Systemic sclerosis in a patient with maxillary antrum carcinoma following external beam radio therapy
2 Dept. of Radiotherapy, CMC and Hospital, Ludhiana - 141 008, Punjab, India
Dept. of Radiology, CMC and Hospital, Ludhiana - 141 008, Punjab
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Chakravarthi R, Singh V. Systemic sclerosis in a patient with maxillary antrum carcinoma following external beam radio therapy. Indian J Dermatol Venereol Leprol 2003;69:195
To the editor
Few cases of scleroderma have been reporte in patients after large field external beam irradiation c breast and chest wall. We present details of a patient who developed systemic sclerosis after small field irradiation c maxillary antrum. Changes began in fingers of right hand and spread beyond it. We consider that irradiation i associated with triggering of the progressive systemi sclerosis.
A 50-year-old female presented with epistaxi for lost 6 months. A nasoontral mass was detected an excised through Caldwell Luc′s approach. The patient wa referred for post operative radiation therapy for the residuc disease in right maxillary sinus which was also extending t ethmoid and sphenoid sinus. A dose of 60 Gy in 30 fraction over 6 weeks was delivered through enterior and laterc facial fields. The tumor regressed completely. No associate medication was prescribed during radiotherapy.
During the lost week of radiotherapy patier developed pain in both wrist, fingers and knee joint wit progressive inability to flex fingers and wrist joint. Durin next 3 months she also had symptoms of Raynaud′s phenomenon and her skin gradually became firm, thickene and eventually tightly bound to underlying subcutaneou tissue, involving initially upper extremity, followed by th face and trunk. There was no evidence of any gastric, renal or haematological involvement. The investigations reveale an ASO titre of 400 lU/dl and Anti-d-DNA value of 12.2IU ml. Autoimmune panel also included ANA-1.4units, C3 178.2 mg/dl and C4-24.4mg/dl. A provisional diagnosis c progressive systemic sclerosis was considered and patier was treated with tab. penicillamine 250mg OD and partiall responded to treatment. 14 months later she developed local recurrence involving right maxillary and ethmoid sinuses and was considered a poor risk operative case in view of progressive systemic sclerosis and advised symptomatic treatment.
Progressive systemic sclerosis is a multisystem disorder of unknown cause characterized by fibrosis of the skin, blood vessels and visceral organs. Pathogenesis involves immunologic mechanisms, vascular damage and activation of fibroblasts. Tumor necrosis factor can induce endothelial injury as well as stimulate fibrosis.
The mechanism of the association remains uncertain. The condition could be due to fibroblastic response to irradiation. Radiation pneumonitis, for example, may progress to fibroblast proliferation in the lung. Radiation nephritis may lead to a progressive fibrosis ending in renal failure. Similar changes in the dermis may act as a stimulus to morphoea formation.
Colver et al described nine female patients, seven with carcinoma of the breast, who developed morphoea after radiotherapy. Verbow, described four patients in whom scieroderma (systemic sclerosis in three and morphoea in one), developed within 18 months of detection of breastcarcinoma. Two patients, one with systemic sclerosis and one with morphoea, had received radiotherapy.
An earlier report described systemic sclerosis appearing within 6 months of radiotherapy treated carcinoma in two patients and two patients in whom systemic sclerosis preceded the breast carcinoma by less then two years. Thus, although an association between development of morphoea and prior radiotherapy appears real there is also a wider relationship between carcinoma and scleroderma.
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