Langerhans cell histiocytosis presented as bilateral otitis media with effusion, a rare case report.

Background: Langerhans cell histiocytosis (LCH) or histiocytosis X is considered as a rare disease that may have effect on multiple organs. The initial presentation of LCH is varied. The signs and symptoms of otologic histiocytosis can be the same as the acute or chronic infectious ear diseases. Definitive diagnosis of LCH is confirmed by biopsy and immunohistochemically staining of S-100 protein and CD1a antigen. Chemotherapy is the main mode of treatment. Case presentation: In this report, we explained the clinical manifestation, diagnosis and treatment of a case of 15 month-old girl with diagnosed of LCH that initially presented with otitis media with effusion (OME). Conclusion: LCH is a rare disease that presented with variable sign and symptoms and have an effect on multiple organs. LCH should be regarded in cases with recurrent ear infection without response to medical treatments. Moreover, biopsy with IHC is the gold standard of diagnosis and chemotherapy is the main form of treatment.

Erdheim-Chester Disease of the Breast: First Review and First Case of Isolated Severe Gynecomastia.

(1) Introduction: Erdheim-Chester disease (ECD) is a life-threatening condition and often a diagnostic challenge. It has recently been classified as a hematopoietic tumour, and the cases of ECD reported in the literature has dramatically increased during the last 15 years. (2) Methods: We describe the case of a 57-year-old male patient with severe gynecomastia, with a detailed description of his diagnostic iter and consequent surgical operation. We provide the first systematic review of the literature of breast involvement in ECD, following PRISMA guidelines, including 13 studies and 16 patients. (3) Results: Our report resulted to be the first case of gynecomastia as a single clinical and imaging feature of ECD described in English literature. A total of 81.3% of patients included were female. Among them, 76.9% had unilateral and nodular presentation, while male patients presented bilateral heterogeneous breast enlargement. Globally, 87.5% expressed breast alterations as their first manifestations of ECD. Only 50% presented skeletal involvement. (4) Conclusion: The reported case represents a unique addition to the literature. We found two different patterns in ECD-related breast involvement between male and female patients, an unusual M/F ratio, and a lower rate of bone involvement. Breast involvement is frequently the first clinical feature; therefore, breast caregivers should be aware of this dangerous and most likely underestimated condition.

Spontaneous pneumothorax with pulmonary Langerhans cell histiocytosis (PLCH) in an adult heavy cigarette smoker-A case report.

Pulmonary Langerhans cell histiocytosis is a rare disease caused by the proliferation of CD1a-positive histiocyte-like cells infiltrating the lung's interstitial layer. Most cases affect young to middle-aged persons, especially adult heavy cigarette smokers. A 49-year-old male heavy smoker (40 pack-year), with non-productive cough, dyspnoea and desaturation, presented with a right-sided pneumothorax on chest x-ray with total atelectasis. Chest computed tomography (CT) revealed bilateral multiple thick-walled infiltrated cysts and multiple ground-glass nodules throughout the entire lung. Surgery with minimal invasive thoracoscopic lung biopsy and pleurodesis was performed. Pathology showed histiocyte-like cells aggregates in the pulmonary parenchyma. Immunohistochemical stain demonstrated CD1a(+), S100(+) and CD68(+). After 3 months of smoking cessation, clear improvement was evidenced with a chest CT showing bilateral multiple thin-walled rounded cysts and multiple ground-glass nodules that are smaller in size and decreased in numbers. Early minimal invasive thoracoscopic lung biopsy and pleurodesis can also be a choice if the development of secondary spontaneous pneumothorax occurs.

Langerhans cell histiocytosis in the oral and maxillofacial region: An update.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation and infiltration of histiocytic cells. METHODS: This review focused on the main aspects associated with LCH. RESULTS: LCH can involve single or multiple organs and systems, with bone and skin being the most commonly affected sites. Regarding skeletal manifestations, the jawbones are involved in about 20%-30% of all cases. Such lesions may present as unilocular or multilocular images mainly affecting the posterior mandible. Oral soft tissue lesions may also occur, with the gingiva and hard palate being the most frequently affected sites. CONCLUSION: The diagnosis and management of LCH are challenging, requiring a multidisciplinary approach, with dentists playing a central role since oral manifestations can be the first sign of the condition.

