CalcinosisCalcinosis Cutis: Cutis:
Emerging Trends in Emerging Trends in
DermatoradiologyDermatoradiology
Peter Peter LioLio, Harvard Medical School Year, Harvard Medical School Year-- IVIV
Gillian Lieberman, MDGillian Lieberman, MD
September 2000
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““Routine CXR”…Routine CXR”…
Special Thanks to Dr. Hochman for this image.
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What is What is CalcinosisCalcinosis??
CalcinosisCalcinosis refers to pathologic calcification refers to pathologic calcification
of the skin and soft tissues.of the skin and soft tissues.
It occurs in a variety of systemic and It occurs in a variety of systemic and
localized conditions.localized conditions.
The deposits are generally composed of The deposits are generally composed of
calcium phosphate crystals which is why we calcium phosphate crystals which is why we
can see them on can see them on radiograpicradiograpic imaging.imaging.
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Classical DermatologyClassical Dermatology
In classical In classical
dermatology, the dermatology, the
skin lesion is skin lesion is
correlated with correlated with
pathologic findings pathologic findings
in the skin.in the skin. http://www.dermis.net/bilddb/diagnose/englisch/i709330.htm
http://www.autopsydb.org/dermpath.htm
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““DermatoradiologyDermatoradiology””
In In dermatoradiologydermatoradiology, the nature of the , the nature of the
heavy metal Calcium and its salts allow for heavy metal Calcium and its salts allow for
radiographic visualization. radiographic visualization.
http://www.rad.washington.edu/maintf/cases/unk39/answers.html
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Classic DermatologyClassic Dermatology
13 year old female 13 year old female
with firm, whitewith firm, white--
yellow nodules on yellow nodules on
an an erythematouserythematous
base in her thumb.base in her thumb.
From: http://www.dermis.net/bilddb/diagnose/englisch/i709330.htm
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DermatoradiologyDermatoradiology
Note the areas of Note the areas of
subcutaneous subcutaneous
calcifications, calcifications,
especially at the first especially at the first
and third phalanges.and third phalanges.
This radiograph is This radiograph is
from a different from a different
patient but you get the patient but you get the
idea...idea...
From: http://www.rad.washington.edu/maintf/cases/unk39/answers.html
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CalcinosisCalcinosis: The Breakdown: The Breakdown
Metastatic calcificationsMetastatic calcifications
Result from persistent Result from persistent
hypercalcemiahypercalcemia or or
hyperphosphatemiahyperphosphatemia
Ca x PO4 > 70Ca x PO4 > 70
Dystrophic calcificationsDystrophic calcifications
Follow a local Follow a local
metabolic or metabolic or
degenerative tissue degenerative tissue
abnormalityabnormality
Ectopic calcification (i.e. calcification outside of bone) is Ectopic calcification (i.e. calcification outside of bone) is
generally classified into two groups:generally classified into two groups:
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DescriptorsDescriptors
CalcinosisCalcinosis may be may be
isolated to a small isolated to a small
area:area:
CalcinosisCalcinosis
CircumscriptaCircumscripta
CalcinosisCalcinosis may be may be
diffuse:diffuse:
CalcinosisCalcinosis
UniversalisUniversalis
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PathophysiologyPathophysiology
Poorly understood…Poorly understood…
Theories include:Theories include:
Local tissue injury which Local tissue injury which
increases cell membrane increases cell membrane
permeability, thus allowing permeability, thus allowing
cytosoliccytosolic influx of sufficient influx of sufficient
calcium to exceed the capacity calcium to exceed the capacity
of mitochondria to sequester of mitochondria to sequester
calcium and phosphate, and calcium and phosphate, and
leading to the precipitation of leading to the precipitation of
cytosoliccytosolic CaPO4.CaPO4.
Local elevations in Local elevations in
alkaline alkaline phosphatasephosphatase
activity which lead to activity which lead to
hydrolysis of extracellular hydrolysis of extracellular
pyrophosphates that pyrophosphates that
normally inhibit calcium normally inhibit calcium
deposition.deposition.
