scleromyxedema a presentation of this rare case and the problem we meet 2003/1/30 by r liu chih-min
TRANSCRIPT
ScleromyxedemaScleromyxedema
A Presentation of this rare case A Presentation of this rare case and the problem we meetand the problem we meet
2003/1/302003/1/30By R Liu Chih-MinBy R Liu Chih-Min
IntroductionIntroduction
EPIDEMIOLOGYEPIDEMIOLOGY ScleromyxedemaScleromyxedema is an uncommon disease; approxim is an uncommon disease; approxim
ately 114 cases have been reported in the Englishlangately 114 cases have been reported in the Englishlanguage literature uage literature
It typically affects middle-aged adults without sex preIt typically affects middle-aged adults without sex predilection dilection
Idiopathic cutaneous mucinosis Idiopathic cutaneous mucinosis generalized VS localized lichen myxedematosus generalized VS localized lichen myxedematosus
IntroductionIntroduction
PATHOGENESISPATHOGENESIS Paraprotein roleParaprotein role
Paraprotein levels correlate with neither extent nor progression Paraprotein levels correlate with neither extent nor progression of the diseaseof the disease. However, whereas scleromyxedema serum enha. However, whereas scleromyxedema serum enhances fibroblast proliferation, an immunoglobulin purified from nces fibroblast proliferation, an immunoglobulin purified from the paraprotein-containing serum proved unable to stimulate fithe paraprotein-containing serum proved unable to stimulate fibroblasts to proliferate in vitro, which broblasts to proliferate in vitro, which suggests a pathogenetic suggests a pathogenetic role of a circulating factor other than the paraproteinrole of a circulating factor other than the paraprotein. .
IntroductionIntroduction
HISTOLOGICAL TYPESHISTOLOGICAL TYPES The histopathology is distinct for scleromyxedema with The histopathology is distinct for scleromyxedema with
collections of collections of mucinmucin associated with a marked proliferat associated with a marked proliferation of fibroblasts in the ion of fibroblasts in the upper and mid-dermisupper and mid-dermis
IntroductionIntroduction
Diagnosis ofDiagnosis of scleromyxedemascleromyxedema (1)(1)generalized papular and sclerodermoid eruptiongeneralized papular and sclerodermoid eruption
(2)(2)mucin deposition, fibroblast proliferation, and fibrosismucin deposition, fibroblast proliferation, and fibrosis
(3)(3)monoclonal gammopathymonoclonal gammopathy
(4)(4) the absence of thyroid disease the absence of thyroid disease
IntroductionIntroduction
DISEASE ASSOCIATIONSDISEASE ASSOCIATIONS Slight to severe muscle weaknessSlight to severe muscle weakness
severe proximal muscle weakness is found in 27%severe proximal muscle weakness is found in 27% Mucin deposition has been found in only 2 patients Mucin deposition has been found in only 2 patients
Paraproteinemia in scleromyxedemaParaproteinemia in scleromyxedema Associated with many systemic disorders and, almost constantly, with Associated with many systemic disorders and, almost constantly, with
paraproteinemia (83.2%)paraproteinemia (83.2%) Usually IgG with light chains Usually IgG with light chains
JointsJoints Reported in 10.5% of the patients Reported in 10.5% of the patients Arthralgia, migratory arthritis, and seronegative polyarthritis with occArthralgia, migratory arthritis, and seronegative polyarthritis with occ
asional mucin depositionasional mucin deposition
IntroductionIntroduction LungsLungs
Dyspnea is found in 16.7% of patientsDyspnea is found in 16.7% of patients Restrictive or obstructive lung involvement Restrictive or obstructive lung involvement Rarely pulmonary hypertension developed, mucin deposition was fouRarely pulmonary hypertension developed, mucin deposition was fou
nd in the large pulmonary veins and arterynd in the large pulmonary veins and artery Disturbances of the central nervous systemDisturbances of the central nervous system
15% of patients 15% of patients EsophagusEsophagus
