treatment
depending on antimicrobial susceptibilities
D. Oral prednisone
E. Skin grafting of ulcerations
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Discussion
Pyoderma Gangrenosum• There are many weird causes of
ulcerations
• Weirdest of all: pyoderma gangrenosum
• Clues in this patient:
– Irregular, violaceous undermined border
– Pathergy (triggered by trauma, surgical debridement, and attempts to graft)
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Clinical Pearls• Pyoderma gangrenosum can occur
anywhere on body including the breasts
• Characteristic clinical presentation– Rapidly progressive ulcerative process– Begins as a small pustule which breaks down
forming an ulcer– Satellite papules may appear at the border,
break down, and then fuse with the central ulcer
– Fully developed: painful, undermined ulcer with blue/purple border
– Heals with atrophic scar
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CP1133683-121
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Associated conditions
• 50% have no underlying cause
• Associations (50%)
– IBD - Most common(1.5%-5% of IBD
patients get PG)
–RA, seronegative arthritis (>1/3 PG
pts have arthritis
–Hematologic abnormalities,
malignancies
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Edge of the ulcer, dense infiltrate of PMN undermining the epidermis.
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All special stains and tissue cultures fail to reveal infection… c/w
pyoderma gangrenosum.
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CASE
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• 59 yo woman admitted
with 1 yr. history of
expanding leg ulcers
• On prednisone,
cyclosporine and
azathioprine for
presumed diagnosis of
pyoderma
gangrenosum
• No biopsy
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What is your diagnosis?
A. Pyoderma gangrenosum
B. Lymphoma
C. Infection
D. Occlusive vasculopathy
E. Calciphylaxis
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Sporotrichosis
Admission 4 months 1 year
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Bottom Line with PG
• Diagnosis of exclusion
• BUT! It’s important to do our best since
treating a patient for PG when they DON’T
have it with steroids and/or
immunosuppression has it’s own set of
associated risks, and can certainly
aggravate the actual condition
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Common diseases misdiagnosed as PG• Vascular occlusive disease
• Chronic venous disease
• Vasculitis
• Neoplasm
• Exogenous tissue
injury/Factitial Disease
• Spider bite
• Infection
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Lessons learned
• Pyoderma gangrenosum can be
underdiagnosed
• Pyoderma gangrenosum can be
overdiagnosed
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Summary: Pearls and Pitfalls in Hospital
Dermatology
• Erythroderma
• Pruritus
• Drugs – Simple, Complex, Misc
• BUGS: Infection and infection prevention
• Purpura
• Neutrophilic dermatoses – PG
• Handout
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Comments/questions?