THE concept of xanthomatosis includes a series of pathological conditions, in which lipoid substances are deposited in certain tissues of the body as a result of a disturbance of fat metabolism, which may be an essential primary process or one secondary to other constitutional disease, such as diabetes mellitus. These accumulations are found in various tissues, the composite picture differing greatly over a rather wide range of clinical cases, but in general considered by present-day investigators to represent a fundamentally similar pathologic process in all. It has also been variously termed “lipoid granulomatosis,” “lipoid reticulo-endotheliosis,” “lipoid gout,” “lipoid histiocytosis,” and “generalized xanthomatosis.” Case Reports Case 1. F. B., R-3680, a white boy aged three and one-half years, was admitted to the hospital July 29, 1927, with the complaint of multiple abscesses of the skull, exophthalmos of the right eye, ankylosis of the left shoulder, and pain in the region of the right hip. With him came a history of his having injured the shoulder in a fall in March, 1926. An abscess of the left tonsil had been opened in October, 1926, and one in the right temporal region arising without history of injury or other apparent cause in January, 1927. Other swellings had since appeared on the head, but had not been opened. The right knee pained occasionally. He had been delivered normally at full term and breast fed. Scarlet fever had occurred at nine months and severe mumps in March, 1927, each with good recovery. There was no history of other infections. His father, 26 years of age, his mother, 25 years of age, and his six sisters were all in good health. Examination.—The right eye was displaced downward and outward, but was otherwise found normal upon examination by the division of ophthalmology. A soft, fluctuant, non-expansile mass about 5×6 cm. was present over the left parietal region, not inflamed. A soft mass 2.5 cm. in diameter underlay a sinus in the right temporalarea which was discharging a serous fluid. The left shoulder was ankylosed in a position of adduction. The patient limped on the right leg, with pain in the right hip on motion. The physical findings were otherwise of no significance. Laboratory study gave the following findings: Hemoglobin, 70 per cent; red blood cells, 3,700,000; white blood cells, 15,000 to 17,000 with polymorphonuclears (neutrophiles) 50 per cent, polymorphonuclears (eosinophils) 1 per cent, lymphocytes 41 per cent, large mononuclears and transitionals 8 per cent. Anisocytosis and poikilocytosis were present. The Wassermann (Kölmer technic) was negative on three examinations, the last two after provocative treatment. The von Pirquet skin test was negative.
Read full abstract