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SCLERODERMA SECONDARY TO SILICA
          EXPOSURE


            CASE REPORT.

           Dr. Nitee Sagar Ramburn
                 MAURITIUS

      Department of Dermatology & STD.
        1st Affiliated Hospital to D.M.U.
In recent years, there has been several cases of
connective tissue diseases, especially scleroderma,
following exposure to silica and silicone.



Below here is described a 50 year old Chinese man,
with a history of exposure to silica for several years,
developing scleroderma-like disease and pulmonary
silicosis, in a very progressive manner past 1 year.
Case report

 Patient Name: Wan De Xian
 Age: 50
 Gender: Male


  Chief Complaints :
- Whole body tightness past 1 year.
- Dyspnoea for approximately 2
weeks.
- Mild difficulty in swallowing solids.

   From History :
-All started 1 year back.
-Hands, feet, face were swollen and tight feeling.
-Developed on his extremities, trunks, neck, with
Pigmentation .
Physical examination
-Whole body skin toughness
-Swelling of both hands with stiff movement of the
Fingers.
-Raynaud’s sign positive (+)
-Skin appeared shiny and thickened around the
forearm and hands.
-Flexion deformity of fingers with some ulceration
on the plantar side.
-Ulcerations over the right sole, posteriorly.
Pigmentation of skin:
-back & lower limbs-Local Temperature of the
distal end of toes 。
-Having difficulty talking.
-Sensation of stiffness while swallowing.
-Could not close his eyes completely.
Pigmentation of skin:
-body-chest wall & abdomen
-neck & trunks
-back & lower limbs-Local Temperature of the distal end of toes
decreased.
  2 weeks before admission:
-difficulty in breathing
-pain and swelling of fingers
-difficulty swallowing (solid food)
-dry oral mucosa




No muscle pain, or ulceration in mouth cavity.
No history of weight loss or appetite.
No past medical and family illness.

Work History
Working in glass factory since 10 years, with
constant contact to silica.
  Investigation
-Blood test (17/11/2010):
ESR 28↑, WBC 4,9×10⁹/L, NEU 80,3%↑,
LYMPH 11,8%. IgG 1750 mg/dl↑,
IgA 1050 mg/dl↑, CRP 11mg/L↑.
-ANA Titre 1:1000+
Type of Ana Scl-70 Antibody +ve.
 -S.Protein
 Eletrophoresis 47,3% low
 ALB                18g/dL
 α-1: 3.4, α-2: 8.0,β: 10.7, ɣ: 30.6 High.


-Blood Glucose 7,45 mmol/L
-Urine test: Protein +1.
-ECG: T-wave changes. Premature ventricular beats.

CT SCAN Abdomen: fluid in Thoracic cavity, Heart
cavity, and scrotum.

CT SCAN Chest: early stage of lung fibrosis, interstitial
   pneumonia,
↑Supraclavicular and axillary Lymph Nodes, ↑fluid
level in heart cavity.
 Conclusion: findings caused by scleroderma.
-Barium Meal: Esophageal activity slow.
-Ultrasound: Fluid in thoracic cavity
   Right side 33mm, Left side 28mm.
 22/11//2010:
Heart cavity fluid: LEFT ventricle-During
contraction: 22mm, during dilatation 15mm. RIGHT
ventricle-during contraction: 4mm, during dilation
0mm.
Right Atrium: 35×8mm.

-Cardiac Ultrasound (18/11/2010)
Range of fluid on LEFT ventricle when contraction:
19mm, when dilation: 9mm.
Range of fluid in RIGHT ventricle when
contraction: 6mm, when dilatation: 4mm.

-Biopsy report 15684 - Suggestive of scleroderma.
Increased and thickened dermal collagen that involves dermis; Loss of
cutaneous appendages.
   Treatment

   Prednisone 30mg daily
   Chinese herb(effects to activate blood
    circulation)
   Furosemide 20 mg BD
   NSAIDS: Brufen 300 mg BD
   Beraprost(inhibits platelet aggregation) 40µg
    TID
   Colchicine 0.5 mg BD
   Spironolactone(k+ sparing diuretic) 20 mg TDS
Discussion :


   Progressive systemic sclerosis - multisystem
    disease.

