seeing the signs: visual recognition of autoimmune
TRANSCRIPT
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Seeing the Signs: Visual Recognition of
Autoimmune Connective Tissue Diseases
Utah Association of Family Practitioners CME Meeting at
Snowbird, UT 1:00-1:30 pm, Saturday, February 13, 2016
Rick Sontheimer, M.D.Professor of Dermatology
Univ. of Utah School of Medicine
Snowbird/Alta
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Potential Conflicts of Interest2016
• Consultant– Centocor (Remicade-
infliximab)
– Genentech (Raptiva-efalizumab)
– Alexion (eculizumab)
– MediQuestTherapeutics
– P&G (ChelaDerm)
– Celgene*
– Sanofi/Biogen*
– Clearview Health* Partners
• 3Gen – Research partner
• Paid speaker– Winthrop (Sanofi)
• Plaquenil(hydroxychloroquine)
– Amgen (etanercept-Enbrel)
– Connetics/Stiefel
• Royalties– Lippincott,
Williams
& Wilkins*
*Active within past 5 years
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Learning Objectives
• Compare and contrast the presenting and
Hallmark cutaneous manifestations of lupus
erythematosus and dermatomyositis
• Compare and contrast the presenting and
Hallmark cutaneous manifestations of morphea
and systemic sclerosis
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Distinguishing the Cutaneous
Manifestations of LE and DM
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Skin involvement is
2nd most prevalent
clinical manifestation
of SLE
and
2nd most common
presenting clinical
manifestation
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Comprehensive List of Skin Lesions Associated with LE
LE-SPECIFIC
Acute Cutaneous LE
Localized ACLE
Generalized ACLE
Ten-like ACLE
Subacute Cutaneous LE
Annular
Papulosquamous
Mixed patterns
Chronic Cutaneous LE
"Classical" DLE
Localized
Generalized
Hypertrophic DLE
LE profundus
Mucosal DLE
LE tumidus
Chilblains LE
DLE-lichen planus overlap
LE-NONSPECIFICCutaneous vascular disease
Vasculitis
Leukocytoclastic
Palpable purpura
Urticarial vasculitis
Periarteritis nodosa-like
Vasculopathy
Dego's disease-like
Atrophy blanche-like
Periungual telangiectasia
Livedo reticularis
Thrombophlebitis
Raynaud's phenomenon
Erythromelalgia (erythermalgia)
Alopecia (nonscarring)
"Lupus hair"
Telogen effluvium
Alopecia aerata
Sclerodacytly
Rheumatoid nodules
Calcinosis cutis
LE nonspecific bullous lesions
Epidermolysis bullosa acquisita-like bullous LE
Dermatitis herpetiformis-like bullous LE
Pemphigus erythematosus
Bullous pemphigoid
Porphyria cutanea tarda
Urticaria
Papulo-nodular mucinosis
Nail changes (red lunulae, dyschromasia)
Cutis laxa/anetoderma/mid-dermal elastolysis
Pigmentary changes
Acanthosis nigricans (Type B insulin resistance)
Erythema multiforme (Rowell’s syndrome)
Leg ulcers
Lichen planus
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Primary Skin Change of
Cutaneous LE
Photosensitive macular
erythema and/or
papulosquamous papules and
plaques
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a
LE-Specific Skin Disease
Interface Dermatitis
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Acute Cutaneous LE (ACLE)
Localized
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Butterfly Pattern Red Face
Differential Dx
• ACLE/SCLE
• Acne rosacea
• Contact dermatitis (photo, airborne)
• Photosensitive drug eruptions
• Seborrheic dermatitis
When the diagnosis is in doubt, punch it out!