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Differential Diagnosis (Differential Diagnosis (DdxDdx))
Dystrophic (95Dystrophic (95--98%)98%)
Metastatic Calcification (1Metastatic Calcification (1--2%)2%)
Idiopathic Idiopathic TumoralTumoral CalcinosisCalcinosis (<1%)(<1%)
Metastatic Metastatic OsteosarcomaOsteosarcoma (<<1%)(<<1%)
11°° Soft Tissue Soft Tissue OsteosarcomaOsteosarcoma (<<1%)(<<1%)
HeterotopicHeterotopic OssificationOssification
CalciphylaxisCalciphylaxis
Calcium Pyrophosphate Calcium Pyrophosphate DihydrateDihydrate Deposition Disease Deposition Disease
CPPD(1CPPD(1--2%)2%)
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DdxDdx Dystrophic CalcificationDystrophic Calcification
((pneumonic)VINDICATEpneumonic)VINDICATE::
VascularVascular
Venous insufficiencyVenous insufficiency
InfectionInfection
Parasites*Parasites*
NeoplasmNeoplasm
Primary bonePrimary bone--forming tumorforming tumor
Tumor necrosisTumor necrosis
DrugsDrugs
Vitamin DVitamin D
AutoimmuneAutoimmune
DermatomyositisDermatomyositis
SclerodermaScleroderma
SLESLE
TraumaTrauma
**“Rice grain” = cysticercosis
** Small crecentic = dracunculiasis
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Differential Diagnosis: A Differential Diagnosis: A
Radiologist’s PerspectiveRadiologist’s Perspective
Idiopathic Idiopathic TumoralTumoral CalcinosisCalcinosis
MassMass--like like calcificcalcific deposits about joints deposits about joints
Usually normal or slightly elevated serum Usually normal or slightly elevated serum
calcium and phosphate calcium and phosphate
CalcinosisCalcinosis universalisuniversalis
Associated with scleroderma or Associated with scleroderma or
dermatomyositisdermatomyositis
Calcification is usually thin, plaqueCalcification is usually thin, plaque--like and in like and in
skin and subcutaneous tissues skin and subcutaneous tissues
No large, lobular massesNo large, lobular masses
CalcinosisCalcinosis circumscriptacircumscripta
40% are associated with scleroderma, 40% are associated with scleroderma,
dermatomyositisdermatomyositis or or Raynaud'sRaynaud's
Calcifications are thin, and occur in fingertips Calcifications are thin, and occur in fingertips
and "toeand "toe--tips" tips"
MilkMilk--alkali syndrome alkali syndrome
Large, calcified Large, calcified periarticularperiarticular masses masses
Calcification also of lung, kidneys, vessels Calcification also of lung, kidneys, vessels
Metastatic calcification (hyperparathyroidism Metastatic calcification (hyperparathyroidism
and chronic renal disease) and chronic renal disease)
Serum PTH and calcium levels abnormal Serum PTH and calcium levels abnormal
Soft tissue calcifications are usually fine and Soft tissue calcifications are usually fine and
punctatepunctate
HeterotopicHeterotopic ossification ossification
Often associated with some predisposing Often associated with some predisposing
condition condition
Prior musculoskeletal trauma Prior musculoskeletal trauma
Prior surgery Prior surgery
Scleroderma Scleroderma
Usually thin calcifications of hands, feet Usually thin calcifications of hands, feet
DermatomyositisDermatomyositis
Usually associated with fine, reticular Usually associated with fine, reticular
calcifications calcifications
http://www.rad.washington.edu/maintf/cases/unk39/answers.html
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Menu of radiologic tests for Menu of radiologic tests for
imaging ectopic calcificationsimaging ectopic calcifications
Radiographic plain filmRadiographic plain film
CT scanCT scan-- most sensitivemost sensitive
RN bone scanRN bone scan
MRIMRI-- sometimessometimes
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PLAIN FILMSPLAIN FILMS
PolymyositisPolymyositis
Dystrophic Dystrophic calcinosiscalcinosis: :
Plain film of the hand of a Plain film of the hand of a
woman with woman with polymyositispolymyositis..
Note extensive Note extensive calcinosiscalcinosis
involving both involving both
subcutaneous tissues and subcutaneous tissues and
tendon sheaths.tendon sheaths.
From: Koopman: Arthritis and Allied Conditions, 13th ed., 1997.
