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Scleromyxedema

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					Scleromyxedema
 A Presentation of this rare case
   and the problem we meet

                                     2003/1/30
                            By R Liu Chih-Min
                   Introduction
   EPIDEMIOLOGY
        Scleromyxedema is an uncommon disease;
         approximately 114 cases have been reported in the
         Englishlanguage literature
        It typically affects middle-aged adults without sex
         predilection
        Idiopathic cutaneous mucinosis
        generalized VS localized lichen myxedematosus
                         Introduction
   PATHOGENESIS
       Paraprotein role
              Paraprotein levels correlate with neither extent nor progression
               of the disease. However, whereas scleromyxedema serum
               enhances fibroblast proliferation, an immunoglobulin purified
               from the paraprotein-containing serum proved unable to
               stimulate fibroblasts to proliferate in vitro, which suggests a
               pathogenetic role of a circulating factor other than the
               paraprotein.
                      Introduction
   HISTOLOGICAL TYPES
         The histopathology is distinct for scleromyxedema with
          collections of mucin associated with a marked
          proliferation of fibroblasts in the upper and mid-dermis
                      Introduction
   Diagnosis of scleromyxedema
       (1) generalized papular and sclerodermoid eruption
       (2) mucin deposition, fibroblast proliferation, and fibrosis

       (3) monoclonal gammopathy

       (4) the absence of thyroid disease
                           Introduction
   DISEASE ASSOCIATIONS
         Slight to severe muscle weakness
               severe proximal muscle weakness is found in 27%
                Mucin deposition has been found in only 2 patients
         Paraproteinemia in scleromyxedema
               Associated with many systemic disorders and, almost constantly, with
                paraproteinemia (83.2%)
               Usually IgG with light chains
         Joints
               Reported in 10.5% of the patients
               Arthralgia, migratory arthritis, and seronegative polyarthritis with
                occasional mucin deposition
                     Introduction
   Lungs
         Dyspnea is found in 16.7% of patients
         Restrictive or obstructive lung involvement
         Rarely pulmonary hypertension developed, mucin deposition was
          found in the large pulmonary veins and artery
   Disturbances of the central nervous system
         15% of patients
   Esophagus
         Dysphagia and nasal regurgitation are reported by 31.6% of patients
   Kidney
   Heart
   Optic
   Larynx
                        Introduction
   PROGNOSIS AND TREATMENT
       Recurrence
              Spontaneous improvement and resolution, even after 15 years,
               have been described
       Treatment
              Corticosteroids; Retinoid
              Response to high-dose intravenous immunoglobulin (hdIVIg).
              Complete Remission of Scleromyxedema Following
               Autologous Stem Cell Transplantation
                         History
   <Basic data>
          Sex: female
          Age: 46 y/o


   <Chief complaint>
          Rapid growing hematoma noted on 2002/12/15
                              History
   <Present illness>
          2000/5: Progressive four limbs weakness
               Proximal weakness and myalgia
          2001/1: Admitted to CGMH
               NCV, EMG revealed myopathy but muscle biopsy: (-)
          2001/4: NTUH
               Bedridden, joint cintracture
               IgG elevation; Bences-Jones protine
               Skin biopsy: mucin deposition
          Scleromyxedema was diagnosted
               Multiple systemic manifastation: myopath, dysphagia, serum
                paraprotine elevation
                             History
   <Present illness>
          2002/12/15: Right thigh petechiae
               Her family
               Rapid extended to 5*5cm
          Hematoma ruptured with wound necrosis
               Wound debridement was arranged on 2002/1/8
          Left knee subluxation
                             History
   <Physical Examination>
          Extrimity:
               Right thigh 15*7cm hematoma with skin necrosis
               Multiple joints contractures with deformity
          Motor
               MP: distal
               DTR: (-)
          Sensation: fine
   <Impression>
          Scleromyxedema
          Right thigh hematoma with local cellulitis
About our patient in OR

             2003/1/8
   First operation for right thigh
      hematoma debridement
         Post-OP Complication
   Spontaneous scalp hematoma
   Massive hematoma evacuation. ( > 500ml)
   PRBC 2U: Hb 9.9
                         Course
   Wound:
       Oozing; PRBC 6U + Cryoprecipitate 10U
   Lab:
       Bleeding time: 10.5 sec to > 20 sec
       R/O platelet dysfunction
       R/O VWF disease
  2nd operation
    2003/1/17
Protection and avoid stressful
  sheering during operation
                       Course
   <OP Note>
       ETGA, prone
       Diffuse oozing
       Hard to close
       Cover open wound
   <Post-OP>
       Still oozing
       Hb: 12.2 to 5.4; PRBC 4U
Protection
        Hematoma protected
         and compression

        Portection of face
Protection

        Dislocation of hip joint



        Diffuse oozing
Protection
Protection