Hand-Schuller-Christian Disease Research Articles

Absence of thirst in association with hydrocephalus.

DEHYDRATION is commonly seen in patients with impaired consciousness whose thirst mechanism is undamaged, but who are unable to ask for water.1 A few cases have been described, however, in conscious patients who, for a variety of reasons, have lost the ability to experience thirst. Avioli and his associates,2 for example, have recently reported absence of thirst in a twelve-year-old girl with extensive hypothalamic damage caused by Schuller–Christian disease. In the case presented below, the patient, although fully alert and carrying on normal activities, required hospitalization twice in a five-year period for severe dehydration and hypernatremia. Investigation revealed an inability . . .

Histiocytosis X (Schuller-Christian disease): a clinico-pathological survey, review of ten patients and the results of prednisone therapy.

Histiocytosis X (Schuller-Christian disease): a clinico-pathological survey, review of ten patients and the results of prednisone therapy.

PULMONARY EOSINOPHILIC GRANULOMA: A CLINICAL AND PATHOLOGIC DISCUSSION

Excerpt INTRODUCTION In 1953 Lichtenstein1proposed the name histiocytosis X to designate the three related disease complexes, Schuller-Christian disease, Letterer-Siwe disease, and eosinophilic g...

Hand-Schüller-Christian disease; report of a case with unusual lung changes.

Hand-Schüller-Christian disease; report of a case with unusual lung changes.

Hand-Schüller-Christian disease or eosinophilic xanthomatous granuloma

A case of Hand-Schüller-Christian disease is presented with detailed description of lesions found at necropsy. Some of the theories concerning pathogenesis are briefly discussed.

Two Cases of Hand-Schuller-Christian Disease in Infancy

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 the same time they appear clinically and in certain pathological respects to occupy a position intermediate between the more common varieties of Hand-SchullerChristian disease and the non-lipoid reticuloendotheliosis of infants known as Letterer-Siwe disease. It is considered that this report provides further evidence in support of the view that these two conditions are closely related, particularly on clinical grounds, although some pathological evidence is brought forward to suggest that there may be good reason to separate Letterer-Siwe disease and the acute variety of Hand-Schuller-Christian disease on the basis of histology.

RETICULOGRANULOMA

ONE OF the most interesting recent developments in the field of pathology is the concept, first advanced by Farber and Green, 1 that granuloma of is the mildest form of a group of reticuloendothelial diseases. Other members of this group of peculiar inflammatory histiocytosis (Jaffe and Lichtenstein 2 ) are Hand-Schuller-Christian and Letterer-Siwe diseases. If this concept is correct, it was to be expected that cases of eosinophilic granuloma would be observed in which other organs besides the bones are affected. The first case of this kind was published by Curtis and Cawley. 3 Their patient was a 2 year old girl who had cutaneous manifestations of specific character, in addition to multiple involvement of bones. The case which forms the basis of our report was seen at the Marshfield Clinic in January 1946 and was diagnosed clinically as Schuller-Christian disease. Tissue sections of the bone lesion, however, were

Supplementary Remarks to Article on schuller-Christian's Disease

Supplementary Remarks to Article on schuller-Christian's Disease

SYSTEMIC RETICULOENDOTHELIAL GRANULOMA

SYSTEMIC reticuloendothelial granuloma is the generic term used by Wallgren 1 in 1940 to include Letterer-Siwe disease and Schuller-Christian disease. During the past two decades there have been numerous articles devoted to this subject, largely by pathologists, pediatrists, internists and orthopedists. Dermatologists, however, have apparently paid little attention to the cutaneous manifestations of systemic reticuloendothelial granuloma, judging from the paucity of articles which have appeared in dermatologic journals. In articles written within the past few years, Farber, 2 Mallory 3 and Jaffe and Lichtenstein 4 also included eosinophilic granuloma of bone in the group of systemic reticuloendothelial granulomas. The following classification was proposed by Mallory: 3 The disease may mainfest itself in infancy or early childhood in a rapidly fatal form in which the lesions are widely distributed both through the soft tissues (especially the lymphoid tissues) and the skeleton (so-called Letterer-Siwe disease, reticulosis or nonlipid histiocytosis). It

Serum Lipids and Their Value in Diagnosis

Normocholesteremia and Foam-Cell Formation Increased xanthoma formation in essential xanthomatosis of the normocholesteremic type. This condition is synonymous with Schuller—Christian disease, lipi...

