11/5/2012
Disclosures
My Fingers Are Blue:
Benign or Worrisome?
• I’m a rheumatologist
• “I have no actual or potential conflict on
interest in relation to this program.”
Mark F. Hoeltzel, MD
Division of Rheumatology
Children’s Mercy Hospitals & Clinics
November 15, 2012
Clinical Advances in Pediatrics
Episodic Cold Blue Fingers in
Adolescents
Objectives
• By 1pm, the learner (that’s you) will be able
to…
– appropriately triage a teenager with discolored
fingers, including work up and referral
– distinguish
di ti
i hb
between
t
b
benign
i di
digital
it l color
l changes,
h
and those more concerning for a larger disease
process
– list at least three treatment strategies for
Raynauds Phenomenon
– shamelessly admit that you’ve always secretly
wanted to be a rheumatologist
Then...
• Differential Diagnoses (most common to
least common)
– Benign Acrocyanosis
– Raynaud
Raynaud’s
s Phenomenon
– Chillblains = Pernio
– (Frostbite)
– (Erythromylalgia)
“Classic” Raynauds
• Well demarcated triphasic color changes
– White (Vasospasm)
– Blue/Gray, Purple, Black (O2 extraction,
ischemia)
– Red, warm, swollen, stinging (Reperfusion)
Acrocyanosis
Raynauds
• In the hand, only involves the fingers, never
thumb, and never proximal to the MCPs
• Episodic, triggered by cold, stress
• May be accompanied by pain, numbness,
parathesias
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11/5/2012
Raynauds
Benign Acrocyanosis
•
•
•
•
More generalized blue/purple discoloration
May involve fingers, thumbs, hand, up into wrist
Episodes less distinct, and often persistent
Triggers less apparent, although usually
exacerbated
b db
by cold.
ld
• Associated with slow capillary refill and sometimes
hyperhidrosis or livedo reticularis (mottling)
• Other than feeling cold, typically asymptomatic
Benign Acrocyanosis
Pathophysiology
• Two hits
– Peripheral vasospasm in response to cold
– Dysregulation of determinants of vascular tone
•
•
•
•
•
•
Autonomic stimuli
Circulating catecholamines
Response characteristics of vascular smooth muscle
Injury
Vasoactive drugs
Autoimmune disease?
Now…
“Most things in medicine are very black and
white… Everything that is gray is referred
to rheumatology clinic.”
-Anonynmous
Anonynmous
Acrocyanosis
Raynauds
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11/5/2012
Raynauds Phenomenon
•
•
•
•
•
•
A vasospastic phenomenon
Described by Raynaud in 1862
15% of children (self-reported)
80% female
Mean age of onset 12.3 +/- 4.3 years
Mean age of diagnosis 13.4 +/- 4 years
Raynauds Phenomenon
Raynauds Phenomenon
• Triggered by cold, stress
• May be associated with CNS stimulants
and malnutrition (anorexia, bulimia)
• 46% monophasic,
h i 32% bi
biphasic,
h i 22%
triphasic
– No difference in this ratio between primary
and secondary RP
Primary Raynauds
• No associated systemic disease
• Most common variant in kids (69%) and
adults (80%)
Secondary Raynauds
• More likely to have ischemic damage or
nailfold capillary changes
• More likely associated with positive ANA
• Mean
M
time
ti
between
b t
RP and
dd
development
l
t
of disease 2-6 years
Raynauds Phenomenon
• Physical Exam
– Normal (most
common in
p
primary)
y)
– Periungual
capillary changes
– Cuticle staining
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11/5/2012
RP – Physical Exam
• Tapering of fingers due to loss of
subcutaneous pulp
• Finger pitting
Associated Diseases
• Scleroderma**
– Sclerodactyly with shiny, thick or atrophic skin
and flexion contractures
– SOB, reflux, dysphagia, sub cutaneous calcinosis
– Associated with +ANA,
ANA, anti-SCL70
anti SCL70 ab (dcSSC),
anti-centromere ab (lcSSC, aka CREST)
• Dermatomyositis
– Muscle weakness, fatigue, Gottron’s papules
• Mixed Connective Tissue Disease
• Lupus
• JIA/RA
Benign Acrocyanosis
•
•
•
•
•
•
•
Benign Acrocyanosis
Slowed blood flow
Common in adolescent girls
Episodic or persistent
Exacerbated by cold or stress
Thought to be due to an exaggerated vasomotor response
Usually asymptomatic
Associated with
– CNS stimulants
– malnutrition
– smoking
• Other signs of autonomic dysfunction
– Hyperhidrosis, livedo reticularis, orthostatic hypotension
Approach to Color Change
• All patients:
– Thorough history and physical exam looking
for red flags
– Consider CBC
CBC, CK
CK, ANA
ANA, TSH
TSH, T4
• If no color changes during evaluation, ask
for pictures and follow-up
• If any Red Flags, then refer to
Rheumatology!
