Scleroderma secondary to silica exposure dr n.s.ramburn
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 ofconnective 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 pulmonarysilicosis, 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 2weeks.- 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, withPigmentation .
Physical examination-Whole body skin toughness-Swelling of both hands with stiff movement of theFingers.-Raynaud’s sign positive (+)
-Skin appeared shiny and thickened around theforearm and hands.-Flexion deformity of fingers with some ulcerationon the plantar side.
Pigmentation of skin:-back & lower limbs-Local Temperature of thedistal 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 toesdecreased.
2 weeks before admission:-difficulty in breathing-pain and swelling of fingers-difficulty swallowing (solid food)-dry oral mucosaNo muscle pain, or ulceration in mouth cavity.No history of weight loss or appetite.No past medical and family illness.Work HistoryWorking in glass factory since 10 years, withconstant 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, Heartcavity, and scrotum.CT SCAN Chest: early stage of lung fibrosis, interstitial pneumonia,↑Supraclavicular and axillary Lymph Nodes, ↑fluidlevel 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-Duringcontraction: 22mm, during dilatation 15mm. RIGHTventricle-during contraction: 4mm, during dilation0mm.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 whencontraction: 6mm, when dilatation: 4mm.-Biopsy report 15684 - Suggestive of scleroderma.
Increased and thickened dermal collagen that involves dermis; Loss ofcutaneous appendages.
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 elaboratefactors that cause chronic inflammation andfibroblastic proliferation resulting in progressivefibrosis.- Silica known to be a highly immunogenicsubstance and can induce an autoimmune responseleading to the development of connective tissuediseases.
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, thepresence of silicosis, and PSS was conductedin white South African gold miners by means ofa case-control study.79 cases of PSS were matched by year of birthwith an equal number of control minersselected randomly but bearing in mind theadministrative channel through which the casehad come to be identified.Analysis showed no association betweensilicosis and PSS but did show that thecumulative life time silica exposure wassignificantly higher in the cases compared withcontrols.
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 thosepredisposed to PSS by a mechanism other than is thecase in the pathogenesis of silicosis.This is despite the fact that some of the autoimmunephenomena detected in the laboratory are similar in bothPSS and silicosis.The fact that intensity of exposure is importantsuggests that doses of silica high enough tooverwhelm the lung associated lymphoid system allowappreciable amounts of silica access to other tissues,especially immunologically active tissues.People in contact with silica should be monitored every1-2 years with routine tests and CT Scan, to check the level and amount of Silica, thus avoiding complications.