   Characteristics:
    Degeneration of the skin, heart, lungs, kidneys,
    gastrointestinal tract and synovia.

 Etiology :
 Not been clearly elucidated;
 Overproduction of qualitatively normal collagen
  in the affected organs.
 Exposures to silica dust, et al


   Described often as occupational disease.
- Silicosis is the fibrotic disease in the lung due inhalation
    of silica dust (silicone dioxide)


- Recognized as an occupational hazard


- Lung macropages exposed to silica elaborate
factors that cause chronic inflammation and
fibroblastic proliferation resulting in progressive
fibrosis.


- Silica known to be a highly immunogenic
substance and can induce an autoimmune response
leading to the development of connective tissue
diseases.
   PSS in most cases develops gradually after 14 or
    more years of exposure to Silica.

   Our patient has been in contact to Silica for
    about 10 years and PSS developed at an unusual
    rapidity of a one year period.
   Year 2000 – Research at Epidemiology Research
    Unit, Medical Bureau for Occupational Diseases,
    Johannesburg, South Africa.


Relation between exposure to silica dust, the
presence of silicosis, and PSS was conducted
in white South African gold miners by means of
a case-control study.


79 cases of PSS were matched by year of birth
with an equal number of control miners
selected randomly but bearing in mind the
administrative channel through which the case
had come to be identified.


Analysis showed no association between
silicosis and PSS but did show that the
cumulative life time silica exposure was
significantly higher in the cases compared with
controls.
 This difference was due to a difference
   in the intensity of exposure to silica
    during mining service rather than a
     difference in duration of service.
   Conclusions :


Silica therefore seems to exercise its action in those
predisposed to PSS by a mechanism other than is the
case in the pathogenesis of silicosis.



This is despite the fact that some of the autoimmune
phenomena detected in the laboratory are similar in both
PSS and silicosis.



The fact that intensity of exposure is important
suggests that doses of silica high enough to
overwhelm the lung associated lymphoid system allow
appreciable amounts of silica access to other tissues,
especially immunologically active tissues.



People in contact with silica should be monitored every
1-2 years with routine tests and CT Scan, to check the level
   and amount of Silica, thus avoiding complications.
Thank you.

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Scleroderma secondary to silica exposure dr n.s.ramburn

