Langerhans' cell histiocytosis diagnosed due to dermatological perianal lesion / Histiocitose de células de Langerhans diagnosticada por lesão perianal dermatológica

Abstract Langerhans' cell histiocytosis is a rare disease characterized by proliferation of Langerhans cells in the body. It affects mainly males, predominantly in childhood. Ulcerated plaques are one of the cutaneous forms of presentation. Diagnostic confirmation is done through immunohistochemistry. As therapeutic options, topical corticosteroids and chemotherapy are good choices. The case is reported of a male patient, aged 14, with perianal ulceration. He consulted a coloproctologist, who performed a biopsy of the region and started local triamcinolone applications. Immunohistochemistry diagnosed Langerhans' cells histiocytosis. Further investigation revealed diabetes insipidus, osteolytic lesions in the skull and lower limbs, enlarged liver, and encephalic alterations. Chemotherapy was started with Vinblastine, with significant improvement of the lesions.

Resumo A histiocitose de células de Langerhans é uma doença rara caracterizada pela proliferação de células de Langerhans no corpo. A doença afeta principalmente os homens, predominantemente na infância. Placas ulceradas são uma das formas cutâneas de apresentação. A confirmação diagnóstica é feita através de análise imuno-histoquímica. Como opções terapêuticas, corticosteroides tópicos e quimioterapia são boas escolhas. O caso aqui relatado é de um paciente do sexo masculino, com idade de 14 anos, com ulceração perianal. Ele consultou um coloproctologista, que realizou uma biópsia da região e iniciou o tratamento com aplicações locais de triancinolona. A análise imunohistoquímica diagnosticou histiocitose de células de Langerhans. Outros exames revelaram diabetes insipidus, lesões osteolíticas no crânio e nos membros inferiores, aumento do fígado e alterações encefálicas. A quimioterapia foi iniciada com vimblastina, com melhora significativa das lesões.

Oral manifestion of Langerhans cell histiocytosis mimicking inflammation.

Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.

Hand-Schüller-Christian disease.

Langerhan cell histiocytosis, formerly known as histiocytosis X, traditionally denotes a group of diseases that stem from proliferative reticuloendothelial disturbances.The etiology and pathogenesis of the disease remain debatable. In this paper we report a case of Langerhan cell histiocytosis in a 3 1 / 2 -year-old child who reported to the department of oral pathology with gingival enlargements. The radiological features and histopathological features are also discussed.

Histiocitosis de Células de Langerhans en hueso parietal / Langerhans cell histiocytosis in bone parietal

La Histiocitosis de Células de Langerhans es un grupo de enfermedades con un compromiso mono o multisistémico; presenta manifestaciones clínicas y complicaciones que varían según el tejido afectado y la extensi¢n de la lesión; caracterizada por la proliferación de células de Langerhans. Se reporta el caso de una paciente femenina de 2 años y medio de edad, que presenta una masa de 6-7 cm en la zona parietal izquierda, dolorosa, que deforma la anatomía craneal; el cuadro evolucionó en 22 días y asoció anorexia. Un estudio por tomografía axial computarizada, mostró una fractura del hueso parietal asociada a un tumor homogéneo sólido en los tejidos blandos adyacentes, sin calcificaciones. Se realizó una resecci¢n tumoral, craneotomía y craneoplastía. Los estudios inmunohistoquímicos demostaron células S100, CD1a y CD68 positivas...

Langerhans Cell Histiocytosis in the Juvenile Mandible

Eosinophilic granuloma of the mandibular condyle / 대한구강악안면방사선학회지

The present study reports a case of eosinophilic granuloma of the mandibular condyle. Eosinophilic granulomas on the mandibular condyle are very rare, but there are several common clinical and radiographic presentations. The clinical presentations involve swelling on preauricular area, limitation of opening, TMJ pain, etc. The radiographic presentations involve radiolucent lytic condylar lesion with or without pathologic fracture. Sometimes new bone formations are observed. The purpose of the article is to add new cases to the literatures.

Multiple langerhans cell histiocytosis at the lung and mandible: Case report

Hitiocytosis X, recently termed Langergans cell histiocytosis, is a rare disease histologically characterized by an abnormal proliferation of histiocytes, and of which the pathophysiology is still ambiguous. The clinical presentation is highly varied. Typically, there is a solitary or multiple bony involvements, and less frequently, lesions are found in other organs such as the lungs, liver, lymph nodes, skin, and mucosae. Interestingly, so far, there has been no report which shows multiple developments in both soft and hard tissues. In this report, we present a case of hitiocytosis X in both mandible and lung. This may provide some information for understanding the pathophysilogy of the histiocytosis X.