A possible case of Langerhans-cell histiocytosis? Differential diagnosis in a rare case from the Late Antiquity Bavaria (Germany).

OBJECTIVE: To outline the importance of accurate diagnosis in ancient rare diseases by presenting a possible case of Langerhans-cell histiocytosis. MATERIALS: Skeletal elements from a well-preserved skeleton of a nine to eleven-year-old, probably female child who lived around 300-400 AD Late Roman Neuburg / Donau (Germany). METHODS: Macroscopic, radiologic, light and scanning-electron microscopic and physical techniques were used. RESULTS: Resorptive defects, particularly in the cranium, but also in the left hip bone and the right femur, suggest the presence of Langerhans-cell histiocytosis macroscopically and radiologically. The presence of morphological changes along the edges of osteolytic lesions and in the diploic spaces appear to be post-mortem artifacts based on microscopic investigation and elemental analysis. CONCLUSIONS: Re-evaluation of morphological structures and elemental constitution of lesions is critical to differential diagnosis. In the case examined here, the identification of post-mortem structures rules out the former diagnosis of Langerhans-cell histiocytosis. Re-evaluation of cases of rare diseases require applying a range of methods during the analysis, as every single case makes a difference in the numbers of this very small group of diseases. SIGNIFICANCE: This study emphasizes the importance of utilizing different analytical techniques to avoid false diagnoses. LIMITATIONS: Not all morphological features can reliably be diagnosed using microscopic and elemental techniques. SUGGESTIONS FOR FURTHER RESEARCH: In the case of rare diseases that are difficult to diagnose, the widest possible spectrum of techniques should always be used, particularly microscopy.

Isolated Cutaneous Langerhans Cell Histiocytosis Presenting in an Adult Male.

Langerhans cell histiocytosis (LCH) is an infrequent clonal proliferative disorder of myeloid dendritic cells. It has a wide variety of cutaneous manifestations and retains the possibility of systemic implications. Because LCH is predominantly a disease of childhood, there are well-established clinical definitions, as well as guidelines regarding workup and treatment, in the context of pediatric disease. Here we present a case of isolated cutaneous LCH in an adult male, followed by a discussion of our diagnostic plan and treatment course. The patient exhibited a small, excoriated, yellow papule on his inferior forehead during a skin examination. The specimen underwent tangential shave biopsy; histopathologic evaluation with appropriate immunohistochemical staining confirmed a diagnosis of cutaneous LCH. After thorough investigation via serologic and imaging diagnostics, we confirmed isolated cutaneous disease. The patient underwent wide local excision (WLE) with no evidence of recurrence. It is crucial to appropriately screen all patients diagnosed with cutaneous LCH for internal organ involvement. The authors aim to highlight the need for further investigations to ultimately dictate standardized management and treatment for isolated cutaneous LCH in the adult population.

Solitary congenital Langerhans cell histiocytoma: A pattern of benign, spontaneous regression in patients with single lesion disease.

Langerhans cell histiocytosis (LCH) is the neoplastic proliferation of dendritic langerin-positive histiocytes manifesting as either single system unifocal, single system multifocal, or multisystem disease. The designation Hashimoto-Pritzker, or self-healing LCH, has fallen out of favor since it is impossible to predict at time of diagnosis whether the disease is truly self-remitting or capable of spreading to other organ systems. We review the English literature on solitary congenital Langerhans cell histiocytoma, draw novel conclusions from the data provided by 81 cases in the literature, and illustrate a typical presentation of the diagnosis with a previously unreported patient. Each of the patients diagnosed with solitary congenital histiocytoma experienced spontaneous resolution and had no signs of systemic disease at latest follow-up. Furthermore, we offer an analysis of the histopathological findings available from the 81 cases and our patient. Based on our study observations, we propose solitary congenital Langerhans cell histiocytoma may portend a good prognosis and represent a distinct entity. However, until further confirmation with prospective studies, we recommend clinicians continue conducting appropriate workup to rule out systemic involvement.