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ACLEGeneralized
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LE DM
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Acute Cutaneous LE
• Very photosensitive, non-scarring
• (+) ANA, a-dsDNA, a-Sm
• Strong association with active SLE
• Usually managed by rheumatologists,
internists
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Subacute Cutaneous LE (SCLE)
Papulosquamous Annular
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SCLE
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Histopathology of SCLE
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Direct immunofluorescence
microscopy exam of SCLE
Dust-like pattern of IgG at dermal-
epidermal junction
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Drug-Induced SCLE• DIURETICS
– Thiazides
– Spironolactone
• CALCIUM CHANNEL BLOCKERS– Diltiazem
– Nifedipine
– Nitrendipine
– Verapamil
• ACE inhibitors– Captopril
– Cilazapril
• Acid Blockers– Ranitidine
– Omeprazole
• NSAIDS– Naproxen
– Piroxicam
• Beta Blocker– Oxprenolol
– Acebutolol
• Lipid lowering– Pravastatin
– Simvastatin
• ANTIMICROBIALS– Griseofulvin
– Terbinafine
• ANTIHISTAMINES– Cinnarazine/triethylperazine
• Anti-seizure– Phenytoin
• Antimalarials– Hydroxychloroquine
• Sulfonylureas– glyburide
• Chemotherapy– Taxotere/tamoxifen
• Others– Leufonamide
– INF-a
– Procainamide
– Inhalants• Insecticides/fertilizer
• Tiotropium
– d-penicillamine
– Etanercept/infliximab
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Drug-induced SCLE Reports: 2011-Present
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SCLE – A Snapshot
• Nonscarring, highly photosensitive
• ANA (+); anti-Ro/SS-A & anti-La/SS-B
• Genetic associations– 8.1 ancestral haplotype
• A*01, B*08, DRB1*0301, DQB1*0201, TNFAB* a2b3 (TNF-α -308A), C2*C, C4 null
– C1QA-Gly70GGG/A
• Associations: SSj, neonatal LE
• Can be drug-induced
• Approximate 10% - severe SLE
• ~75% respond to antimalarials
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Classical Discoid LE - Localized
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Classical Discoid LE - Localized
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DLE – Activity/Damage
Active Inactive (Damage)
Erythema, Adherent scale
Induration, Follicular
plugging
Pigmentary change,
Atrophy, Telangiectasia
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Is It DLE or SCLE?
Active
Induration? Yes-DLE
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DLE? When in DoubtCheck the Ears
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Classical Discoid LE - Generalized
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Classical Discoid LE- Generalized
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Chronic Cutaneous LE Variations on the Theme
• Hypertrophic/verrucous LE
– Hyperkeratosis (SSCA-like)
• LE tumidus
– Urticaria-like plaques
• LE panniculitis
– Subcutaneous nodules
• Chilblains LE
– Acral vascupopathic changes (fingers/toes)
– Familial cases associated with TREX1 gene
mutations
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Chronic Cutaneous LE“Classical DLE”
• Typically scarring, less photosensitive
• (-) ANA
• 20-30% of SLE patients get DLE
lesions
• < 5% presenting with isolated localized
DLE for 1-2 years develop SLE;
somewhat higher with generalized DLE
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Dermatomyositis
A member of the idiopathic
inflammatory myopathies (IIM)
that produces unique patterns of
inflammatory injury to skin and/or
proximal skeletal muscles
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Skin Disease in Dermatomyositis
• Disease-defining (Hallmark) inflammatory skin changes (with interface dermatitis)• Constant, defining
• Miscellaneous associated
– Often rare
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Miscellaneous, Less Commonly
Encountered Skin Changes in DM
• Acquired icthyosis
• Erythroderma
• Facial swelling without erythema
• Follicular hyperkeratosis
• Hypertrichosis
• Lichen planus
• Linear IgA bullous dermatosis
• Acquired lipoatrophy
• Malakoplakia
• Malignant erythema (suffusion)
• Mechanic’s hand
• Mucous membrane lesions
• Mucinous plaques of the palmar creases
• Mucinosis, cutaneous
• Nasal septal perforation
• Panniculitis
• Pityriasis rubra pilaris
• Steroid-induced acanthosisnigricans
• Urticaria, urticarial vasculitis
• Vasculopathic ulcers
• Vulvar and scrotal involvement
• Zebra-like stripes (centripetal flagellate erythema)
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Hallmark skin disease
seen in
Classical DM or CADM
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Primary Skin Change of DM
Heliotrope rash -- No
Confluent macular violaceous erythema
Can be more difficult to discern in darkly
pigmented individuals
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Heliotrope
rash
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B
DM Skin Biopsy Similar to LE-
Specific Skin Disease Biopsy
Lupus band
typically
negative in
DM
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Pruritic Violaceous Scalp Erythema
