Hand-Schuller-Christian Disease Research Articles

Multi-System Histiocytosis of Langerhans Cells in Boys Aged 2 Years (Histopathology Review)

Langerhans cell histiocytosis (HSL) or histiocytosis X, eosinophilic granuloma, letterer siwe disease, hand schuller christian disease and hashimoto pritzker disease is a disorder caused by the accumulation and proliferation of langerhans cells in various organs of the body that causes tissue damage. The highest prevalence of HSL occurs in children less than 3 years old, with a ratio of 2:1 for boys and girls. The classic appearance of skin manifestations is an eruption resembling seborrheic dermatitis in the folds, axillae, scalp, retroauricular and trunk areas. The appearance of the lesion is yellow to brownish red papules, purpuric and crusted papules that can occur together with erosion. Histopathological examination with staining with S100 or cluster of differentiation 1a (CD1a) revealed Langerhans cell infiltration. The results of immunohistochemistry with IHC S-100 staining, CD1a showed a picture of Langerhans cells and histiocytes. The purpose of this paper is to study the histopathological features of HSL to better than to diagnosis and treatment of HSL. A 2-year-old boy was consulted with the chief complaint of itchy red nodules on the head, chest and back area. Complaints accompanied by diarrhea, often feel thirsty and hungry and lose weight. Dermatological status in the facial region, the anterior and posterior trunks showed lenticular papules with umbilication (pin points) with erosions covered with necrotic tissue above, on the scalp, facial, retroauricular and trunk regions, yellow to brownish papules were crusted with erosions. Histopathology with hematoxylin and eosin (HE) staining showed the presence of small fragments of skin tissue, the epidermis was partially composed intact, the dermis was filled with histiocytes, plasma cells, eosinophils, mast cell impressions and polymorphonuclear leukocytes. Immunohistochemical examination with staining IHC S-100 and CD1a showed positive. Histiocytosis is a group of disorders characterized by the accumulation of cells derived from dendritic cells accompanied by inflammation and causing tissue damage. Histopathological and immunohistochemical examinations serve to establish the diagnosis. The results of immunohistochemical examination with IHC S-100 staining, CD1a will show a picture of Langerhans cells and histiocytes

HAND–SCHULLER–CHRISTIAN DISEASE PRESENTING WITH A CLASSICAL TRIAD AND ORAL MANIFESTATIONS: A RARE CASE REPORT

Hand–Schuller–Christian disease is a multifocal disease that characteristically involves the bone and extraskeletal involvement of the reticuloendothelial system. The most common age group affected is children between 1 and 5 years old. It is a disseminated form of Langerhans cell histiocytosis with a typical triad of exophthalmos, diabetes insipidus, and lytic bone lesions, although seen in only half of the patients. We present such an uncommon case presenting with the classical triad along with oral manifestation such as floating teeth and hepatitis with typical radiological findings.

A rare case report of Langerhans cell histiocytosis (Hand–Schuller–Christian disease) and review of literature

A rare case report of Langerhans cell histiocytosis (Hand–Schuller–Christian disease) and review of literature

Hand–Schuller–Christian disease with condylar aplasia: A rare and unique case

Langerhans cell histiocytosis (LCH) is characterized histologically by a monoclonal proliferation of large mononuclear cells accompanied by a prominent eosinophil infiltrate. The pathogenesis of LCH is unknown. Hand–Schuller–Christian (HSC) disease is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in various organs. The clinical manifestations arise as a result of accumulation and infiltration of the Langerhans cells in organs and tissues. HSC is characterized by a classic triad of exophthalmos, diabetes insipidus, and lytic lesions in the skull. It is usually manifested in children but can also affect adults. Here, we present a case of HSC disease in a 12-year-old boy with oral manifestations.