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PLAIN FILMSPLAIN FILMS
Juvenile Juvenile DermatomyositisDermatomyositis
55--year old female with year old female with
an 18 month history of an 18 month history of
difficulty walking.difficulty walking.
Note extensive soft Note extensive soft
tissue calcifications: tissue calcifications:
Not in a Vascular Not in a Vascular
PatternPattern
Not attached to Not attached to
bonebone
From: www.hawaii.edu/medicine/pediatrics/pemxray/v6c11.html7
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Juvenile Juvenile DermatomyositisDermatomyositis
18 year old boy with 18 year old boy with
DermatomyositisDermatomyositis: :
extensive deep extensive deep
calcified deposits are calcified deposits are
seen about each hip seen about each hip
and extending into and extending into
the thighs.the thighs.
Eddy MC, Leelawattana R, McAlister WH, Whyte MP. Calcinosis universalis complicating
juvenile dermatomyositis: resolution during probenecid therapy. J Clin Endocrinol Metab.
1997 Nov;82(11):3536-42.
Superior ramus of pubis
Inferior ramus of pubis
Ischial tuberosity
Lesser trochanter
Greater trochanter
Obturator foramen
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Juvenile Juvenile DermatomyositisDermatomyositis
Same 18 year old Same 18 year old
boy with boy with
DermatomyositisDermatomyositis: :
extensive deep extensive deep
calcified deposits calcified deposits
are seen about the are seen about the
shoulder extending shoulder extending
into the arm. into the arm.
The clustering The clustering
around joints around joints
mimics mimics tumoraltumoral
calcinosiscalcinosis..
Eddy et al., 1997.
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Idiopathic Idiopathic TumoralTumoral CalcinosisCalcinosis
Note extensive soft Note extensive soft
tissue calcifications.tissue calcifications.
Multiple Multiple lobulatedlobulated, ,
dense calcifications are dense calcifications are
noted around joints in noted around joints in
the hands and wrist and the hands and wrist and
in other soft tissues. in other soft tissues.
From: http://www.rad.washington.edu/maintf/cases/unk39/answers.html
Linear collection of Linear collection of
calcification tracking calcification tracking
along a tendon sheath.along a tendon sheath.
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Idiopathic Idiopathic TumoralTumoral CalcinosisCalcinosis
The soft tissue calcifications may be single or multiple, The soft tissue calcifications may be single or multiple,
lobulatedlobulated, cystic or solid. , cystic or solid.
Often occurs at hips, elbows and shoulders. Often occurs at hips, elbows and shoulders.
The masses may range in size from 1 to 20 cm in diameter. The masses may range in size from 1 to 20 cm in diameter.
Family history in 30 Family history in 30 -- 40 % of cases. 40 % of cases.
Onset during childhood or adolescence. Onset during childhood or adolescence.
May be increased prevalence in patients of African May be increased prevalence in patients of African
descent. descent.
http://www.rad.washington.edu/maintf/cases/unk39/answers.html
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RADIONUCLIDE BONE SCANRADIONUCLIDE BONE SCAN
Idiopathic Idiopathic TumoralTumoral CalcinosisCalcinosis
Bone scan Bone scan
demonstrates demonstrates
prominent uptake in prominent uptake in
soft tissues, soft tissues,
especially around especially around
the pelvis and the pelvis and
shouldersshoulders
From: http://www.rad.washington.edu/maintf/cases/unk39/answers.html
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Idiopathic Idiopathic TumoralTumoral CalcinosisCalcinosis
What type of study What type of study
is this?is this?
MRIMRI……
Why?…Why?…
That’s right! That’s right!
Because the bone Because the bone
cortex is black… cortex is black…
And the fat is And the fat is
white!white!
From: http://www.rad.washington.edu/maintf/cases/unk39/answers.html
MRI (T2) of the hips reveals large cystic areas of low signal
intensity (arrows). This patient had Idiopathic Tumoral
Calcinosis.
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Computed TomographyComputed Tomography
CT of the pelvis in CT of the pelvis in
a patient with SLE.a patient with SLE.
Note extensive Note extensive
calcifications calcifications
(contrast the high (contrast the high
attenuation with attenuation with
that of MRI) in the that of MRI) in the
subcutaneous subcutaneous
tissue.tissue.