Dysphagia and nasal regurgitation are reported by 31.6% of patients Dysphagia and nasal regurgitation are reported by 31.6% of patients KidneyKidney HeartHeart OpticOptic LarynxLarynx
IntroductionIntroduction
PROGNOSIS AND TREATMENTPROGNOSIS AND TREATMENT RecurrenceRecurrence
Spontaneous improvement and resolution, even after 15 years, Spontaneous improvement and resolution, even after 15 years, have been described have been described
TreatmentTreatment Corticosteroids; Retinoid Corticosteroids; Retinoid Response to high-dose intravenous immunoglobulin (hdIVIg). Response to high-dose intravenous immunoglobulin (hdIVIg). Complete Remission of Scleromyxedema Following AutoloComplete Remission of Scleromyxedema Following Autolo
gous Stem Cell Transplantationgous Stem Cell Transplantation
HistoryHistory
<Basic data><Basic data> Sex: femaleSex: female Age: 46 y/oAge: 46 y/o
<Chief complaint> <Chief complaint> Rapid growing hematoma noted on 2002/12/15Rapid growing hematoma noted on 2002/12/15
HistoryHistory
<Present illness><Present illness> 2000/5: Progressive four limbs weakness2000/5: Progressive four limbs weakness
Proximal weakness and myalgiaProximal weakness and myalgia 2001/1: Admitted to CGMH2001/1: Admitted to CGMH
NCV, EMG revealed myopathy but muscle biopsy: (-)NCV, EMG revealed myopathy but muscle biopsy: (-) 2001/4: NTUH2001/4: NTUH
Bedridden, joint cintractureBedridden, joint cintracture IgG elevation; Bences-Jones protineIgG elevation; Bences-Jones protine Skin biopsy: mucin depositionSkin biopsy: mucin deposition
Scleromyxedema was diagnostedScleromyxedema was diagnosted Multiple systemic manifastation: myopath, dysphagia, serum pMultiple systemic manifastation: myopath, dysphagia, serum p
araprotine elevationaraprotine elevation
HistoryHistory
<Present illness><Present illness> 2002/12/15: Right thigh petechiae2002/12/15: Right thigh petechiae
Her familyHer family Rapid extended to 5*5cmRapid extended to 5*5cm
Hematoma ruptured with wound necrosisHematoma ruptured with wound necrosis Wound debridement was arranged on 2002/1/8Wound debridement was arranged on 2002/1/8
Left knee subluxationLeft knee subluxation
HistoryHistory
<Physical Examination><Physical Examination> Extrimity: Extrimity:
Right thigh 15*7cm hematoma with skin necrosisRight thigh 15*7cm hematoma with skin necrosis Multiple joints contractures with deformityMultiple joints contractures with deformity
MotorMotor MP: distalMP: distal DTR: (-)DTR: (-)
Sensation: fineSensation: fine <Impression><Impression>
ScleromyxedemaScleromyxedema Right thigh hematoma with local cellulitisRight thigh hematoma with local cellulitis
About our patient in ORAbout our patient in OR
2003/1/82003/1/8
First operation for right thigh hematoFirst operation for right thigh hematoma debridementma debridement
Post-OP ComplicationPost-OP Complication
Spontaneous scalp hematomaSpontaneous scalp hematoma Massive hematoma evacuation. ( > 500ml)Massive hematoma evacuation. ( > 500ml) PRBC 2U: Hb 9.9PRBC 2U: Hb 9.9
CourseCourse
Wound: Wound: Oozing; PRBC 6U + Cryoprecipitate 10U Oozing; PRBC 6U + Cryoprecipitate 10U
Lab: Lab: Bleeding time: 10.5 sec to > 20 secBleeding time: 10.5 sec to > 20 sec R/O platelet dysfunctionR/O platelet dysfunction R/O VWF diseaseR/O VWF disease
2nd operation 2nd operation 2003/1/172003/1/17
Protection and avoid stressful sheerinProtection and avoid stressful sheering during operationg during operation
CourseCourse
<OP Note><OP Note> ETGA, prone ETGA, prone Diffuse oozingDiffuse oozing Hard to closeHard to close Cover open wound Cover open wound
<Post-OP><Post-OP> Still oozingStill oozing Hb: 12.2 to 5.4; Hb: 12.2 to 5.4; PRBC 4UPRBC 4U
ProtectionProtection
Hematoma protected anHematoma protected and compressiond compression
Portection of facePortection of face
ProtectionProtection
Dislocation of hip jointDislocation of hip joint
Diffuse oozingDiffuse oozing
ProtectionProtection
ProtectionProtection