Excessive fibrosis of supraclavicular lymph-node granulomas in a patient with progressive systemic sclerosis

Summary

The case of a 29-year-old white female with a 7-year history of typical scleroderma is presented who developed excessive fibrosis of the supraclavicular lymph nodes. After 3 years of disease, firm right supraclavicular lymphadenopathy appeared, accompanied by a high fever. Biopsy revealed non-caseating granulomas and short-term antituberculous therapy was ineffective. The symptoms finally responded to steroids, but adenopathy persisted. A second biopsy, 40 days after the first, disclosed a similar picture with some degree of fibrosis of the granulomas. Four years later, with stony hard right supraclavicular adenopathy persisting, a third biopsy showed excessive fibrosis of the granulomas within the node and destruction of its architecture. It is postulated that the primary disease of this patient might be responsible for this clinical picture. The present seems to be the first report of such a case in the literature.