Dexmedetomidine: A Saviour in a Child with Hand Schuller Christian Disease for MRI

Hand Schuller Christian disease (HSC) primarily affects infants and children. Its classical presentation includes triad of exophthalmos, diabetes insipid us, and calvariallytic lesions. Systemic involvement may include hepatomegaly, lymphadenopathy, dermatological, gastrointestinal tract, renal, pulmonary and CNS involvement. CNS involvement includes convulsions, increased intracranial pressure, focal neurological deficits, mental retardation, hearing disturbance, and tremors. We encountered a 4 year old female child with HSC disease who was posted for elective MRI scan under anaesthesia. Patient’s history, physical examination, and lab reports revealed multitude of problems enumerated in case report. Such patients when posted for anaesthesia pose a major challenge in view of multiple organ involvement. Polydipsia made difficult to keep the patient fasting and giving general anaesthesia as per standard protocol became risky. The loose teeth with bleeding gums and protruded mandible would have compounded the mask ventilation, LMA placement or laryngoscopy and intubation, manifolds. We managed this case successfully with intravenous sedation with inj. dexmedetomidine at 1 μg/kg/min over 10 min and then maintenance infusion at 0.1 μg/kg/min. Spontaneous ventilation was supplemented with oxygen (FiO2 0.60). MRI scanning was completed in 45 minutes comfortably. Hence, dexmeditomidine offers advantage in such patients if anaesthesia has to be given in remote locations like MRI suite.

Hand Schuller Christian Disease: A Rare Case Report with Oral Manifestation

Langerhan's Cell Histiocytosis (LCH) is disorders which include abnormalities that result from abnormal proliferation of langerhan's cells or their precursors. LCH is clinically classified into three types-eosinophilic granuloma, Hand Schuller Christian disease and Abt-Letterer- Siwe disease. It is usually manifested in childhood as well as in adulthood. The clinical manifestations are the result of the accumulation and infilteration of the langerhan cells in organs and tissues. Here is a rare case report of 6-year-old boy with extraoral manifestation of exopthalmic right eye and oral manifestation of mobility of teeth and with typical radiological findings. Basing on the clinical, radiological and histopathological examination the diagnosis of Hand Schuller Christian Disease was given.

A case of Hand-Schüller-Christian disease

[Objective] A case of 34-year-old male was admitted for persistant painful skin ulcer in both axillary regions for 1 year as well as oppression in the chest,fatigue and polyuria for 3 years.He also suffered from diabetes insipidus and exophthalmos.Skin examination showed infiltrated nodules and ulcerated plaques mainly located in the subaxillary and genital regions,as well as widespread skin rashes,scaly or crusted papules.Histopathology revealed a diffuse infiltrate of Langerhans's cells throughout the entire dermis.The tumor cells were positive for CD1a and S-100.Finally,a diagnosis of Hand-Schuller-Christian disease was made based on the histopathological and immunohistochemical findings.At present,no specific treatment is available for Hand-Schuller-Christian disease.Generally speaking,combined chemotherapy is recommended for Hand-Schuller-Christian disease with multisystemic involvement,and surgical operation or radiotherapy for unifocal lesions. Key words: Histiocytosis, Langerhans cells ; Adult; Pathology

Langerhans cell histiocytosis: clinical analysis of 126 cases

Objective To analyze lesional and clinical characteristics of Langerhans cell histiocytosis in children.Methods A clinical retrospective study was performed on 126 patients with Langerhans cell histiocytosis collected from 2006 to 2011 at the Hunan Children's Hospital.Results Of the 126 patients,the youngest was 2months old,and the oldest was 9 years old.The ratio of male to female was 2.5 ∶ 1.Clinical manifestations included eczematid,seborrheic lesions,hemorrhagic maculopapules,yellow nodules and white macules.Of the three clinical phenotypes of Langerhans cell histiocytosis,Letter-Siwe disease was the most prevalent,and most cases of LetterSiwe disease were associated with hepatosplenomegaly,abnormal chest X-ray,impaired hematopoietic function and multifocal bone injuries.The clinical grade was mainly Ⅲ and Ⅳ in patients with Letter-Siwe disease,Ⅰ in patients with eosinophilic granuloma,and varied from Ⅰ to Ⅳ in patients with Hand-Schuller-Christian disease with Ⅱ as the most common.Of these patients,those with eosinophilic granuloma had the oldest average age with bone as the only affected organ,while those with Letter-Siwe disease had the youngest average age with the greatest number of affected organs.The treatment of Langerhans cell histiocytosis included surgical operation and combined chemotherapy.Conclusions Langerhans cell histiocytosis has characteristic skin lesions and diverse clinical manifestations.Pathology has diagnostic significance to Langerhans cell histiocytosis.Therapy strategies and curative effects are dependent on the severity of,and the organs affected by Langerhans cell histiocytosis.

