Scleromyxedema A Presentation of this rare case and the problem we meet 2003/1/30 By R Liu Chih-Min.

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