Case Of Histiocytosis Research Articles

A Case of Histiocytosis X in the Scapula

A boy aged 9 admitted to our clinic on July 1975, complaining of pain and swelling in the right scapular region. Roentgenograms showed a round osteolytic defect, about two centimeters in diameter in the right scapula. The area of bone destruction was filled with yellowish tissue, which was easily curetted from the bone. The histological picture revealed a granulomatous lesion with widely scattered histiocytic cells, and with foci of eosinophilic infiltration. Culture of the tissue did not given bacterial growth. By roentgenograms, the defect showed definite healing sixteen months after curettage.

Histiocytosis X in an Adult with Skin and Uncommon Central Nervous System Involvement

Report of a case of histiocytosis X with adult onset, widespread skin lesions and rare central nervous system involvement resulting in dizziness and ataxia. On autopsy histiocytosis X infiltrates in the central nervous system were demonstrated with granuloma formation in the temporal lobe and nucleus dentatus of the cerebellum.

Histiocytosis X: role of radiation therapy in management with special reference to dose levels employed.

The authors present 30 cases of histiocytosis X and recommend that the disease be classified as local or generalized. Indications for radiotherapy include lesions recurring after simple surgery, lesions in difficult areas, and adjunctive treatment concommitant with surgery and∕or chemotherapy in generalized disease. Dose levels of radiation should not exceed 1,000 rads—the acceptable range for a typical lesion is 550–600 rads, Higher dose levels are not effective and are potentially hazardous to these patients who are usually in the pediatric age group.

The radiology of the bone changes in histiocytosis X

Twenty cases of Histiocytosis X with skeletal involvement have been reviewed. Various points are worthy of special mention. The lesion often appears rapidly in a single site or in multiple foci. Subsequent healing with effective treatment, or sometimes spontaneously, may also occur over a short time. The fact that the lesion may be ubiquitous or remain limited must be recognized. If one lesion is found, a search for further foci is required. Radiology offers little guide to prognosis. Many other conditions may be mimicked.

A cytoplasmic membranous complex in histiocytosis X.

In biopsy specimens obtained from 54 cases of histiocytosis X, and examined by electron microscope, a cytoplasmic membranous complex was observed in a small number of the characteristic cells, either in bone, skin, and lung samples, or in cell cultures obtained from bone lesions. This complex appears to be related to the granular endoplasmic reticulum. Its description includes a study with a tilting microscope; its nature and significance so far are unknown. Similar features observed in other materials, especially in experimental tumors, are reviewed.

HISTIOCYTOSIS X

1. The clinical, histological and radiological findings in 129 cases of histiocytosis X have been reviewed and the natural history of the disease is discussed. 2. Certain clinical and histological factors emerge as having prognostic significance. 3. The treatment in thirty-four personally reviewed cases is discussed and some suggestions are made as to the treatment of particular types of case.

Histiocytosis X: Problems in diagnosis

The dentist may play an important role in establishing the diagnosis of histiocytosis X, since the disease often manifests itself initially in the oral cavity. Some of the more important clinical findings that should arouse the clinician's suspicion include (1) marked periodontal disease or cysts of unknown etiology which do not respond to ordinary therapy, (2) delayed healing following extractions, (3) precocious eruption of deciduous teeth, and (4) diabetes insipidus and/or exophthalmos. Two cases of histiocytosis X have been reported. They illustrate not only some of the characteristic findings in this disease but also the importance of grasping the significance of these findings. Since repeated biopsies may be needed before a definitive diagnosis is made, clinical impressions are important.

Histiocytosis x and double diabetes.

SUMMARYA case of histiocytosis X is reported. This is remarkable because of the prolonged symptomatic course of 22 years, and the association of diabetes insipidus with diabetes mellitus, only once previously recorded in a patient with histiocytosis X.The patient had severe pulmonary involvement with eosinophilic granuloma which was diagnosed by lung biopsy. Treatment with steroids produced considerable histological resolution of active disease. He developed a postoperative pneumothorax, to which these patients are susceptible, and died after a myocardial infarction.There is a short review of the literature relevant to the unusual features of this case.

Xanthomatous cutaneous lesions of histiocytosis X.

The cutaneous manifestations of histiocytosis X, particularly the Hand-Schuller-Christian type, are reviewed. Xanthomatous-appearing lesions, which are rare with histiocytosis X, can be classified according to their clinical and histologic features as (I) yellowish papular and seborrheic lesions; (2) xanthoma disseminatum; (3) juvenile xanthogranuloma; (4) plane, papular, and tuberous xanthomata secondary to complicating biliary cirrhosis; and (5) xanthelasma palpebrarum. The multiple histopathologic patterns that may be found in biopsies of such lesions are illustrated. The rare cases of histiocytosis X associated with cutaneous lesions that show features consistent with xanthoma disseminatum, juvenile xanthogranuloma, and reticulohistiocytoma suggest that these various entities are related clinicopathologic reaction patterns which form a spectrum of histiocytic proliferative disease. Although in most instances these reaction patterns are quite distinct, the occasional occurrence of cases with overlapping features should not be surprising.

Histiocytosis X: Chronic disseminated form (Schüller-Christian disease) with marked arteriosclerosis in a young woman

A case of histiocytosis X, Schüller-Christian disease, in a young woman is presented. The unusual features of this patient are the presence of marked arteriosclerosis of the aorta and large arteries of the lower extremities without hypercholesterolemia, and the continual appearance of fresh lesions over a period of twenty-seven years.

Two cases of acetic acid burns of the cornea.

The magnetic resonance imaging features of two cases of histiocytosis X in the hypothalamus and one in the cerebral hemispheres are described. The lesions were best seen with spin-echo technique using a long repetition time (TR = 1580 msec) and long echo time (TE = 80 msec). Sagittal images provided the best anatomicotopographic evaluation of the lesions. Comparison with computed tomography with and without contrast enhancement showed an advantage with magnetic resonance imaging.