A Case of Primary Benign Histiocytosis X of the Stomach / 대한소화기내시경학회지

To our knowledge, there are only six cases of histiocytosis X confined to the stomach. A rare case of gastric polyposis caused by infiltration of histiocytes is reported. A 35-year-old Korean woman with no particular complaints was found to have numerous polyps all over the gastric wall revealed by radiographic and endoscopic examination. Endoscopic biopsy of polyps showed that there were diffuse solid infiltration of hidstiocytes with indistinct cytoplasmic membrane and an absence of phagocytosis in laminar propria mucosae. Immunohistiochemical stain showed positive reaction for S-100 and CD68 and a negative reaction for cytokeratin in the infiltrating histiocytes. Electron microscopic examination showed foamy vacuoles with digested debris around nucleus and displaced cellular organelles to periphery. Systemic examination showed no abonormalities outside the stomach.

Clinical Characteristics of Pulmonary Histiocytosis X / 결핵

BACKGROUND: Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. METHOD: Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. RESULT: There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. CONCLUSION: In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

A Case of Primary Pulmonary Histiocytosis-X associated With Central diabetes insipidus / 결핵

Pulmonary histiocytosis X is a granulomatous disorder of the lung of unknown cause. Patients with this disease often complain of cough, dyspnea on exertion and , occaionally, chest pain from pneumothorax or bone involvement. However, DI is uncommon in these patients. We report a case of primary pulmonary histiocytosis X with central diabetes insipidus. A 23-year-old man presented with dyspnea suffered from dry cough, exertional dyspnea, polydipsia and polyuria for 4 months. He was a heavy smoker. He was found to have reticulonodular interstitial opacities on chest X-ray film. High-resolution computed tomography revealed thin-walled cysts of various sizes in both lungs. Open lung biopsy was done. On light microscopic examination revealed proliferation and infiltration of Langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and electronmicroscopic examination showed Birbeck granules in Langerhans cells. Water deprivation test showed central-type diabetes insipidus and brain MRI showed no abnormal lesion on suprasellar region. Smoking cessation was recommended. He was treated with oral desmopressin.

Histiocytosis X Involving Orbital Soft Tissue

Histiocytosis X is an uncommon multisystemic disease of unknown etiology which is characterized by the accumulation of histiocytes in various tissues, and variable clinical course. In patients with histiocytosis X, orbital involvement is common and has a distinct prediction for the superotemporal orbital bone. Orbital involvement is almost invariably associated with a osteolytic lesion of the orbital wall. Disease confined to orbital soft tissues without bone involvement is rare. We report the clinicopathologic features of an unusual form of histiocytosis X in a 2-year-old female who showed a rapidly growing lower eyelid mass. No other ocular or extraocular involvement was detected. The mass was excised and diagnosis was confirmed by hematoxylin eosin staining, which showed large histiocytes and a number of eosinophils, plasma cells and lymphocytes. In addition, the histiocytes were immunohistochemically stain for s-100 protein.

Intrathoracic Lesion Showing Multiple Cysts and Pneumothorax / 결핵

Pulmonary histiocytosis X is an idiopathic benign disease characterized by proliferation and infiltration of lung tissue by characteristic Langerhans cells and eosinophils. Pulmonary histiocytosis X is common in young male adults, and shows variable clinical characteristics. We experienced a case of pathologically proven pulmonary histiocytosis X in a 30-year-old man who visit to our hospital due to chest discomfort and cough. The chest radiograph of our patient shows right pneumothorax and characteristic multiple thin-walled cysts on the both upper lung fields. The HRCT shows multiple thin-walled cysts, a few scattered nodules in both upper and right middle lung, and right pneumothorax.

Immunohistochemical, Ultrastructural Studies and Flow Cytometric DNA Analysis in Langerhans Cell Histiocytosis / 中华皮肤科杂志

The skin biopsy specimens from fifteen patients with Langerhans cell histiocytosis were studied. Thirteen out of fifteen cases were positive to S100 protein antibody and peanut agglutinin, but negative to Mac387 and lysozyme antibodies, and three cases were positive for CD1 antibody by immunohistochemistry. Birbeck granules were demonstrated in one of the three cases by electron microscopy. Ten cases were analysed by flow cytometry, six cases (60%) were aneuploid, DNA index was 1.38?0.32, proliferative index was 38.69%. These results showed that these pathognomonic histiocytes were of Langerhans cell origin and their potential malignancy could not be ruled out.

