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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis within the orbit generally occurs in children and is marked by periorbital pain and swelling. Although these lesions respond well to minimal local curettage and concomitant corticosteroid injection, this disorder can be extremely aggressive and destructive. Recurrences usually occur within 12 months to 18 months after diagnosis. Herein, we discuss a patient who initially had mastoid and maxillary lesions and developed unilateral orbital involvement with periorbital pain and edema after a 10-year disease-free interval, and review other cases of late recurrence. The patient's symptoms resolved after curettage and injection of intralesional steroids. Clinicians should be aware that Langerhans cell histiocytosis may reappear at other sites, including the orbit, even after several years of quiescence.
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PMID:Langerhans cell histiocytosis of the orbit 10 years after involvement at other sites. 1835 21

Langerhans cell histiocytosis frequently manifests as lesions of the skull. The disease can present with a wide spectrum of forms, from an isolated eosinophilic granuloma to multiple lesions with diffuse systemic involvement. The authors report the case of a 12-year-old boy with a 1-month history of left temporal and periorbital pain and headaches. Noncontrast computed tomography of the head was done at the time of initial presentation and was interpreted as normal. Over the next month, the patient continued to have headaches and periorbital swelling and began having bloody discharge from his nose. Magnetic resonance imaging of the brain ordered by his pediatrician showed a lesion in the left infratemporal fossa, left orbit, and sphenoid bone. The lesion was biopsied and confirmed to be Langerhans cell histiocytosis. We describe a novel multidisciplinary approach for treatment of this tumor.
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PMID:Novel multidisciplinary approach for treatment of langerhans cell histiocytosis of the skull base. 1859 19

Langerhans cell histiocytosis (LCH) is a rare disease, probably an atypical myeloproliferative syndrome, with variable clinical presentation and behavior. In this report, we focus on bone involvement by LCH and treatment with zoledronic acid in six patients as they progressed after chemotherapy and radiotherapy. Zoledronic acid appeared safe and produced significant relief in pain.
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PMID:Zoledronic acid in treatment of bone lesions by Langerhans cell histiocytosis. 1901 58

The hereditary dentine disorders, dentinogenesis imperfecta (DGI) and dentine dysplasia (DD), comprise a group of autosomal dominant genetic conditions characterised by abnormal dentine structure affecting either the primary or both the primary and secondary dentitions. DGI is reported to have an incidence of 1 in 6,000 to 1 in 8,000, whereas that of DD type 1 is 1 in 100,000. Clinically, the teeth are discoloured and show structural defects such as bulbous crowns and small pulp chambers radiographically. The underlying defect of mineralisation often results in shearing of the overlying enamel leaving exposed weakened dentine which is prone to wear. Currently, three sub-types of DGI and two sub-types of DD are recognised but this categorisation may change when other causative mutations are found. DGI type I is inherited with osteogenesis imperfecta and recent genetic studies have shown that mutations in the genes encoding collagen type 1, COL1A1 and COL1A2, underlie this condition. All other forms of DGI and DD, except DD-1, appear to result from mutations in the gene encoding dentine sialophosphoprotein (DSPP), suggesting that these conditions are allelic. Diagnosis is based on family history, pedigree construction and detailed clinical examination, while genetic diagnosis may become useful in the future once sufficient disease-causing mutations have been discovered. Differential diagnoses include hypocalcified forms of amelogenesis imperfecta, congenital erythropoietic porphyria, conditions leading to early tooth loss (Kostmann's disease, cyclic neutropenia, Chediak-Hegashi syndrome, histiocytosis X, Papillon-Lefevre syndrome), permanent teeth discolouration due to tetracyclines, Vitamin D-dependent and vitamin D-resistant rickets. Treatment involves removal of sources of infection or pain, improvement of aesthetics and protection of the posterior teeth from wear. Beginning in infancy, treatment usually continues into adulthood with a number of options including the use of crowns, over-dentures and dental implants depending on the age of the patient and the condition of the dentition. Where diagnosis occurs early in life and treatment follows the outlined recommendations, good aesthetics and function can be obtained.
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PMID:Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. 1902 96