English

Langerhans cell disease previously known as Histiocytosis X is a non lipidreticuloendotheliosis that presents with single or multiple lesions distributed in the soft tissues, lymph nodes, various organs and bones, especially in areas of marked reticulo endothelial activity. Histiocytosis X encompasses three conditions which include a localized form (Eosinophilic granuloma), a chronic disseminated form (Hand Schuller-Christian disease) and an acute or subacute disseminated form (Letterer-Siwe disease). Eosinophilic granuloma is characterized by solitary or multiple skeletal lesions with no extra skeletal involvement whereas Hand Schuller- Christian disease manifests with widespread skeletal and extra skeletal lesions with a chronic clinical course. Letterer-Siwe disease presents with widespread lesions of both skeletal and extra skeletal tissues with a more acute severe clinical course and a high mortality rate. Most often these patients report initially to the dentist because of the significant oral involvement including gingival swelling and mobility of teeth. Such a rare case of acute disseminated Langerhans cell disease that presented to the dental clinic is discussed in this article.

Is Cutaneous Langerhans Cell Histiocytosis in Infants Self-Regressing?

Cutaneous Langerhans cell histiocytosis (LCH) is a spectrum of diseases encompassing eosinophilic granuloma, Hand-Schuller-Christian disease,

Extensive Basal Cell Carcinoma and Disseminated Lesions in Hand-Schüller-Christian disease

Sir, Langerhans’ cell histiocytosis (LCH) includes four clinical entities with a common characteristic of infiltration of LC in different tissues. Biopsy of suspicious lesions and staining for CD1a and S100 protein or antilangerin are needed to establish a definitive diagnosis of LCH. Electron microscopy to identify Birbeck granules is performed less frequently because of time and cost constraints (1, 2). The pathogenesis remains unclear. A neoplastic aetiology has been suggested due to the existence of a single clone in the infiltration, although no cytogenetic abnormalities have been found (3, 4). Most investigators consider LCH to result from an immunological dysfunction (2, 5, 6). The presence of aggregates of other immunologically active cells, decreased numbers of suppressor T lymphocytes and high levels of cytokines in the lesions suggest an exaggerated physiological response of LC to an antigen, or an appropiate response to abnormal signals from other immune cells (1, 7, 8). Although numerous facts are known about the cell mediators, it has not been possible to prove the participation of any factor in the unchaining of the clonal proliferation of the cells in the LCH (9). We present here a new case of basal cell carcinoma (BCC) in association with one type of LCH, HandSchuller-Christian disease, with a probably late specific cutaneous infiltration of the LC.

各種治療にもかかわらず再発を繰り返すＨａｎｄ‐Ｓｃｈｕｅｌｌｅｒ‐Ｃｈｒｉｓｔｉａｎ病の１例

We describe a 37-year-old woman with Hand- Schuller-Christian disease. She was given the diagnosis by a neurosurgeon because of diabetes insipidus and eosinophilic granuloma of the temporal bone. She subsequently had pain in the mandible. She was given a diagnosis of eosinophilic granuloma in the mandible by an oral surgeon. She underwent curettage of the tumor and received chemotherapy with vinblastine sulfate, etoposide, and prednisolone as well as radiotherapy. Despite treatment, the tumor repeatedly recurred. She was operated on nine times by neurosurgeons and twice by oral surgeons during the past 8 years. She is free of disease 20 months after the latest operation.

