皮肤钙质沉着症的风湿放射学新进展

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 2 ““Routine CXR”…Routine CXR”… Special Thanks to Dr. Hochman for this image. 3 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. 4 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 5 ““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 6 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 7 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 8 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: 9 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 10 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. 11 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%) 12 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 13 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 14 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 15 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. 16 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 17 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 18 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. 19 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. 20 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 21 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 22 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. 23 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 24 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… 25 Master YodaMaster Yoda Courtesy of LucasFilm ® ? Scleroderma 26 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. 27 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. 28 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... 29 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