  • 1. SCLERODERMA SECONDARY TO SILICA EXPOSURE CASE REPORT. Dr. Nitee Sagar Ramburn MAURITIUS Department of Dermatology & STD. 1st Affiliated Hospital to D.M.U.
  • 2. In recent years, there has been several cases of connective tissue diseases, especially scleroderma, following exposure to silica and silicone. Below here is described a 50 year old Chinese man, with a history of exposure to silica for several years, developing scleroderma-like disease and pulmonary silicosis, in a very progressive manner past 1 year.
  • 3. Case report  Patient Name: Wan De Xian  Age: 50  Gender: Male  Chief Complaints : - Whole body tightness past 1 year. - Dyspnoea for approximately 2 weeks. - Mild difficulty in swallowing solids. From History : -All started 1 year back. -Hands, feet, face were swollen and tight feeling. -Developed on his extremities, trunks, neck, with Pigmentation .
  • 4. Physical examination -Whole body skin toughness -Swelling of both hands with stiff movement of the Fingers. -Raynaud’s sign positive (+)
  • 5. -Skin appeared shiny and thickened around the forearm and hands. -Flexion deformity of fingers with some ulceration on the plantar side.
  • 6. -Ulcerations over the right sole, posteriorly.
  • 7. Pigmentation of skin: -back & lower limbs-Local Temperature of the distal end of toes 。
  • 8. -Having difficulty talking. -Sensation of stiffness while swallowing. -Could not close his eyes completely.
  • 9. Pigmentation of skin: -body-chest wall & abdomen -neck & trunks -back & lower limbs-Local Temperature of the distal end of toes decreased.
  • 10.  2 weeks before admission: -difficulty in breathing -pain and swelling of fingers -difficulty swallowing (solid food) -dry oral mucosa No muscle pain, or ulceration in mouth cavity. No history of weight loss or appetite. No past medical and family illness. Work History Working in glass factory since 10 years, with constant contact to silica.
  • 11.  Investigation -Blood test (17/11/2010): ESR 28↑, WBC 4,9×10⁹/L, NEU 80,3%↑, LYMPH 11,8%. IgG 1750 mg/dl↑, IgA 1050 mg/dl↑, CRP 11mg/L↑. -ANA Titre 1:1000+ Type of Ana Scl-70 Antibody +ve.  -S.Protein  Eletrophoresis 47,3% low  ALB 18g/dL  α-1: 3.4, α-2: 8.0,β: 10.7, ɣ: 30.6 High. -Blood Glucose 7,45 mmol/L -Urine test: Protein +1. -ECG: T-wave changes. Premature ventricular beats. CT SCAN Abdomen: fluid in Thoracic cavity, Heart cavity, and scrotum. CT SCAN Chest: early stage of lung fibrosis, interstitial pneumonia, ↑Supraclavicular and axillary Lymph Nodes, ↑fluid level in heart cavity.  Conclusion: findings caused by scleroderma.
  • 12. -Barium Meal: Esophageal activity slow. -Ultrasound: Fluid in thoracic cavity Right side 33mm, Left side 28mm.  22/11//2010: Heart cavity fluid: LEFT ventricle-During contraction: 22mm, during dilatation 15mm. RIGHT ventricle-during contraction: 4mm, during dilation 0mm. Right Atrium: 35×8mm. -Cardiac Ultrasound (18/11/2010) Range of fluid on LEFT ventricle when contraction: 19mm, when dilation: 9mm. Range of fluid in RIGHT ventricle when contraction: 6mm, when dilatation: 4mm. -Biopsy report 15684 - Suggestive of scleroderma.
  • 13. Increased and thickened dermal collagen that involves dermis; Loss of cutaneous appendages.
  • 14. Treatment  Prednisone 30mg daily  Chinese herb(effects to activate blood circulation)  Furosemide 20 mg BD  NSAIDS: Brufen 300 mg BD  Beraprost(inhibits platelet aggregation) 40µg TID  Colchicine 0.5 mg BD  Spironolactone(k+ sparing diuretic) 20 mg TDS
  • 15. Discussion :  Progressive systemic sclerosis - multisystem disease.  Characteristics: Degeneration of the skin, heart, lungs, kidneys, gastrointestinal tract and synovia.  Etiology :  Not been clearly elucidated;  Overproduction of qualitatively normal collagen in the affected organs.  Exposures to silica dust, et al  Described often as occupational disease.
  • 16. - Silicosis is the fibrotic disease in the lung due inhalation of silica dust (silicone dioxide) - Recognized as an occupational hazard - Lung macropages exposed to silica elaborate factors that cause chronic inflammation and fibroblastic proliferation resulting in progressive fibrosis. - Silica known to be a highly immunogenic substance and can induce an autoimmune response leading to the development of connective tissue diseases.
  • 17. PSS in most cases develops gradually after 14 or more years of exposure to Silica.  Our patient has been in contact to Silica for about 10 years and PSS developed at an unusual rapidity of a one year period.
  • 18. Year 2000 – Research at Epidemiology Research Unit, Medical Bureau for Occupational Diseases, Johannesburg, South Africa. Relation between exposure to silica dust, the presence of silicosis, and PSS was conducted in white South African gold miners by means of a case-control study. 79 cases of PSS were matched by year of birth with an equal number of control miners selected randomly but bearing in mind the administrative channel through which the case had come to be identified. Analysis showed no association between silicosis and PSS but did show that the cumulative life time silica exposure was significantly higher in the cases compared with controls.
  • 19.  This difference was due to a difference in the intensity of exposure to silica during mining service rather than a difference in duration of service.
  • 20. Conclusions : Silica therefore seems to exercise its action in those predisposed to PSS by a mechanism other than is the case in the pathogenesis of silicosis. This is despite the fact that some of the autoimmune phenomena detected in the laboratory are similar in both PSS and silicosis. The fact that intensity of exposure is important suggests that doses of silica high enough to overwhelm the lung associated lymphoid system allow appreciable amounts of silica access to other tissues, especially immunologically active tissues. People in contact with silica should be monitored every 1-2 years with routine tests and CT Scan, to check the level and amount of Silica, thus avoiding complications.