Bilateral eosinophilic granuloma of temporal bone

Eosinophilic granuloma is a localised painful lesion of either of skull bone, ribs or pelvis with a good prognosis. Calvarial swellings have an affinity to temporal bone. Recently eosinophilic granuloma has been grouped along wiht Letterer Siwe disease and Hand Schuller Christian disease under a common heading called Histiocytosis-X.

Eosinophilic granuloma of the bone in Hand–Schüller‐Christian disease: Extensive in vivo eosinophil degranulation and subsequent binding of released eosinophil peroxidase (EPO) to other inflammatory cells

The eosinophils from bone granuloma, bone marrow, and peripheral blood of a patient with Hand-Schüller-Christian disease (HSCD) were studied by electron microscopy and cytochemistry. Impressive eosinophil degranulation was observed. Extracellular release of eosinophil peroxidase (EPO) and EPO binding to surrounding cells were seen in the granuloma and bone marrow. Cells with peroxidase-positive plasma membrane were also observed in peripheral blood. The pattern of eosinophil degranulation showed quite different features from those described so far. In the granuloma, the process begins with intracytoplasmic release of the granule matrix content, as revealed by both extensive extragranular accumulation of EPO and progressive decrease of the matrix electron density. Core dissolution follows thereafter, leading to complete disappearance of the granules. At the end of the process, the cells show rupture of the plasma membrane and release of their content into the surrounding environment. This pattern of secretion was also observed in blood and marrow eosinophils of the patient. In view of the previously reported findings that EPO binding to inflammatory cells influences their functions, EPO release and binding to surrounding cells in HSCD may play a role in the evolution of the inflammatory lesion in the disease.

A new use of corticosteroids in the combination therapy of a single orbital localization of Hand- Schuller-Christian disease

A case of Hand-Schuller-Christian disease with a single orbital localization successfully treated with surgical curettage and radiotherapy plus local infiltrations of corticosteroids is reported. This form of combination therapy, not previously described, is recommended for such single lesions.

Response of Growth-Retarded Patients with Hand–Schüller–Christian Disease to Growth Hormone Therapy

Abstract Five patients with growth retardation due to Hand–Schuller–Christian disease were treated for a maximum of two years with human growth hormone, which was administered intramuscularly, three times per week, in doses of 2 IU. All patients experienced a significant increment in growth rate in comparison to the year before the therapy. During the first year of therapy the mean growth rate was 7.5 cm—an increase of 5.2 cm over the pretreatment year (p< 0.01). The growth rate during the second year of treatment was less, with a mean increase of 2.8 cm per year (P<0.05). These results are similar to the response of patients with idiopathic growth hormone deficiency and add further evidence that the growth retardation associated with Hand–Schuller–Christian disease is due to an acquired growth hormone secretory deficiency. (N Engl J Med 292:332–333, 1975)

Cerebellar Dysfunction in Hand-Schuller-Christian Disease

Diabetes insipidus and growth retardation are common manifestations of hypothalamic involvement in patients with Hand-Schuller-Christian disease (HSCD). Involvement of other areas of the central nervous system (CNS) has been reported infrequently. We have studied 13 patients with HSCD. Four of these patients exhibited signs of extrahypothalamic CNS disease, manifested primarily by impairment of cerebellar function. One patient showed bilateral dentate nuclear calcification, a radiologic sign not previously reported in this disease. Although histologic examination was not possible, the nature of the clinical and radiologic signs suggest that intracerebellar involvement in patients with HSCD is more common than previously appreciated.

Pituitary function in Hand-Schüller-Christian disease. Evidence for deficient growth-hormone release in patients with short stature.