Langerhans Cell Histiocytosis Presenting as Progressively Worsening Neck Pain: A Case Report With a Review of Literature.

A 29-year-old female with past medical history of chronic serous otitis media presented with worsening neck stiffness and pain over a period of 2 weeks. The patient described non-specific symptoms that were localized to the right side of her neck. She presented to the hospital only when the pain was so extreme that it limited her range of motion. The differential for acute neck pain without fever, chills or any inciting trauma is vast. They include medical emergencies such as meningitis, acute coronary syndromes and extend to rheumatologic diseases or simply musculoskeletal strain. On review of systems, she denied dizziness, headache, vision changes, dysphagia, or other facial pain. Based on the severity of her pain, she underwent a Computed Tomography scan of the neck, which was concerning for erosive calavarial lesions. Further imaging revealed multiple lytic foci and erosions from the right maxillary sinus to the right mandible to the C1 vertebra. Following requisite surgical intervention, she was found to have Langerhans cell histiocytosis, a rare disease of myeloid cells, usually affecting pediatric populations. Little is known about the adult manifestations of Langerhans Cell Histiocytosis. This review contributes to broadening the literature on this topic which can present with complaints as typical as neck pain.

Infiltrating the Heart and Kidney: A Rare Pediatric Case of Multisystem Langerhans Cell Histiocytosis from Pakistan.

Langerhans cell histiocytosis (LCH) is a rare, clonal disease of the monocyte-macrophage system, varying in its clinical presentation from mere self-healing skin and bone lesions to life-threatening multi-system disease. In descending order of frequency, the disease is known to involve the skeleton, skin, lymph nodes and lesser often, the liver, spleen, lungs, hematopoietic and central nervous systems. Here, we present a pediatric case of multi-system LCH in a five-year-old child, unique in its evident cardiac and renal involvement alongside other organ systems and important in how the diagnosis was aided by a fine needle aspiration cytology instead of the costlier histopathological procedures, in a setting with limited resources.

Langerhans cell histiocytosis of the orbit and frontal sinus of the adult woman: A first case report in Poland.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a term describing a clonal proliferation of pathologic Langerhans cells (histiocytes), which may manifest as unisystem (unifocal or multifocal) or multisystem disease. LCH is a rare cause of the orbital tumor with the predilection to its lateral wall which is particularly common in children. CASE DESCRIPTION: We report an unusual case of a 33-year-old woman, 6 months after childbirth, who presented with the edema of the right orbit and upper eyelid with headaches. On physical examination, the patient had a right superior and lateral swelling of the eyelid and the orbit and right enophthalmos, without blurred vision. Magnetic resonance imaging showed well-defined, expansile, intensely homogeneously enhancing mass lesion in the right superolateral orbital rim with the destruction of the upper wall of the orbit, growing into the frontal sinus and frontal part of the cranium with the bold of the dura mater in this region. Radical excision of the tumor was achieved through a right fronto-temporo-orbito-zygomatic craniotomy. Histopathological examination had confirmed the diagnosis of the LCH. The patient was discharged home with a modified Rankin Scale score of 0. CONCLUSION: The main purpose of this case report is that LCH should be considered as one of the possible causes of quickly appearing tumor of the orbit in adults.

Langerhans Cell Histiocytosis in an Adult with Oral Cavity Involvement: Posing a Diagnostic Challenge.

Langerhans cell histiocytosis (LCH) is a rare hematological disorder characterized by local or generalized, uncontrolled proliferation and infiltration of Langerhans type of histiocytic cells. It represents a spectrum of clinicopathologic disorders, ranging from a highly aggressive and frequently fatal multisystem disease to an easily cured solitary lesion of bone. Involvement of children and the younger age group is more common than the adults. Oral cavity involvement occurs early in LCH, but the initial symptoms are generally nonspecific, often causing misdiagnosis. This report describes a rare case of chronic localized LCH in an adult patient, with involvement of oral cavity. A 34-year-old male patient presented with multiple nodulo-papular, ulcerated lesions in gingiva involving both the jaws (primarily mandible) and the left buccal mucosa, in addition to regional teeth mobility. The most striking feature was that even after extraction of mobile teeth, the lesions persisted. After recording proper history, performing clinical and radiological evaluation, an incisional biopsy was performed followed by histopathology and immunohistochemistry to reach a confirmatory diagnosis of LCH, thereby implementing early and appropriate initiation of treatment.

Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report.

BACKGROUND: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects - by order of decreasing frequency - the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults. CASE PRESENTATION: We describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen. CONCLUSIONS: Gastrointestinal tract lesions are a rare manifestation of Langerhans cell histiocytosis, especially when associated with extraintestinal involvement, such as the lungs. Chemotherapy protocols have not been well established for the treatment of the disease. The clinical impact of the effective chemotherapy regimen used to treat this uncommon presentation of Langerhans cell histiocytosis will be viewed in this case report.

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.

A rare 'incidentaloma' found on low-dose CT screening for lung cancer: 'scanner beware'.

Screening for lung cancer with low-dose computed tomography (LDCT) has been shown to reduce mortality and has been recommended by the U.S. Preventive Services Task Force for adults 55 to 80 years of age with a 30 pack-year smoking history who are either current smokers or those that quit within 15 years. However, the overwhelming majority of abnormalities detected are not from malignancy. We report a case of pulmonary Langerhans' cell histiocytosis, here-to-fore thought of as extremely uncommon, and make readers aware that this may be increasingly found as LDCT is more widely adopted.

Langerhans Cell Histiocytosis - A Challenge for the Dental Professional.

Langerhans cell histiocytosis (LCH) is a group of rare disorders histologically characterized by the proliferation of LC, involving multiple organs and systems. Typically, there is bone involvement and, less frequently, lesions may be found in the lungs, liver, lymph nodes, skin, and mucosae. Oral soft tissue lesions without bone involvement are rare. Antigenic markers that react with CD1a glycoprotein, cytoplasmatic protein S100 detected by immunoperoxidase staining, and/or presence of Birbeck granules on electron microscopic examination are required for a definitive diagnosis of LCH. In this article, we report a case of LCH, which had presented with multiple oral lesions without any other systemic signs and symptoms. Management of such children with periodontal manifestations should include hematological and, if possible, immunological investigations at an early stage. Careful clinical examination, good diagnostic skill, and awareness of characteristic cytological features of LCH can lead to earlier diagnosis and treatment with minimal deformity.

Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids.

Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy.

Histiocytosis X and Pericarditis - A Rare Association and a Difficult Diagnosis.

Histiocytic disorders are a group of rare diseases with systemic involvement and with multiple clinical manifestations. We present the case of a 51 years old patient investigated for dyspnea with orthopnea, dry cough, asthenia, muscular weakness and ataxia. The association of previous symptoms with skin lesions, diabetes insipidus, partial hypophyseal insufficiency and pericarditis induced many diagnostic debates. The diagnosis is Histiocytosis X must be sustained by tissue biopsy with immunohistochemical assay or genetic testing. The particularity of our patient is the presence of pericarditis, rarely associated with histiocytosis. Collaboration between medical specialties is mandatory in order to treat this disorder.

Excessive pediatric fasciitis necrotisans due to Pseudomonas aeruginosa infection successfully treated with negative pressure wound therapy.

The case of a 10-year old female child is described with a history of myeloproliferative disorder having skin, bone and visceral involvement. Bone marrow biopsy revealed histiocytosis X. During chemotherapy necrotizing fasciitis of the lower abdominal wall was diagnosed. Multiple microbiological cultures taken from the wound base revealed Pseudomonas aeruginosa infection. Surgical necrectomy and application of negative pressure wound therapy (NPWT) was started together with intensive care treatment for sepsis. As both wound and general condition of the patient improved, autologous split thickness skin grafting was carried out in two sitting under continuing NPWT application. The applied skin grafts showed excellent take, the perilesional subcutaneous recesses resolved and complete healing was achieved after 28 days of NPWT treatment. Proper dermatological diagnosis and immediate escharectomy complemented with application of NPWT can be life-saving in the treatment of necrotizing fasciitis.