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Scleromyxedema

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

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Scleromyxedema

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

  2. 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

  3. 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.

  4. 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

  5. Introduction • Diagnosis ofscleromyxedema • generalized papular and sclerodermoid eruption • mucin deposition, fibroblast proliferation, and fibrosis • monoclonal gammopathy • the absence of thyroid disease

  6. 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

  7. 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

  8. 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

  9. History • <Basic data> • Sex: female • Age: 46 y/o • <Chief complaint> • Rapid growing hematoma noted on 2002/12/15

  10. 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

  11. 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

  12. 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

  13. About our patient in OR 2003/1/8 First operation for right thigh hematoma debridement

  14. Post-OP Complication • Spontaneous scalp hematoma • Massive hematoma evacuation. ( > 500ml) • PRBC 2U: Hb 9.9

  15. Course • Wound: • Oozing; PRBC 6U + Cryoprecipitate 10U • Lab: • Bleeding time: 10.5 sec to > 20 sec • R/O platelet dysfunction • R/O VWF disease

  16. 2nd operation 2003/1/17 Protection and avoid stressful sheering during operation

  17. 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

  18. Protection • Hematoma protected and compression • Portection of face

  19. Dislocation of hip joint Diffuse oozing Protection

  20. Protection

  21. Protection

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