A Case of Orbital Langerhans Cell Histiocytosis in an Adult.

We describe a rare case of orbital Langerhans cell histiocytosis in a 39-year-old female patient who presented right orbital pain and edema of the upper right eyelid. Surgery showed a friable lesion and underlying bone irregularity. Morphological aspects and immunohistochemical profile favored the diagnosis of Langerhans cell histiocytosis, which was confirmed with evidence of Langerin expression. The staging tests did not reveal any organ involvement, so we decided to follow the algorithm proposed by Euro Histio Net: in case of unifocal disease and in a single organ, clinical surveillance was preferred. This case aims to raise awareness of a manifestation of Langerhans cell histiocytosis, which should always be considered as a differential diagnosis in adults with osteolytic orbital lesions. PMID: 31493367 [PubMed - in process]
Source: Acta Medica Portuguesa - Category: General Medicine Tags: Acta Med Port Source Type: research

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Abstract This is a case of a pediatric male who presented with a postauricular mass and pain. Computed tomography and magnetic resonance imaging suggested a diagnosis of Langerhans cell histiocytosis. Characteristic imaging findings and initial workup for Langerhans cell histiocytosis are described. PMID: 31558057 [PubMed - as supplied by publisher]
Source: Ear, Nose and Throat Journal - Category: ENT & OMF Authors: Tags: Ear Nose Throat J Source Type: research
We report a rare case of mandibular condyle unifocal HL mimicking a temporomandibular joint disorder. CASE REPORT: A 44-year-old patient presented with a left temporomandibular disorder with painful left preauricular swelling. The imaging assessment found a bone lesion of the left mandibular condyle. A curettage with biopsy was used to diagnose HL. Six months later, the patient had no more pain. DISCUSSION: The craniofacial clinical expressions of HL mainly concern the bones, which can cause: pain, swelling, fracture, compression of noble organs. The other sites are: oral cavity, skin, lymph nodes, or eyes. Isolate...
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
Differential diagnosis of Langerhans Cell Histiocytosis (LCH) in the jaw is essential for early treatment including systemic therapy. Records of 17 patients (6 men and 11 women; mean age, 14 years) with histologically confirmed LCH were reviewed. All the lesions occurred in the mandible. Most of the cases (n=12) were intraosseous type LCH, only 5 patients had alveolar type LCH. Patients complained of facial swelling and pain most likely. In the 14 patients who underwent CT and/or MR imaging, all LCH lesions were osteolytic, with a mean size of 23 mm. LCH presented as expansile lesions with periosteal new bone formation, ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
This article presents the case of a 9-year old patient who came in for treatment with facial swelling, pain, tooth mobility and intraoral ulcer in the vestibular sulcus of the right mandibular angle region. Imaging and incisional biopsy were obtained. After the histopathological examination confirmed the diagnosis of eosinophilic granuloma, the lesion was removed by curettage and a reconstruction titanium plate was inserted to avoid a pathological fracture. After 14 years of follow-up no recurrence was found and mandibular growth was not impaired. A discussion about this type of tumor is presented, based on the current lit...
Source: RGO - Revista Gaucha de Odontologia - Category: Dentistry Source Type: research
CONCLUSION: The most common presentation in ECD is periorbital XLL. Other presentations include non-facial cutaneous xanthomas, panniculitis-like lesions, and granuloma annulare-like lesions. Associated symptoms at presentation include bone/extremity pain and weight loss. This article is protected by copyright. All rights reserved. PMID: 31120137 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
Rationale: Langerhans cell histiocytosis (LCH) involving adult cervical vertebrae is relatively rare clinically. Patient concerns: An 18-year-old male patient exhibited a 1-month history of neck pain, restricted neck mobility, and numbness and weakness of both upper limbs. The patient reported no pain at other sites, exhibited no fever or night sweats, and was unable to recall any recent injury. Diagnoses: On the basis of the radiological features of the lesion and laboratory tests, there was a high possibility that the patient had a tuberculosis lesion. Postoperative GeneXpert and Mycobacterium tuberculosis (MTB) ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Hairy cell leukemia (HCL) is an uncommon but distinct form of mature B-cell neoplasm that originates from activated late B-cells. It represents only 2% of all adult lymphoid leukemia; patients are predominantly middle-aged to elderly males with a median age of 50 years and is characterized by pancytopenia, monocytopenia and usually associated with massive splenomegaly.HCL associated with BRAF mutation 100% of cases, it's associated with hematological and oncological malignancies such as melanoma and papillary thyroid cancer with positive BRAF in 40 % of cases.Although the association of both cancers (HCL &papillary thy...
Source: Blood - Category: Hematology Authors: Tags: 603. Oncogenes and Tumor Suppressors Source Type: research
Conclusions: Cladribine therapy may be effective in patient with progressive PLCH disease in children.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric bronchology Source Type: research
In this report, the reconstruction of an osseous defect with an iliac graft and a modified implant ‐supported hybrid prosthesis after surgical excision of an eosinophilic granuloma in the mandible of a 27‐year‐old male patient was described. The patient was satisfied with the functional and esthetic results of the implant‐supported restoration and a 6‐year follow‐up showed no sign of recurrence.
Source: Special Care in Dentistry - Category: Dentistry Authors: Tags: CASE HISTORY REPORT Source Type: research
Rationale: Langerhans’ cell histiocytosis (LCH), also called histiocytosis X, is an uncommon disorder manifesting in a variety of ways. Although LCH can involve various organs including bone, skin, and lymph nodes, multisystem involvement of LCH is rare in adults. Patient concerns: A 31-year-old woman first presented to our hospital with left leg pain. She had a history of a 20-kg weight gain over three months. Diagnoses: X-ray, magnetic resonance imaging (MRI), computed tomography (CT), and bone scan images revealed enhancing lesions in the left femur and right temporal bone, multiple cystic lesions in the l...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
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