Sclerosing Lesions Research Articles

Melorheostosis and the sclerotomes: a radiological correlation.

Melorheostosis is characterised radiologically by hyperostotic linear densities in bone. These densities have a peculiarly segmental distribution which does not correspond with the anatomical course of blood vessels or mixed nerve roots of the limbs. So far this disbritubion has lacked any valid explanation, although it has been suggested to be a developmental error as a result of an embryonic metameric disturbance. Inman and Saunders in 1944 described a sensory nerve supply to skeletal structures with 'sclerotomes' representing the zones of the skeleton supplied by individual spinal sensory nerves. Radiographs of 30 cases from the Radiological Museum of the Institute of Orthopaedics, London, have been reviewed and an attempt has been made to correlate the sclerosing lesions with the sclerotomes. The investigation was handicapped by paucity of films and clinical information, but in 19 cases the skeletal abnormalities were considered to correspond with a single sclerotome or part thereof. These studies were convincing when films of an affected hand or foot were available. In the remaining 11 cases multiple sclerotomes appeared to be involved and the clinical manifestations were correspondingly more severe. It is proposed that melorheostosis may be the late result of a segmental sensory nerve lesion, to account for its sclerotomal distribution. The association with linear scleroderma is discussed, since it has been suggested that these cutaneous lesions are related to the same nerve segment. Eight cases showed paraarticular ossification of soft tissues which may be related to involvement of a corresponding myotome.

A Nonencapsulated Sclerosing Lesion of the Breast

A histological study of 15 examples of a unique proliferative and tubular lesion (Fenoglio and Lattes) of the breast was performed. Although it consistently lacked infiltration of adipose tissue, the commonality of some of its morphologic features and those of tubular cancer provokes the possibility that these lesions may represent incipient tubular cancers. Nevertheless, such lesions may be satisfactorily treated by simple excision. Since we are certain only about its topographic and clinical behavior, but not its precise biologic nature, we regard the designation nonencapsulated sclerosing lesion most appropriate. Stromal and periductal elastosis was not infrequently encountered in examples of overt sclerosing and blunt ductal adenosis which may mimic, but can readily be distinguished from, the nonencapsulated sclerosing lesion.

Intrarenal platelet consumption in the diffuse proliferative nephritis of systemic lupus erythematosus.

1. Platelet survival and an index of the localization of platelets in the kidney were studied in patients with the proliferative nephritis of systemic lupus erythematosus, either focal or diffuse, and in control subjects. Platelet survival was reduced in patients with proliferative lupus nephritis, more in those with diffuse rather than focal renal involvement. 2. The index of renal platelet localization in patients with diffuse proliferative nephritis suggested an intrarenal platelet consumption not found in other groups. 3. A patient with the classical platelet autoantibody disease, idiopathic thrombocytopenic purpura, also showed reduced platelet survival but localization of platelets was in the spleen rather than the kidney. 4. Intrarenal platelet consumption in diffuse proliferative lupus nephritis may be an epiphenomenon of pre-existing scarring or platelet aggregation secondary to immune complex-formation, which contributes to the progressive sclerosing lesions of this form of nephritis.

PATHOLOGY OF THE NEPHROTIC SYNDROME IN CHILDREN: A Report for the International Study of Kidney Disease in Children

The renal biopsy findings in 127 previously untreated children with the nephrotic syndrome of recent origin are reported. "Minimal changes" were observed in 98 (77%) patients, and most responded to corticosteroid therapy. In addition to the well-known forms of chronic glomerulonephritis, two distinct but less well recognised conditions are described. In one, focal sclerosing lesions involve the glomeruli to an increasing extent, and may ultimately lead to renal failure; in its early stage the condition may be difficult or even impossible to distinguish from " minimal changes ". Most cases are steroid-resistant. In the other condition there is mild mesangial thickening and proliferation similar to that observed in resolving post-streptococcal nephritis. Although some cases may be steroid-resistant and the clinical course protracted, the prognosis is generally favourable.

Hereditary multiple diaphyseal sclerosis (ribbing).

In 1949 Ribbing (3) described a peculiar form of diaphyseal sclerosis which he had observed in four of six siblings and which differed in some respects from any lesions previously reported. He was unable to determine the cause or nature of the condition and gave it the name of hereditary multiple diaphyseal sclerosis. We have observed two examples, occurring in brothers, of a type of sclerosis resembling that described by Ribbing. Since this appears to be an unusual form of bone disease or dysplasia we are reporting our cases at this time. The essential finding in this disease consists in a fusiform thickening of a portion of the diaphysis of a long bone. The involved area is extremely dense and the cancellous structure largely or completely obliterated. The lesions have been quite similar in their roentgen appearances, varying only in the length of the involved area and the intensity of the sclerosis. There is a tendency toward symmetrical changes and to multiplicity of bones involved, with the tibia and femur most frequently affected. The ends of the shafts are normal. The general health of the patients has been unimpaired, and other congenital stigmata have been absent (exception, our Case 2). The lesions may exist without symptoms or may cause local pain of a deep boring character. The time of onset of the sclerosis is unknown, but in none of the cases have symptoms begun before puberty. A brief résumé of Ribbing's cases is given below: 1. Father, dead at age 68. No record of any skeletal roentgenograms. 2. Mother, living, age 63. No symptoms of osseous disease. Skeletal roentgen study showed normal findings. 3. Son, born May 7, 1905. No symptoms; skeletal roentgenograms normal. Has three children, all with normal skeletal roentgenograms. 4. Daughter, born Nov. 8, 1906. No symptoms; skeletal roentgenograms normal. Has three children, all normal. 5. Daughter, born April 17, 1914. Beginning in 1940, experienced pain in both legs. The pain in the right leg had shown improvement since 1944 and there had been some improvement in the left. In 1941 roentgenograms showed a 7-cm. area of thickening and sclerosis in the middle of the right tibia. There was some increase in the lesion by the end of 1942 and at this time a similar lesion was observed in the left tibia. In 1948, examination showed a marked eburnation about 20 cm. in length in each tibial shaft. The medullary cavity was practically obliterated and the outline of the bone slightly expanded. Examination of the remainder of the skeleton showed a similar sclerosing lesion in the left femur and another in the distal part of the left radius. The patient has one child, whose skeletal roentgenograms are normal. 6. Son, born April 12, 1918. Suffered an injury to the left leg in 1940 by a blow. This was followed by a week of discomfort and then subsidence of symptoms. Several months later a firm swelling was noticed at the site of the former injury.