Cold Blue Fingers
• Red Flags
–
–
–
–
–
–
–
–
–
–
–
–
Pain
Numbness
Tri-phasic color changes
Sores or pits on finger tips
Well demarcated color changes involving fingers only
Swelling
Nodules
Periungual capillary changes
Cuticle staining
Skin tightening
Loss of normal range of motion of the fingers
Positive ANA
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Approach to Suspected Benign
Acrocyanosis
Cold Blue Fingers
• Reassuring
– Excessive sweating
– No white color change
– Involves more than just digits
– Normal physical exam
– Normal Labs
•
•
•
•
•
Reduce cold exposure
Increase water intake (and salt)
Trial off ADHD meds(?)
Address malnutrition concerns
Educate about Raynauds phenomenon,
and follow-up once in 1-3 months for
repeat evaluation
• Pictures!
Treatment of Symptomatic
Raynauds
• Cold precautions
– central and peripheral (layers, hats, hand
warmers)
Refer to Rheumatology
• Any red flags
– Symptomatic
– Abnormal physical exam
– Abnormal labs
labs…
• Discontinue ADHD meds
• Medical Therapy:
–
–
–
–
Calcium channel blockers
Nitro-paste
SSRI’s
Viagra, Cealis
• When in doubt, call or refer!
• Surgical Therapy
– Surgical sympathectomy
Pernio (aka Chilblains)
• Localized inflammatory skin lesion resulting from abnormal
response to cold (non-freezing cold)
• Fingers (proximal, dorsal), toes
• Single or multiple erythematous or violaceous edematous lesions
Pernio (aka Chilblains)
•
•
•
•
Young women (thin body habitus)
Rare
Intense pain, itching, burning
Typically resolves in 1-2 weeks, but may be
chronic
h i
• Can be associated with
autoimmune disease (lupus)
• Treatment supportive,
although nifedipine has been
shown to be helpful
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Frost Bite
• Literal freezing of tissue with resultant
necrosis
Summary
• The most common digital color changes in
adolescents are benign acrocyanosis and
Raynauds phenomenon
• These two entities appear to fall on the same
spectrum
• The majority of cases are benign, but maybe
associated with or the initial presentation of
autoimmune disease (secondary RP)
• Abnormal physical exam findings and
abnormal labs are predictive of autoimmune
disease (particularly scleroderma)
Summary
• Work up should include labs (consider
CBC, CK, ANA, TSH, T4)
• Treatment strategies include cold
avoidance drug withdrawal,
avoidance,
withdrawal vasodilatory
medications, and surgery
• Referral to rheumatology should be
considered, especially if “red flags”
present
References
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Goldman W, et al. Association Between Treatment With Central Nervous System Stimulants and
Raynaud’s Syndrome in Children. Arthritis & Rheumatism 58(2):563-566, 2008.
Heidrich H. Functional Vascular Diseases: Raynaud’s Syndrome, Acrocyanosis, and Erythromelalgia.
VASA 39:33-41, 2010.
Jones GT, et al. Occurrence of Raynaud’s Phenomenon in Children 12-15 Years. Arthritis &
Rheumatism 48(12):3518-3521,2003.
Kurklinsky AK, et al. Acrocyanosis: The Flying Dutchman. Vasc Med 16(4):288-301, 2011.
Nigrovic PA, et al. Raynaud’s Phenomenon in Children: A retrospective Review of 123 Patients.
Pediatrics 111(4):715-721, 2003.
Ojha
j A, et al. Comorbidities in Pediatric Patients with Postural Orthostatic Tachycardia
y
Syndrome.
y
J
Pediatr 158:20-23, 2011.
Pavlov-Dolijanovic S, et al. The Prognostic Value of Nailfold Capillary Changes for the Development of
Connective Tissue Disease in Children and Adolescents with Primary Raynaud Phenomenon: A Follo-up
Study of 250 Patients. Ped Derm 23(5):437-442,2006.
Schulze UME, et al. Dermatologic Findings in Anorexia and Bulimia Nervosa of Childhood and
Adolescence. Pediatric Dermatology 16(2):90-94, 1999.
Simon TD, et al. Pernio in Pediatrics. Pediatrics 116(3):e472-e475, 2005.
Stewart M, Morling JR. Oral Vasodilators for Primary Raynaud’s Phenomenon. Cochrane Database
Syst Rev. 2012.
Syed RH, Moore TL. Methylphenidate and Dextroamphetamine Induced Peripheral Vasculopathy. J Clin
Rheumatol 14:30-33, 2008.
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