EOSINOPHILIC GRANLOMA OF BONE SYNONYMOUS WITH SCHÜLLERCHRISTIAN DISEASE, LIPID GRANULOMA, ESSENTIAL XANTHOMATOSIS OF NORMOCHOLESTEREMIC TYPE AND EOSINOPHILIC XANTHOMATOUS GRANULOMA

To the Editor: —The reader of the article Eosinophilic Granuloma of Bone: Report of Case with Multiple Lesions of Bone and Pulmonary Infiltration, by A. Weinstein, H. C. Francis and B. F. Sprofkin, published in this journal ( 79 :176 [Feb.] 1947) may become confused on the issue of whether eosinophilic granuloma of the bone is newly discovered entity or whether it is part of systemic disorder formerly described under different designations such as Schuller-Christian disease (Rowland, 1928), lipid granulomatosis (by Chester, 1930, and Fraser, 1934) and essential xanthomatosis of the normocholesteremic type (Thannhauser and Magendantz, 1937). The following presentation illustrating historically the conception of the pathogenesis of the may contribute toward the clarification of some misunderstanding concerning its classification. Schuller described in 1915 1 of the membraneous bones of the skull, referring later, in 1921, 2 to a peculiar syndrome of dyspituitarism. Christian in 1919 3 called attention to clinical syndrome consisting in defects in membraneous bones,

Observations on Over One Hundred Cases of Myelogenous and Lymphatic Leukemia

The leukemias constitute a subdivision in the group of reticulo-endothelial neoplasias and present symptoms and clinical features which closely parallel those of the entire group. In order of the approximate frequency of their appearance, the reticulo-endothelial neoplasias can be listed as follows: 1. Leukemias (myelogenous, lymphogenous, monocytic, etc.) 2. Lymphosarcoma, including reticulum-cell sarcoma, large- and small-cell lymphosarcoma. 3. Malignant lymphadenoma or Hodgkin's disease. 4. Multiple myeloma, plasma-cell myeloma. 5. Endothelioma. 6. Ewing's tumor or endothelial myeloma of bone. 7. Tumors of the storage reticulum, including Niemann-Pick disease, Schuller-Christian's disease, Gaucher's disease. Except in the case of the storage-cell tumors, the life expectancy, type of morbidity, and response to radiation or drug therapy follow a closely similar pattern in all these diseases, though there are numerous individual variations by virtue of which the subclassifications have arisen. In the well...

Hand–Schüller–Christian Disease

Hand-Schuller-Christian disease is a chronic disseminated form of Hystiocytosis-X in which there is minimal or moderate visceral involvement in addition to bone lesions. The manifestations of the disease usually begin at two or three years of age. The commonest site of origin is the intraosseous reticulum and its derivatives. In this paper a case of Hand- Schuller-Christian disease and its clinical and radiological findings have been presented.

ACUTE IDIOPATHIC CHOLESTEROL GRANULOMATOSIS

In 1893 Hand1described the peculiar of a 3 year old boy with tuberculosis and polyuria; the disorder was later recognized as idiopathic xanthomatosis. In 1915 Schuller,2in roentgen studies of the bones, noted osseous defects, especially common in the skull, associated with exophthalmos and diabetes insipidus. Christian3in 1919 gave the classic description of the disease, which he regarded as resulting from endocrine glandular dysfunction. In 1923 Eppinger4expressed the opinion that this disease occurred as a result of disturbance of lipoid metabolism, a theory which was later confirmed chemically by Epstein and Lorenz.5Rowland6was the first to express the idea that Schuller-Christian disease is a primary disturbance of lipoid metabolism leading to secondary proliferation of the reticuloendothelial cells. This opinion has been widely accepted.7 Letterer8in 1924 described the case of a 6 month old child who had reticuloendotheliosis with little lipoid storage.

