scholarly journals Revealing a rare inflammatory oral manifestation in a 6-year-old child

2019 ◽  
Vol 12 (7) ◽  
pp. e229483
Author(s):  
Samapika Routray ◽  
Amit Kumar Adhya ◽  
Joseph John ◽  
Punit Dikhit
Keyword(s):  
Bone Loss ◽  
Buccal Mucosa ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestation ◽  
Atypical Cells ◽  
History Of ◽  
Permanent Molars ◽  
Superficial Dermis ◽  
Distal Aspect

A 6-year-old child with an episodic history of ulcerations over buccal mucosa was found to have severe inflammation on the palatal aspect of permanent first molars with grade 2 mobility bilaterally. Radiographical features were suggestive of bone loss around permanent molars extending to the distal aspect of the deciduous first molars. The clinical and radiographical findings were indicative of periodontal degeneration without any apparent cause visible intraorally. Further biopsy was done from the rashes present on the malar prominences, which showed nodular aggregates of atypical cells in superficial dermis. These large histiocytic cells with vesicular nuclei and nuclear grooves were immunopositive for CD1a and S100, concluding the diagnosis of Langerhans cell histiocytosis. For treatment, patient was referred to Department of Haemato-oncology and chemotherapy was suggested as per protocol.

Progressive Mucinous Histiocytosis: Importance of Electron Microscopy to Confirm Diagnosis

2010 ◽  
Vol 14 (5) ◽  
pp. 245-248 ◽  
Author(s):  
Iman Hemmati ◽  
W. Alastair McLeod ◽  
Richard I. Crawford
Keyword(s):  
Electron Microscopy ◽  
Light Microscopy ◽  
Clinical Examination ◽  
Crucial Role ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
History Of ◽  
Characteristic Features ◽  
Immunohistochemical Studies

Background: Progressive mucinous histiocytosis (PMH) is a benign, non-Langerhans cell histiocytosis with characteristic ultrastructural features that can be used for diagnosis. Once an important tool in dermatologic diagnosis, electron microscopy has been largely replaced by immunohistochemistry and immunofluorescence techniques today. However, electron microscopy occasionally still plays a crucial role in the diagnosis of dermatologic conditions. We report a case of PMH as an example of a dermatologic disorder that requires electron microscopy for its diagnosis. Methods: A 60-year-old woman presented to our clinic with a history of small, sharply demarcated, skin-colored papules ranging from 2 to 5 mm in diameter distributed over the arms, forearms, and dorsal hands. The results of light microscopy, immunohistochemical studies, and clinical examination were inconclusive. Another biopsy for electron microscopy showed the characteristic features of PMH. Conclusion: This case demonstrates that a dermatopathology service still needs to have access to electron microscopy for diagnostic purposes to successfully diagnose a small number of rare conditions.

scholarly journals Premature Eruption Of Tooth: An Unusual Oral Manifestation In A Case Of Multisystem Langerhans Cell Histiocytosis

2018 ◽  
Vol 3 (3) ◽  
pp. S16-S17
Author(s):  
M. Madhuchandan ◽  
J. MuruguSarasu ◽  
Dewan Pooja ◽  
Gomber Sunil ◽  
Mahajan Supriya
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestation

scholarly journals Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature

2019 ◽  
Vol 11 ◽  
pp. 175883591987801 ◽  
Author(s):  
Norbert Neckel ◽  
Andrej Lissat ◽  
Arendt von Stackelberg ◽  
Nadine Thieme ◽  
Mohemed-Salim Doueiri ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Young Patients ◽  
Specific Treatment ◽  
Langerhans Cell ◽  
Adequate Treatment ◽  
Oral Manifestation ◽  
Stomatognathic System ◽  
Proper Diagnosis ◽  
Specific Agent

Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We report on a rare case of its primary oral manifestation that was treated successfully with the BRAF-specific agent, vemurafenib, after insufficient standard LCH treatment. This case underlines the importance of proper diagnosis and the evaluation of targeted therapy as a valuable tool in LCH treatment. Furthermore, the close collaboration of surgeons, oncologists, and dentists is mandatory to ensure adequate treatment, restore the stomatognathic system in debilitating post-treatment situations, improve quality of life, and ensure effective disease control in infants and young patients.

