Xanthomatosis (Schüller-Christian Syndrome)

Merritt, Katharine

doi:10.1001/archpedi.1933.01960070134010

Cited by 23 publications

(4 citation statements)

References 13 publications

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“…It is difficult to accept Siwe's view that they are atypical examples of Hand-Schuller-Christian disease. In the case reported by Merritt and Paige (1933) the histological appearances of many of the lesions were very similar to those found in the case we have reported. Foam cells were found in the thymus and femur but early lipoid changes were present in these sites in our case also.…”

Section: Discussionsupporting

confidence: 86%

“…Frequently a case, such as that reported by Merritt and Paige (1933) and others, may run a subacute course for many months, and the condition in the later stage tends to resemble the lipophagic form known as Hand-Schuller-Christian disease. The blood cholesterol is usually normal, but in the case reported by van Creveld and Ter Poorten (1935) the blood cholesterol was 147 ARCHIVES OF DISEASE IN CHILDHOOD 596 mg. %.…”

Section: Discussionmentioning

confidence: 98%

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Reticulondothelial Granuloma: A Review with a Report of a Case of Letterer-Siwe Disease

Claireaux¹,

Lewis²

1950

Archives of Disease in Childhood

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 98%

Reticulondothelial Granuloma: A Review with a Report of a Case of Letterer-Siwe Disease

Claireaux¹,

Lewis²

1950

Archives of Disease in Childhood

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“…This is of considerable interest, for the child presented with a flexural eruption and, although the lesions progressed to affect the trunk and scalp, there was never any involvement of the face such as is usually seen in cases of infantile eczema. A similar affection of the skin with the same distribution and reddish-brown scaly appearance is known to occur in cases of Hand-Schuller-Christian disease (Merritt and Paige, 1933;Lane and Smith, 1939;Curtis and Cawley, 1947), but has not, so far as we know, been reported in cases of Letterer-Siwe disease other than in the one described by Reye. At necropsy there was a remarkable similarity between our two cases.…”

Section: Discussionsupporting

confidence: 58%

Two Cases of Hand-Schuller-Christian Disease in Infancy

Nash¹,

Cavanagh²

1952

Archives of Disease in Childhood

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The diversity of the clinical features of HandSchuller-Christian disease is well recognized. Although it is a disorder affecting chiefly young children, its occurrence in the first year of life is rare.The two cases presented here indicate that the form of the disease found in infants tends to be more rapid in its course and the lesions more widespread than in older children. At October 25, 1944, at the age of 9 months. Her parents and an elder brother and sister were quite well, and the pregnancy and labour had been normal. There was some difficulty with feeding until the age of 6 weeks when breast feeding was replaced by a cow's milk mixture. At the age of 3 weeks her mother noticed that there were three red spots on the roof of the mouth. The significance of this observation is not known and no further mention of it is made in the case notes.At the age of 4 months (five months before admission) the right side of the face became swollen. At first the child was thought to be suffering from mumps but shortly afterwards she was admitted to a hospital near her home, and the cervical lymph nodes were incised. The only material obtained at the operation was said to be congealed blood. The lymph nodes on both sides of the neck increased in size and the child's general condition deteriorated rapidly.On admission she was extremely ill, pale, dyspnoeic and cyanosed. The temperature was 960 F., the pulse 154 per minute and respirations 86 per minute. Large subcutaneous masses were found on both sides of the neck. They were firm, smooth, and fixed to the deep structures. The percussion note at both posterior lung bases was impaired and coarse crepitations were heard in all areas. The abdomen was very distended, the liver just palpable, and the spleen was firm and enlarged to three fingersbreadths (2 in.) below the costal margin.

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“…on the one hand, in the early stages of evolution of Letterer-Siwe disease one may find destructive skeletal lesions resembling cytologically those of eosinophilic granuloma of bone, and, on the other hand, in its chronic stages Letterer-Siwe disease takes on the aspect of Schuller-Christian disease, the skeletal and even certain visceral lesions undergoing lipogranulomatous transformation.' This means that the classification on histological grounds of a given example of the disorder may be difficult, and the literature contains many accounts of illustrative transitional types (Guizetti (1931), Merritt and Paige (1933), Grady and Stewart (1934), Hertzog, Anderson, and Beebe (1940), Curtis and Cawley (1947), Weinstein, Francis, and Sprofkin (1947)). Clinically, however, there is a broad correlation of the different disease groups 97 98 ARCHIVES OF DISEASE IN CHILDHOOD with age, and Mallory (1942) has summarized the position clearly (p. 956) as follows:…”

mentioning

confidence: 99%