Schüller-Christian's Disease

Schuller-Christian's disease is being recognized with increasing frequency, some 150 cases having been reported up to the present time. Usually, it is a rapidly progressive, fatal disease of early childhood. It is much less common in adults. We were able to find in the literature only five reports of patients surviving the age of forty. Our case, we believe, is the sixth in this category. Miss H. D., a forty-seven-year-old, unmarried white woman was seen by one of us (G. C. O.) Feb. 6, 1940, complaining of headache and weakness of seven months' duration. Dizziness and blurred vision were also present. A large central scotoma before the left eye had been observed for four years. Polyuria and polydipsia had been present for twentyone years. The patient's illness began at the age of twenty-six with rapidly increasing thirst and polyuria. Three months later she suffered a nervous breakdown, and was delirious for seven weeks. Following recovery she complained of pain in the left leg because of which, in the co...

ACUTE MASTOIDITIS COMPLICATED BY SCHULLER-CHRISTIAN DISEASE

The unusual combination of a common pyogenic infection and a rare and infrequent metabolic disturbance of the bones of the skull has prompted the following report of a case of Schuller-Christian disease. The basis on which acute streptococcic otitis media and mastoiditis developed in a child 3 years old was felt to be impaired nutrition of the temporal bone. The case presented the usual problem of a simple mastoidectomy. After the evacuation of the postaural abscess, the mastoid cortex was found to be a sequestrum, which was easily removed, with most of the mastoid cells. This was thought to be unusual, and after the completion of the operation, roentgenograms of the skull were taken, which revealed the presence of Schuller-Christian disease involving both sides of the skull, as shown in figure 1. The opposite ear had not shown any evidence of an acute infection, even though roentgenograms revealed a similar

Schüller-Christian Disease

THE case here described is the form of xanthomatosis (1), known as Schuller-Christian's disease, exhibiting “defects in the membranous bones, diabetes insipidus, and exophthalmos” (2), Rowland has shown that this disease belongs to the general group of lipoid disturbances which include Niemann-Pick's, Goucher's disease, and cutaneous xanthomatosis. He believes it is a primary constitutional change affecting lipoid metabolism, and perhaps a superimposed dysfunction of the liver and lungs resulting in excess accumulation of lipoids in the blood and body fluids, and then in massive storage or infiltration in the tissues. This he supports by findings in animal experimentation of cholesterol feeding. Our case belongs to that sub-group which is characterized by excess cholesterinemia, by occurrence in childhood usually between the ages of two and fifteen years, by defects in the flat bones, and a sequence of other signs depending on the location of the deposits. It is obvious that the deposition of the lipoid a...

II.—Schüller's Disease (A Reticulo-Endotheliosis)

Cases of Schuller's disease have been recorded under the following titles:— Schuller-Christian disease. Hand-Schuller-Christian syndrome. Christian's syndrome. Xanthomatosis. Xanthomatose cranio-hypophysaire. Lipoidgranulomatosis. Lipoid histiocytosis. Xanthomatosis Generalisata Ossium. Xanthoma Diabeticorum. In 1915, Artur Schuller, of Vienna, described two cases of bony lesions in young people. His first case was a boy of 16, who had an unusual development of fat, particularly above the clavicles, and in the arms, legs, and abdomen. X-ray examination of the skull revealed multiple erosions in the bone. The sella turcica was small but intact. Exophthalmos was present, and Schuller suggested that the tumour was an angioma of the dura or of the skull bone. His second case was a girl aged 4 years. When 2 years old, she developed left exophthalmos, and at the same time a defect in the skull was observed. A few months later, right-sided exophthalmos suddenly appeared. There was marked increase of thirst and p...