LIPOID GRANULOMATOSIS (XANTHOMATOSIS) WITH MARKED PULMONARY FIBROSIS AND COR PULMONALE AS OUTSTANDING MANIFESTATIONS

Abstract

Attention is called to the fact that the diagnosis of lipoid granulomatosis, in the absence of the Hand-Schuller-Christian triad (defects of membranous bones, exophthalmos and diabetes insipidus), has received little attention. A case is described in which a young man was discovered to have pulmonary fibrosis in 1938. At that time there were no symptoms. Detailed studies in 1940 revealed no etiological factor in this pulmonary fibrosis. In 1946, there was general deterioration of the subject''s health, cardiac enlargement, marked dyspnea on effort, and evidence of right heart failure; clinical observations led to the discovery by X-ray of osseous lesions in the skull, pelvis and several long bones. Biopsy of a skull lesion resulted in diagnosis of lipoid granuloma of the type seen in Schuller-Christian disease. The patient''s downhill course was characterized by increasing cardiac enlargement and progressive right heart failure, unresponsive to conventional therapy for heart failure. Deep X-ray treatment of the lungs was followed by slight diminution in the pulmonary infiltrations, but otherwise the patient''s course was not influenced favorably. It is suggested that in any instance of unexplained pulmonary fibrosis this entity be kept in mind. Early irradiation of pulmonary infiltrations of this disease may be of value.