A Case of Histiocytosis-X

The authors have experienced a case of histiocytosis-X. A 7-year-old male patient had three tender masses, one on the right parietal region, another on the left parietal region, the other on the left occipital region and revealed exophthalmus on his left side. Plain skull films showed multiple punched out skull defects and on lumbar spine films, collapse of body of second lumbar vertebra was detected. Among the masses, the largest right parietal one was excised totally. The final diagnosis revealed histiocytosis-X on pathologic basis and he was given subsequent chemotheraphy.

A Case of Pulmonary Histiocytosis-X Associated with Bilateral, Recurrent, and Spontaneous Pneumothorax / 결핵

Histiocytosis-X is a term used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the Pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilateral, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X which is confined by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.

Histiocytosis-X with Giant Occipital Scalp Mass: A Case Report

Histiocytosis is a relatively rare disorder of the reticuloendothelial system involving the proliferation of histicoytes, granulation tissue, and inflammatory cells in many different organ systems1). Thus, the three manifestations of the same basic pathologic process:Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease have been classified as localized, chronic disseminated and acute disseminated histiocytosis-X. They were therefore included under the term histiocytosis-X and this concept has been generally accepted. The authors have experienced one case of histiocytosis-X, a rare disease. A 11 month-old femal patient presented with gradually enlarged palpable mass on the occipital area. The occipital skull was defected in a punched out fashion. The mass was completely removed. The pathologic findings revealed Histiocytosis-X and the patient was given chemotherapy.

Infantile Solitary Eosinophilic Granuloma of the Lymph Node: A case report

Infantile form of histiocytosis X is commonly presented as multiorgan desseminated form such as Letterer-Siwe disease. Lymph node involvement of histiocytosis X is usually accompanied by adjacent bone or skin lesion. Solitary nodal eosinophilic granuloma without evidence of other organ involvement is very rare. A case herein report is a 11 month-old female infant presented with fever and palpable both inguinal lymph nodes. There was neither skin lesion nor hepatosplenomegaly. Laboratory evaluation was within normal range except increased alkaline phosphatase and many neutrophils in urine. Radiologic examination revealed no remarkable bone lesions. And she showed good clinical outcome without evidence of other organ involvements. On microscopic examination of inguinal lymph node it was replaced by infiltration of histiocytes mainly along the sinusoid. Some of histiocytes showed morphologic features of "histiocytosis X cell" having nuclear grooves or multilobulation. Multinulceated giant cells were frequently see. Numerous eosinphils were also infiltrated and showed multifocal microabscess formation. Immunohistochemical staining revealed that majority of histiocytes were postitive for S-100 protein but multinucleated histriocytes, phagocytic histiocytes and those around the abscess were positive for macrophage marker, suck as CD68 and alpha-1-antichymotrypsin. Interestingly some histiocytes showed positivity for both S-100 protein and macrophage marker. These results suggest that histiocytosis X is proliferative disorder of phenotypically heterogenous population of histiocytes in contrast to the theory that it is a proliferative disorder of Langerhans cells.

Macrophage/dendritic Cell Marker Staining Characteristics of Langerhans cell Granulomatosis(Histiocytosis X)

Histiocytosis X is characterized by aggregates of Langerhans cells with other inflammatory cells. These Langerhans cells are antigen-presenting cells to T lymphocytes and identified by characteristic morphology, ultrastructural demonstration of Birbeck granules and immunologic reactivity with OKT-6 and HLA-DR antibodies. In this report, the tumor arising in a 2-years-old baby was examined byimmunostaining with several macrophage/dendritic cell markers. The main tumor cells showed cytoplasmic and nuclear staining with S-100 protein and ring-like surface and paranuclear staining with PNA. However, they were negative for follicular dendritic cell marker CD21, macrophage markers lysozyme, Mac 387, alpha-1 antitrypsin and CD68, and interdigitating reticulum cell marker ID4 and ID5. These observations demonstrate the usefulness of S-100 protein and PNA for the identification of Langerhans cells in paraffin-embedded tissue.

Histiocytosis-X with Chronic Weeping Ulcers in the Anogenital Areas

No abstract available.

Histiocytosis X with Involvement of Pituitary Stalk: Case Report

A case of histiocytosis X with the involvement of pituitary stalk in a 5 year old boy was described. He presented with diabetes insipidus and the endocrinological study showed growth hormone deficiency. On computerized tomography and magnetic resonance imaging, the pituitary stalk was abnormally thick. Overlooking of the skull lesion on plain X-ray film led to an erroneous diagnosis of germ cell tumor'. Gross total removal of the lesion and chemotherapy were performed. In the differential diagnosis of suprasellar masses, this disease entity should be included. The importance of histological diagnosis of the infundibular lesion in cases of central diabetes insipidus is also emphasized.