From: www.hawaii.edu/medicine/pediatrics/pemxray/v6c11.html7
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Unknown…Unknown…
The patient is a 900 The patient is a 900
year old man from year old man from
the the DegobahDegobah
System…System…
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Master YodaMaster Yoda
Courtesy of LucasFilm ®
? Scleroderma
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Treatment of Treatment of CalcinosisCalcinosis CutisCutis
Medical treatments include:Medical treatments include:
IntralesionalIntralesional corticosteroid injectioncorticosteroid injection
EtidronateEtidronate disodium (a disodium (a bisphosphonatebisphosphonate))
Aluminum hydroxide (phosphateAluminum hydroxide (phosphate--binder)binder)
However, these are not very effective.However, these are not very effective.
Surgical excision has been shown to be of Surgical excision has been shown to be of
benefit, as it can provide symptomatic benefit, as it can provide symptomatic
relief.relief.
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SummarySummary
Soft tissue calcifications are relatively common Soft tissue calcifications are relatively common
radiographic findings.radiographic findings.
Dystrophic calcifications predominate.Dystrophic calcifications predominate.
Often incidental finding, but can present with skin lesions Often incidental finding, but can present with skin lesions
or pain.or pain.
Good history is key. Good history is key.
Plain film to start; other imaging can be helpful if plain Plain film to start; other imaging can be helpful if plain
film is normal.film is normal.
Medical treatment has variable success; surgical removal Medical treatment has variable success; surgical removal
of calcification is indicated if symptomatic.of calcification is indicated if symptomatic.
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BibliographyBibliography
KoopmanKoopman: Arthritis and Allied Conditions, 13: Arthritis and Allied Conditions, 13thth ed., 1997.ed., 1997.
Richardson, M.L. Approaches to differential diagnosis in musculoRichardson, M.L. Approaches to differential diagnosis in musculoskeletal skeletal
imaging: soft tissue calcifications. imaging: soft tissue calcifications.
http://http://www.rad.washington.edu/mskbook/softtissueca.htmlwww.rad.washington.edu/mskbook/softtissueca.html., 1994.., 1994.
Stewart, V.L. Stewart, V.L. HerlingHerling, P., , P., DalinkaDalinka, M.K. Calcification in soft tissues. JAMA , M.K. Calcification in soft tissues. JAMA
1983; 250 (1): 781983; 250 (1): 78--81.81.
http://www.hawaii.edu/medicine/pediatrics/pemxray/v6c11.htmhttp://www.hawaii.edu/medicine/pediatrics/pemxray/v6c11.htm
http://www.rad.washington.edu/maintf/cases/unk39/answers.htmlhttp://www.rad.washington.edu/maintf/cases/unk39/answers.html
Cousins, M.A. Surgical management of Cousins, M.A. Surgical management of calcinosiscalcinosis cutis cutis universalisuniversalis in systemic in systemic
lupus. Arthritis Rheum 1997 Mar; 40(3): 570lupus. Arthritis Rheum 1997 Mar; 40(3): 570--2.2...
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AcknowledgmentsAcknowledgments
Beverlee Turner
Larry Barbaras
Special thanks to Dr. Hochman for the chest X-Ray
The end.
Calcinosis Cutis: Emerging Trends in Dermatoradiology
“Routine CXR”…
What is Calcinosis?
Classical Dermatology
“Dermatoradiology”
Classic Dermatology
Dermatoradiology
Calcinosis: The Breakdown
Descriptors
Pathophysiology
Differential Diagnosis (Ddx)
Ddx Dystrophic Calcification
Differential Diagnosis: A Radiologist’s Perspective
Menu of radiologic tests for imaging ectopic calcifications
PLAIN FILMS�Polymyositis
PLAIN FILMS�Juvenile Dermatomyositis
Juvenile Dermatomyositis
Juvenile Dermatomyositis
Idiopathic Tumoral Calcinosis
Idiopathic Tumoral Calcinosis
�RADIONUCLIDE BONE SCAN�Idiopathic Tumoral Calcinosis
Idiopathic Tumoral Calcinosis
Computed Tomography
Unknown…
Master Yoda
Treatment of Calcinosis Cutis
Summary
Bibliography
Acknowledgments