The Frequency and Natural History of Diabetes Insipidus in Children with Langerhans-Cell Histiocytosis

1989 ◽  
Vol 321 (17) ◽  
pp. 1157-1162 ◽  
Author(s):  
David B. Dunger ◽  
Valerie Broadbent ◽  
Elizabeth Yeoman ◽  
Jonathan R. Seckl ◽  
Stafford L. Lightman ◽  
...  
Keyword(s):  
Natural History ◽  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
History Of

scholarly journals SURG-34. MULTIDISCIPLINARY MANAGEMENT OF A PATIENT WITH OPTIC PATHWAY-HYPOTHALAMIC LANGERHANS CELL HISTIOCYTOSIS: A CASE REPORT

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii210-ii210
Author(s):  
John Emmanuel Custodio ◽  
Kevin Paul Ferraris ◽  
Joseph Erroll Navarro ◽  
Kenny Seng ◽  
Jose Carlos Alcazaren ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
S100 Protein ◽  
Visual Fields ◽  
Langerhans Cell ◽  
Normal Body Mass Index ◽  
Team Approach ◽  
Hypothalamic Region ◽  
Suprasellar Region ◽  
Increased Risk ◽  
History Of

Abstract Langerhans cell histiocytosis (LCH) of the Central Nervous System (CNS) is rare. Isolated involvement of the hypothalamic region is much more extremely rare with only 0.04 to 0.6% of the cases. We report a case of a 33 year-old female who presented with a one-year history of amenorrhea and a five-month history of intermittent headache, memory lapse, and somnolence. The patient was of normal body mass index with normal visual acuity and intact visual fields. Laboratory examinations revealed panhypopituitarism with central diabetes insipidus. Cranial magnetic resonance imaging showed a large lobulated mass measuring 1.9 x 2.2 x 2 cm in the suprasellar region which extended to the pituitary infundibulum, hypothalamus and retrochiasmatic region, with surrounding edema. The patient underwent right orbitozygomatic craniotomy and subtotal excision of the mass through subfrontal and transsylvian approaches. Histopathological examination of langerhans cells were observed with positive immunohistochemical stain for CD1a and S100 protein antigen markers establishing a diagnosis of CNS LCH. Thoracoabdominal computed tomography scan and bone scan were done postoperatively and showed no evidence of extracranial lesions. The patient had been receiving prednisone and vinblastine based chemotherapy regimen. She remains to be asymptomatic and on close surveillance. To date, there is no standardized treatment strategy for CNS LCH in the adult population. An accurate histopathologic diagnosis and a specialized multidisciplinary team approach especially involving Oncology, Neurosurgery, Ophthalmology and Endocrinology are critical to optimally tailor possible effective treatment options for patients with this similar disease. Long-term follow-up is crucial due to the increased risk of local recurrence and multisystemic involvement.

scholarly journals Rare Langerhans cell histiocytosis in children: a case report

2019 ◽  
Vol 7 (4) ◽  
pp. 1367
Author(s):  
Lina Fadil ◽  
Emtenan Almajid
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Plasma Cells ◽  
Oral Lesion ◽  
Langerhans Cell ◽  
Tissue Destruction ◽  
Total Period ◽  
Oral Manifestation ◽  
Initial Symptoms ◽  
Good General Condition

Langerhans cell histiocytosis (LCH) is a rare disease, formally known as histiocytosis X that is characterized by abnormal proliferation of histiocytes derived from bone marrow (Langerhans cells), joined with leucocytes, eosinophils, neutrophils, lymphocytes, plasma cells and giant multi-nucleated cells causing tissue destruction. One of the first signs of LCH is oral manifestation, in some cases, the oral cavity may be the only affected area. With the chance of oral lesion incidence in LCH being 77%.Initial symptoms are generally nonspecific, which can easily cause misdiagnoses.The purpose of reporting this case is to discuss the features of LCH clinically and radiographically and in the role of the dentist when diagnosing such lesions for a proper management.An 11-year-old boy reported a complaint of swelling in the left side of the lower jaw that is asymptomatic and had been gradually increasing in size for the past 6 months without any improvements. After preforming a biopsy and diagnosing the lesion as LCH, the patient was then treated with a dose of vinblastine (6 mg/m2 intravenous bolus) for 24 weeks as a total period. Two years follow up; the patient showed no sign of recurrence and is in good general condition. In conclusion, reporting this case serves as documentation of the proper route of clinical assessment and diagnosis of LCH with the best possible treatment as guidance.

scholarly journals Pulmonary Langerhans cell histiocytosis with inguinal abscess: Unusual presentation

2020 ◽  
Vol 29 (1) ◽  
pp. 64-66
Author(s):  
Kamarul Naim Mohd Hirmizi ◽  
Nurul Yaqeen Mohd Esa ◽  
Mardiana Abdul Aziz ◽  
Nor Salmah Bakar ◽  
Mohammad Hanafiah
Keyword(s):  
Lymph Node ◽  
Langerhans Cell Histiocytosis ◽  
Respiratory Symptoms ◽  
Inguinal Lymph Node ◽  
Langerhans Cell ◽  
Histopathological Analysis ◽  
Intermittent Fever ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
History Of

We report a case of a 30-year-old man who was treated as recurrent right inguinal abscess following a 2-month history of right inguinal swelling and intermittent fever with no respiratory symptoms. Resection of his right inguinal lymph node and the histopathological analysis revealed the diagnosis of Langerhans cell histiocytosis. In addition, the CT of the thorax showed presence of bilateral cystic lung changes consistent with pulmonary Langerhans cell histiocytosis.

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