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V-Sign
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Shawl Sign
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Pruritic Macular Violaceous Erythema
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Göttron’s Sign
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Göttron’s Papules
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Göttron’s Papules
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Periungual telangiectasia
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DM Normal
*
** *
B
C
D
*
Fig. 4
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Holster Sign
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Poikiloderma
atrophicans vasculare(POIKILODERMA-
hyperpigmentation,
hypopigmentation,
telangiectasis, atrophy)
Gottron’s papule-like
lesion over medial
malleolus of ankle
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Spectrum of the Idiopathic
Inflammatory Myopathies
Skin Involvement
Muscle Involvement
Amyopathic DM Polymyositis/
Inclusion Body Myositis
Classical DM
Hypomyopathic DM
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Skin Disease Activity
Clinically-Evident Muscle
Disease Activity
6 months 24 monthsDisease
onset
CDM
Classical DM (60%)
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Skin Disease Activity
Clinically-Evident Muscle
Disease Activity
6 months 24 months
CDM
Disease
onset
Classical DM (30%)
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Skin Disease Activity
Clinically-Evident Muscle
Disease Activity
6 months 24 months
C-ADM
Disease
onset
Clinically-Amyopathic DM (C-ADM)
( ~20% of all DM)
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Clinically-Amyopathic DM
Skin Involvement
Muscle Involvement
Amyopathic DM
Hypomyopathic DM
• CADM is more common than previously thought
• Finite risk of interstitial lung disease, internal malignancy, late-
onset weakness (predictors unknown)
• ANA (+), myositis-specific antibody (-), may have specific
immunogenetic association (MDA-5/CADM140)
Clinically-Amyopathic DM (CADM)
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Management of DM
• Adult-onset DM
– Screen for internal malignancy (greatest risk [25%]
beyond 50 years of age)
• Sex- and age-specific approach
– Screen for interstitial lung disease
• Baseline - pulmonary function tests with diffusion capacity
(PFT) (25% abnormalities)
• Persistent dry cough, dyspnea – repeat PFT, consider referral
to pulmonary medicine/rheumatology
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Management of DM
• Juvenile-onset DM
– Monitor for vasculopathic tissue damage
• Eye, GI tract,
– Monitor for calcinosis cutis
– No significant risk for interstitial lung disease or
internal malignancy
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DM Autoantibodies
• ANA (50-70%)
• Myositis-specific autoantibody (10-20%)
– Jo-1, PL-7, Pl-12 (anti-synthetase antibody
syndrome [myositis, arthritis, Raynaud’s
phenomenon, mechanics hand lesion])
• New autoantibodies (10-30%)
– Melanoma differention antigen 5 (MDA5)
(interstitial lung disease association)
– Transcription intermediary factor 1γ (TIF1γ ) &
Nuclear matrix protein (NXP-2) (internal
malignancy association)
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Mechanic’s Hand
Lesion
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Management of DM
“DM” Is No Longer Enough
Dermatomyositis
Phenotypes
Adult-Onset
Juvenile-Onset
Classic DM Clinically-Amyopathic DM
Classic DM Clinically-Amyopathic DM
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Distinguishing the Cutaneous
Manifestations of Morphea form
Systemic sclerosis
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Scleroderma
Hardened, thickened, hide-bound skin
associated with autoimmune
microvascular injury and replacement of
the dermis and subcutaneous tissue by
dense fibrotic tissue
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Scleroderma Skin Biopsy
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Scleroderma Morphea (Localized Scleroderma)
Systemic Sclerosis
Skin Involvement
Systemic Involvement
Localized
Scleroderma/Morphea SSc siné scleroderma
Systemic Sclerosis
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Scleroderma Skin Biopsy
Same in morphea and systemic sclerosis
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Cutaneous Sclerosis
“Scleroderma”
Localized Scleroderma
(syn. Morphea)
Systemic Sclerosis (SSc)
Pseudoscleroderma
Limited cutaneous
sclerosis (CREST)
Diffuse cutaneous
sclerosis
Plaque GeneralizedLinear Profunda/Deep
Scleroderma variants:
eosinophilic fasciitis,
eosinophilia myalgia
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Presenting Manifestations
of Morphea
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Plaque Morphea
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Linear Scleroderma/
Liner Morphea
En Coup de Sabre
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Generalized
Morphea/
Pansclerotic
morphea
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Systemic Sclerosis
vs.
