|Year : 1997 | Volume
| Issue : 2 | Page : 111-113
Systemic sclerosis in a stone cutter
N Khanna, P D'Souza, A Sud, RK Pandhi
Source of Support: None, Conflict of Interest: None
Several occupational hazards especially exposure to silica have been implicated as eliciting factors for the development of scleroderma-like disorders. We here report a case of manual stone-cutter who developed progressive scleroderma, interstitial lung disease and decreased oesophageal motility after several years of exposure to silica dust.
Keywords: Systemic sclerosis, Silicosis, Interstitial lung disease
|How to cite this article:|
Khanna N, D'Souza P, Sud A, Pandhi R K. Systemic sclerosis in a stone cutter. Indian J Dermatol Venereol Leprol 1997;63:111-3
|How to cite this URL:|
Khanna N, D'Souza P, Sud A, Pandhi R K. Systemic sclerosis in a stone cutter. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2019 Sep 17];63:111-3. Available from: http://www.ijdvl.com/text.asp?1997/63/2/111/4533
Systemic sclerosis-like disorders have been reported to develop following exposure to silica, vinyl chloride, aliphatic and aromatic hydrocarbons, expoxy resins, bleomycin and toxic oil. Except for silica, however, all other substances induce changes that only mimic scleroderma, and are partly reversible after the exposure is discontinued. We here report a case of a stone-cutter who developed systemic sclerosis (SS).
| Case Report|| |
A 53-year-old stone-cutter from Badarpur, Delhi presented with a 4-year history of arthralgia, involving both the knees and elbow joints. At the same time he also noticed Raynaud's phenomenon involving the fingers and toes. One and half to 2 years later, the patient developed a gradual binding down of the skin over the dorsa of hands, forearms, face and legs with finger tip ulcerations and painful gangrenous lesions in several toes and fingers. He also developed progressive dyspnoea initially on exertion and later also at rest. Patient also developed dysphagia. There was no history of fever, cough, weight loss, photosensitivity, muscle weakness or epigastric pain. He had been a manual stone-cutter for the past 30 years. He could not recall any other person in his occupation developing similar complaints.
On general physical examination the patient had mild pallor and axillary lymphadenopathy. The lymph nodes were 3-5 in number on each side, 1.5x2cm in size firm to hard in consistency and arranged in a linear distribution with a firm, fibrotic cord like structure connecting the adjoining lymph nodes [Figure:l]. The overlying skin was adherent to the lymph nodes. Examination of the respiratory system revealed bilateral basal fine crepts.
Cutaneous examination [Figure - 2] showed extensive binding down of skin of the face, dorsa of hands and mid forearm, chest, abdomen and legs. Both hands showed evidence of sclerodactyly, the finger pulp showed pitted scars and there were dry gangrenous changes of distal phalanges of right ring finger and several of the toes. There were facial talengiectasias and limitation of oral opening. Muliple depigmented macular lesions were present over the scalp and lower legs. The finger nails showed ragged cuticles but there were no apparent nail fold talengiectasias.
Laboratory investigations revealed a normocytic hypochromic anemia (Hb-9 gm%) and a leucocyte count of 9, 600/mm. The ESR was raised (60mm/lst hour by Wintrobe). The liver function parameters were within normal limits. Though the blood urea and serum creatinine were within normal limits, the urine examination showed a mild proteinuria. Sputum was negative for acid fast bacilli. Blood for ANA and ANCA was negative. Skin biopsy from the drosum of hand was compatible with systemic sclerosis while lymph node biopsy showed histiocytes containing carbon particles along with marked fibrotic reaction. The X-ray chest revealed interstitial pattern with bilateral pleural effusion and bilateral midzone consolidation. There were multiple scattered calcific densities in the midzone. X-ray of hands and feet showed acro-osteolysis. Barium swallow examination showed decreased peristalsis with hold up of the contrast in the lower thoracic oesophagus with mild dilatation. The pulmonary function tests revealed a restrictive pattern with reduction in diffusion capacity. For his SS he was started on dexamethasone pulse therapy in the dose of 100 mg on 3 consecutive days every month along with pentoxyphylline 600 mg daily. He has been asked to come for regular follow up.
| Discussion|| |
systemic sclerosis is a multisystem disorder characterised by widespread fibrotic and degenerative changes in the skin, vasculature, and internal organs. The aetiology of the disease is unknown. A number of occupational and other exposures have been implicated as potential causes of SS including silica dust, epoxy resins, bleomycin and benzene.
Since the original description by Bramwell, silica has been the substance most related to occupational induction of SS. In 1966, Rodnan et al in study of 60 men with SS found that 43% of them had worked as coal miners or in other occupations marked by prolonged and heavy exposures to silica dust. In a more recent study, Haustein et almention that on basis of epidemiological data, they found that the likelihood for development of scleroderma is approximately 50 times higher in persons exposed to silica dust than in the nonexposed male population. In several countries, SS is recognised as an occupational disease without it being necessary for the patients to have associated pulmonary silicosis.
It is also established now that patients with silica-associated systemic sclerosis (SA-SS) are clinically, serologically and immunologically indistinguishable from idiopathic SS although as a group the SA-SS patients have a higher prevalence of pulmonary involvement (bibasilar fibrosis) and the anti scl-70 antibody. In our patient also the clinical and investigational parameters of SS were indistinguishable from those of' idiopathic SS.
Crystalline silica (quartz) particles less than 1/μm are the most pathogenic in silicosis. These particles are phagocytosed by macrophages and are transported to the regional lymph nodes. Though silica is chemically inert, it is toxic to the macrophages. When the macrophages die, the silica is released and is phagocytosed again. This constitutes a permanent stimulus for the fibroblasts, which increase their production of collagen leading to cutaneous sclerosis, vascular occlusion and pulmonary fibrosis.
| References|| |
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[Figure - 1], [Figure - 2]