Abstract Anterior and posterior pituitary function was evaluated in 13 patients with Hand–Schuller–Christian disease, including eight with growth retardation. Growth-hormone reserve, determined by insulin-induced hypoglycemia, arginine tolerance test or pyrogen stimulation, was deficient in seven of the eight short patients and in three of the patients whose disease developed after adult height had been attained. Tests of gonadotropin secretion and adrenocortical and thyroid function gave normal results. A dehydration test demonstrated complete diabetes insipidus in eight patients and incomplete diabetes insipidus in two. Growth-hormone deficiency was usually found when diabetes insipidus was present. Both abnormalities may be accounted for by a single hypothalamic lesion. The short stature frequently observed in patients with this disease may be in part secondary to quantitative growth-hormone deficiency.

Lipid Anomalies in Cases of Xanthelasma

Flat palpebrai xanthelasmas are described by Hogan and Zimmerman as xanthomatosis without hypercholesterolemia, in which a qualitative rather than quantitative defect of lipid metabolism or transport is at fault. Others · have implicated factors at the cel­ lular level, which cause the deposition of cholesterol in histiocytes. In xanthelasmas, this tissue-factor was believed restricted to the palpebrai foam-cell nests, in contrast to the systemic forms of xanthomatosis with­ out hypercholesterolemia (Hand-SchullerChristian disease, Letterer-Siwe disease, and even eosinophil granuloma or nevoxanthoendothelioma). The frequency of coro­ nary artery disease in females afflicted with plain xanthelasmassuggests a general rather than local deposition of cholesterol. Xanthelasma is the most common type of xanthomas and is readily available for chemical analysis. It seemed promising to study the lipids of the xanthelasma itself, to learn which are its main lipid fractions. Comparative studies were then made of the patients' blood. Erythrocyte lipids are in dy­ namic equilibrium with serum lipids,· therefore the erythrocyte lipid surface which is easily accessible for study was in­ vestigated as one of the sites of a lipid-binding tissue factor.

Histiocytosis X (Eosinophilic Granuloma of Bone, Letterer-Siwe Disease, and Schüller-Christian Disease)

The literature on histiocytosis X has been reviewed from 1952 to date. Clinical and pathological observations during this period tended to substantiate the integrated concept of histiocytosis X as a nosological entity embracing eosinophilic granuloma of bone, Letterer-Siwe disease (when this is non-neoplastic), and Schuller-Christian disease. The impression has been reinforced that the lesion of eosinophilic granuloma is the pathological expression of an early, rather rapidly developing reaction to an etiological agent. This appears to be especially true in the chronic disseminated form of the malady (Schuller-Christian disease). Recent recorded experience has added new sites of involvement—the liver, kidneys, female genitalia, and, possibly, the cranial nerves and eye, as well. Altogether, the manifestations of the disease may be quite protean. Especially noteworthy are the numerous reports on pulmonary eosinophilic granuloma, occurring with or without demonstrable lesions elsewhere. Stress was placed on the practical importance of making a clear distinction between acute (or subacute) histiocytosis X and neoplastic reticuloendotheliosis, both of which require different therapy. From existing evidence it would appear that cytotoxic agents, radioactive isotopes, and the like should be avoided in acute disseminated histiocytosis X since they may seriously injure an already damaged bone marrow. In these circumstances, as in many severe cases of chronic disseminated histiocytosis X (Schuller-Christian disease), the experience of recent years points up the value of steroids in tiding patients over critical situations. Collaterally, the value of selected antibiotics in combating secondary infections and of roentgen therapy, especially for mucocutaneous and skeletal lesions, has been further documented. Among the features brought out by our own material are: (1) specific involvement of the liver in chronic disseminated histiocytosis X, leading to serious intrahepatic obstructive jaundice; (2) extension of eosinophilic granuloma in bone to regional lymph nodes (reinforcing the concept of infection); and (3) reappearance of the lesion of eosinophilic granuloma as the pathological expression of recrudescence in chronic disseminated histiocytosis X (Schuller-Christian disease) of long standing.