Morphea
• Systemic Sclerosis– Presents with Raynaud’s phenomenon
– Nailfold telangiectasia
– Sclerodacytly
– SSc antibodies• Centromere
• Scl-70 (Topoisomerase I)
• Localized Scleroderma/Morphea– Absence of the above
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Presenting Manifestations
of Systemic Sclerosis
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Raynaud’s Phenomenon
Three colors in
sequence:
1. White
(vasconstriction)
2. Blue
(cyanosis)
3. Red
(reactive
hyperemia)
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Raynaud’s Phenomenon
• Primary Raynaud’s phenomenon
(syn. Raynaud’s disease)
• Absence of:
–Periungual microvascular changes
– Sclerodactyly
– SSc autoantibodies• ANA, centromere, topoisomerase-1 (Scl-70)
• Secondary Raynaud’s phenomenon
– SSc
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Periungual
TelangiectasiaSystemic Sclerosis &
Dermatomyositis
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Scleroderma in SSc-Cutaneous Manifestations-
• Raynaud’s phenomenon
• Edema
• Microvasculopathy
• Cutaneous sclerosis (“scleroderma”)
• Matte-like telangiectasis
• Atrophy
• Pigmentary changes
• Calcinosis cutis
• Ulceration
Time
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Edema
Peau d’orange
Sausage Shaped Digits
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Periungual
Telangiectasia
(Microvasculopathy)
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Ptyrigium Inversus Unguium
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Sclerosis
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Sclerosis & Atrophy
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Sclerosis & Atrophy
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Atrophy Autoamputation
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Cutaneous Calcinosis
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Pigmentary Change
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Telangiectasia
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Ulceration
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Systemic Sclerosis
• Two prognostic groups
– SSc with limited cutaneous sclerosis
(CREST syndrome)
• Slower pace, milder course initially
• Risk for pulmonary artery hypertension 15-
20 years later
– SSc with generalized cutaneous
sclerosis
• Rapid pace, severe systemic disease
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(CREST Syndrome)
C - Calcinosis
R - Raynaud’s
E - Esophageal
dysmotility
S - Sclerodactyly
T - Telangiectasia
SSc with limited
cutaneous
sclerosis
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CREST SyndromePrognosis
• Low risk
– Kidney, gastrointestinal tract
• High risk
– Pulmonary and cardiovascular system
• Pulmonary artery hypertension starting 15-
20 years after disease onset
– Presenting clinical symptoms-shortness of
breath/dyspnea on exertion
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SSc with Generalized Cutaneous Sclerosis
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Wasatch Front,
Salt Lake City
Guardsman’s Pass looking
down toward Heber Valley
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LE DM
For Dermatologists
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Distinguishing Cutaneous
LE from Cutaneous DM
• Biopsy unhelpful
• Regional anatomy predilection
– Pruritic scalp involvement
– Face – Nasolabial folds
– Fingers – Knuckles vs hair-bearing
• Finger nail fold microvascular changes
• Pruritus - Presence/absence
• Muscle inflammation
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For Non-Dermatologists
Greenwald’s Law of LupusGreenwald RA. Greenwald's law of lupus. J
Rheumatol. 1992 Sep;19(9):1490.
“…anything happening to a patient
with SLE which is not immediately
otherwise explicable will automatically
be blamed on the lupus, regardless of
pathophysiologic validity."
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LE-Specific Skin Disease
Acute Cutaneous LE
Subacute cutaneous LE
Chronic Cutaneous LE
LE-Nonspecific Skin
Disease
LCV
Urticarial vasculitis
Livedo reticularis
Secondary to Treatment of
LE
Steroid acne
Drug eruptions
Opportunistic Infections
True-True and Unrelated
Psoriasis
Eczema
BCC
Greenwald’s Law of Lupus
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Recurrent tinea corporis in a man with
terbinafine-induced SCLE was confused
with reactivation of SCLE
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Univ. of Utah Practical Dermatology
for Primary Care CME Course
7th Annual Course
• What – Intensive one-day course on Dx and Rx of
skin problems commonly encountered in the
primary care setting. Includes podium
presentations and a hands-on skin biopsy workshop
• When - Friday, April 8, 2016
• Where - Alumni Hall, Medical Education Building
• For more information: [email protected]
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Thank You
East Canyon, Fall 2013