A 2 years old male child was admitted in Dhaka Shishu Hospital with one month history of fever, swelling and pain in joints of right leg. Hematological and microbiological investigation revealed normal except radiological findings when there were destructive lesions of affected bones and thought to be Langerhan's cell histiocytosis but did not respond to treatment. Subsequently, he was transfer to Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University (BSMMU) for proper management. On examination, the boy was mildly pale, severe bone pain, no organomegaly but few occipital group of lymphnode were palpable. He had extensive red tender swelling over the hands and legs. Hematological values showed high WBC, low platelet count and lymphoblasts. Biochemical values were high serum LDH and serum uric acid. Skeletal survey showed diffuse osteolytic lesion and osteoporosis with evidence of transverse metaphyseal radiolucent bands (leukemic line), lamellar periosteal reactions and cortical erosions widespread throughout the skeleton with subperiosteal new bone formation. Radioisotop of skeleton showed increase uptake at the site of lesions. Bone marrow aspiration findings were suggestive of ALL-L1 and cytochemistry of aspirate showed PAS positive and Sudan black negative. Immunophenotype confirmed as pre-B ALL, Then, protocol based induction had given for 4 weeks. Thereafter, a short course of intensification followed by maintenance therapy had started with significant improvement of physical, hematological and radiological findings. So, an awareness of varied clinical and radiological manifestations of childhood ALL in bone marrow and skeletal system are needed in order to establish a correct diagnosis when the presenting signs and symptoms are enigmatic. It is also important to keep in mind the possibility of extensive skeletal involvement in ALL, especially when a child present with pain, swelling of limb with walking difficulties. The curability of ALL in contrast to metastatic malignancies makes accurate diagnosis of paramount importance.
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PMID:Extensive skeletal lesions in childhood acute lymphoblastic leukemia. 1918 58

This article presents 2 uncommon cases of Langerhans cell histiocytosis of the acetabulum in adults. Langerhans cell histiocytosis is a benign lesion of bone with a diversity of radiographic appearances depending on location. Radiographs of our cases showed a clearly defined radiolucent area at the acetabulum. Magnetic resonance imaging revealed specific findings for Langerhans cell histiocytosis of the acetabulum. Gadolinium-enhanced images showed that abnormal signal was spread to the whole acetabulum surrounding the lesion. Orthopedic surgeons should be aware of the existence of this lesion and attentive to patients who report spontaneous hip pain, although Langerhans cell histiocytosis is a rare condition in adults. It is generally agreed that prognosis is more dependent on age at the time of diagnosis rather than on therapy. We recommend simple curettage for Langerhans cell histiocytosis of the acetabulum in adults to obtain a definite diagnosis and relieve pain.
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PMID:Langerhans cell histiocytosis of the acetabulum in adults. 1938 4

Langerhans cell histiocytosis is a rare disease in childhood. It has a very polymorphous clinical expression, ranging from a single bony disease to a multisystemic disease involving vital organs. Prognosis has been improved by use of chemotherapy. We report a 2-year-old girl with multifocal Langerhans cell histiocytosis of bone and skin, revealed by a pain of the left hip and a limp trailing from 2 months. The diagnosis has been established by histological exam. The patient received chemotherapy and steroids. Outcome was favourable over 6-month follow-up. Trailing limp should evoke diagnosis of Langerhans cell histiocytosis. Skin biopsy should be preferred to a bone biopsy because of its safety.
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PMID:Langerhans cell histiocytosis revealed by painful hip: a case report. 1976 29

Localized Langerhans cell histiocytosis (LLCH), formerly known as eosinophilic granuloma, mainly affects the skull, mandible, vertebrae, and ribs in children and the long bones of adults. Symptoms range from none to pain, swelling, and tenderness over the site of the lesion. General malaise and fever occasionally are present. Radiographically, lesions appear as radiolucent areas with well demarcated borders. LLCH may resolve spontaneously after biopsy in a period of months to years. However, if features include continuous pain, decrease of function, pathologic fractures, migration and resorption of teeth, or rapid progression, then active treatment needs to be considered. Treatment approaches include surgery, radiotherapy, chemotherapy, and intralesional injection of corticosteroids. In children with mandibular LLCH, 1 dose of methyprednisolone succinate injection has proven to be adequate. However, injections have not been performed in cases involving pathologic fracture. We report a new case of LLCH of the mandible that caused a pathologic fracture in an adult patient. Repeated intralesional corticosteroid injections resulted in fracture line disappearance within 14 months and lesion healing by the end of the 36-month follow-up.
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PMID:Treatment of localized Langerhans' cell histiocytosis of the mandible with intralesional steroid injection: report of a case. 2045 43

Langerhans cell histiocytosis (LCH) most commonly occurs as a localized solitary bone lesion and appears predominantly in pediatric patients. LCH is characterized by the proliferation and accumulation of Langerhans cells which may cause pain and adjacent soft-tissue swelling. This disease is of clinical importance to dental professionals because LCH commonly involves the oral and maxillofacial region and early symptoms can be manifested in the jaw and, when overlooked, may result in extensive destruction of the involved structures. Considering the fact that this is a relatively rare entity, close investigation of the cases that are encountered are warranted. Herein we report a case of LCH occurring in the left condyle of an 11-year-old boy that initially showed clinical symptoms mimicking an abscess of the temporomandibular joint. The clinical and radiographic features, differential diagnosis, treatment, and long-term follow-up of this patient with indomethacin are presented.
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PMID:Long-term treatment of Langerhans cell histiocytosis of the mandibular condyle with indomethacin. 2045 43

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.
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PMID:Langerhans cell histiocytosis causing cervical myelopathy in a child. 2061 93


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