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At least 19 recordsLinked to original sources
PubMed1979

[Oral manifestations of Letterer-Siwe disease. Clinical and histological study of lesions centered in the mouth of a infant with acute and disseminated histiocytosis X].

The clinical, radiological and histological aspects of acute histiocytosis X of Letterer-Siwe disease are reviewed after personal observation of such cases. In spite of many advanced hypotheses the etiopathogenesis of Histiocytosis X is wide open to research. The diagnosis became possible by the discovery of histiocyte cells of a special order, the X-bodies, under the E. M. Recent research allows a classification of Letterer-Siwe disease, Hand-Schüller-Christian disease, and the eosinophilic granuloma under the same nosologic grouping. Finally, the prognosis of the Letterer-Siwe disease-so far much in the dark-may be improved by a properly conducted therapy.

Bone and Bones↗
PubMedDate not supplied

Letterer-Siwe disease: report of case.

Initial oral symptoms of Letterer-Siwe disease reveal the importance of a dentist in establishing this diagnosis. This case is important in that the patient was five months old, at which time an early diagnosis was made and treatment had begun. Because most cases occur in young children, a high level of suspicion is necessary, because the oral manifestations and radiographic findings in children may often resemble the periapical, periodontal or cystic involvements frequently seen first by the dentist. The oral lesions were necrotic, hemorrhagic, and degenerative gingival tissues in the maxillary molar area. Loss of alveolar bone was evident. There was precocious eruption of seven primary teeth, which were slightly mobile. Dentally, prophylaxis and fluoride applications were supportive measures.

Bone Marrow↗
PubMed1978

Idiopathic differentiated histiocytosis.

Eighty-nine cases variously diagnosed as histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, or malignant histiocytosis from one institution were reviewed. Follow-up data were obtained for all patients. On the basis of clinicopathologic correlation, the following distinct groups were identified. 1. Disseminated histiocytosis (12 patients) with involvement of more than two organ systems at the time of recognition of the disease, affecting children less than 3 years of age (the patient's general condition is poor and hepatosplenomegaly is common. The patients do poorly, and all the patients in this group of the present study died. 2. Histiocytosis confined to bone, whether monostotic (36 patients) or polyostotic (eight patients). This type is self-healing, does not disseminate, and does not require intensive therapy. Clinically the patients are older and in good general condition during the course of the disease. Histologically histiocytes in these two groups are cytologically benign, and the whole process is inflammatory rather than neoplastic. Presence of giant cells, eosinophils, and necrosis are usually associated with better prognosis. Many neoplastic diseases and infectious processes manifest as histiocytosis. More than a third of the cases of this study diagnosed as histiocytosis proved to be other processes. This confusion contributes to differences of various reports and difficulty of interpretation of findings.

Adolescent↗
PubMedDate not supplied

Electron microscopic study of the orbital lesion of Hand-Schüller-Christian disease.

The orbital bone lesion of a patient with Hand-Schüller-Christian disease was examined by electron microscopy. Vacuoles in the cytoplasm of foam cells contained granules which were shown by histochemical examination to be cholesterin. Langerhans cell granules in the histiocytes of Hand-Schüller-Christian disease showed a close resemblance to those in Langerhans cell of the epidermis, eosinophilic granuloma and Letterer-Siwe disease. Charcot-Leyden crystals appeared to be formed in the lysosomes of histiocytes in Hand-Schüller-Christian disease. From histopathological and cytological points of view, eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease are classified as histiocytosis X. The simultaneous appearance of Langerhans cell granules and Charcot-Leyden crystals is specific in histiocytosis X.

Bone Diseases↗
PubMed1978

[Adult Letterer-Siwe's disease. Review of literature (author's transl)].

Letterer-Siwe syndrome is usually encountered in children. It very rarely occurs in adults. 23 cases are found in the literature and analyzed: clinical signs are the same as in infantile observations. Prognosis in some cases is surprisingly very good when only the skin is involved.

Adolescent↗
PubMedDate not supplied

A review of histiocytosis X with particular reference to eosinophilic granuloma of the lung.

Electron microscopy confirms the unity of Histiocytosis X (HX) by identifying a common marker organelle in all three forms of the disease (acute disseminated HX or Letterer-Siwe disease; chronic disseminated HX or Hand-Schuller-Christian disease; localized HX or eosinophilic granuloma). The marker organelle of HX is also found in normal Langerhans' cells: these mononuclear cells are distinct from macrophages but are similarly concerned in antigen transport. HX may therefore be considered to represent a pathological proliferation of Langerhans' cells. The natural history and the clinical, radiological and pathological features of primary pulmonary HX (eosinophilic granuloma of the lungs) are described, together with the differential diagnoses which must be considered by the histopathologist.

Adult↗
PubMed1977

Mitotic histiocytes and intranuclear Langerhans cell granules in histiocytosis X.

Several mitotic histiocytes containing Langerhans cell granules were found in the lymph nodes of Letterer-Siwe disease. Some histiocytes of Hand-Schüller-Christian disease contained Langerhans cell granules within the nuclei. These Langerhans cell granules were not in 'nuclear pseudo-inclusions,' but were freely scattered inside the nuclei. We suggest that the Langerhans cell granules may get trapped in the nucleus during mitosis, since several investigators have suggested that mitosis causes other intranuclear organelles to get trapped. We also speculate that cells containing Langerhans cell granules may divide and increase by mitosis.

Cell Nucleus↗
PubMed1979

Histiocytosis X of the ear and temporal bone: review of 22 cases.

Histiocytosis X encompasses three syndromes characterized by the idiopathic proliferation of histiocytes: eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. At the Mayo Clinic between 1926 and 1978, 22 patients with histiocytosis X had involvement of the ear or temporal bone. These patients comprised 15% of all patients with histiocytosis X seen during that period. The ages at onset of the disease ranged from 2 months to 49 years. The most frequent otologic symptom was aural discharge (15 patients), followed in frequency by swelling in the temporal region (11 patients), vertigo (6 patients), and deafness (5 patients). Clinical findings included otitis media (13 patients), otitis externa with or without granulation tissue (10 patients), and osteolytic lesions of the temporal bone (9 patients). A high index of suspicion is required to recognize the otologic manifestations of histiocytosis X for two reasons: the systemic manifestations of the disease are often so dramatic that the ear findings are overlooked, and the otologic findings of histiocytosis X can mimic more common diseases, including simple otitis externa, aural polyps, acute mastoiditis, chronic otitis media, and metastatic lesions.

Adolescent↗