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    1 OF 213

    Nodular macroregenerative tissue as a pattern of regeneration in cholangiopathic disorders.
    Pediatr Radiol 2018 Apr 19. Epub 2018 Apr 19.
    Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., MLC 5031, Cincinnati, OH, 45229, USA.
    Background: Published case series have described central hepatic macroregenerative nodules or masses as a common feature of Alagille syndrome. Our experience suggests this regenerative pattern can be seen more generally in cholangiopathic disorders.

    Objective: To define the frequency of central regenerative tissue in Alagille syndrome and other cholangiopathic disorders and to describe the typical appearance of such regenerative tissue. Read More

    [BRAF-V600E mutation and its clinical significance in children with Langerhans cell histiocytosis].
    Zhongguo Dang Dai Er Ke Za Zhi 2018 Apr;20(4):290-294
    Department of Pediatrics, West China Second University Hospital, Sichuan University/Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610041, China.
    Objective: To investigate the clinical significance of BRAF-V600E mutation in children with Langerhans cell histiocytosis (LCH).

    Methods: Real-time fluorescence quantitative PCR was used to detect BRAF-V600E mutation in paraffin-embedded tissue samples from 26 children with LCH. A retrospective analysis was performed for the association of BRAF-V600E mutation with clinical features and prognosis of children with LCH. Read More

    Concurrent Mastoid Cellulitis and Langerhans Cells Histiocytosis: 
A Challenging Diagnosis.
    Oman Med J 2018 Mar;33(2):167-170
    Department of Otorhinolaryngology, Tengku Ampuan Rahimah Hospital, Klang, Selangor, Malaysia.
    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder, which commonly arises in the bone and may involve other systems. To date, the diagnosis of temporal bone LCH remains a challenge as it may masquerade as a common ear infection. We report a case of a child who presented to us with persistent bilateral ear discharge for four months and was not responding to treatment. Read More

    Frequency of MAP2K1, TP53, and U2AF1 Mutations in BRAF-mutated Langerhans Cell Histiocytosis: Further Characterizing the Genomic Landscape of LCH.
    Am J Surg Pathol 2018 Apr 11. Epub 2018 Apr 11.
    Department of Pathology, University of Chicago, Chicago, IL.
    Langerhans cell histiocytosis is a proliferative disorder of neoplastic Langerhans cells with activating mutations in the Erk signaling pathway. TP53 and U2AF1 mutations have been implicated in other myelomonocytic malignancies and we hypothesized that mutations in these genes may cosegregate in LCH patients according to BRAF mutation status. Towards this end, we collected cases with a pathologic diagnosis of Langerhans cell histiocytosis from Stanford University Hospital. Read More

    A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis.
    Arch Soc Esp Oftalmol 2018 Apr 8. Epub 2018 Apr 8.
    Unidad de Órbita y Oculoplástica, Servicio de Oftalmología, Hospital Universitario de Gerona Dr. Josep Trueta, Gerona, España.
    Clinical Case: A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Read More

    Dermoscopy in the diagnosis of juvenile xanthogranuloma.
    An Bras Dermatol 2018 Jan-Feb;93(1):138-140
    Dermatology Department at Hospital Santa Casa de Curitiba - Curitiba (PR), Brazil.
    Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. Read More

    Oral Juvenile Xanthogranuloma in a child: clinical, histological, and immunohistochemical profile of a rare entity.
    J Cutan Pathol 2018 Apr 6. Epub 2018 Apr 6.
    Oral Pathology Section. Universidad Autónoma de Guadalajara (UAG), México.
    Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG have been reported, but is rare. Read More

    CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.
    Cancer 2018 Apr 6. Epub 2018 Apr 6.
    Texas Children's Cancer Center, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
    Background: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH.

    Methods: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Read More

    Craniofacial Manifestations of Systemic Disorders: CT and MR Imaging Findings and Imaging Approach.
    Radiographics 2018 Apr 6:170145. Epub 2018 Apr 6.
    From the Departments of Radiology (V.C.A.A., M.N.C., H.K., A.F., O.S.), Otolaryngology-Head and Neck Surgery (O.S.), and Radiation Oncology (O.S.), Boston University Medical Center, Boston University School of Medicine, 820 Harrison Ave, 3rd Floor, Boston, MA 02118.
    Many systemic diseases or conditions can affect the maxillofacial bones; however, they are often overlooked or incidentally found at routine brain or head and neck imaging performed for other reasons. Early identification of some conditions may significantly affect patient care and alter outcomes. Early recognition of nonneoplastic hematologic disorders, such as thalassemia and sickle cell disease, may help initiate earlier treatment and prevent serious complications. Read More

    Langerhans cell histiocytosis with hemorrhagic uveitis and exudative retinal detachment.
    Int Med Case Rep J 2018 26;11:65-68. Epub 2018 Mar 26.
    Fellow of Vitreo-Retina Surgery, Lumbini Eye Institute, Siddharthanagar, Nepal.
    Bilateral intraocular involvement in Langerhans cell histiocytosis (LCH) is uncommon. A 15-year-old boy presented with painless decreased vision in right and painful left red eye of 2 weeks duration. Visual acuity was 20/500 and 20/200, respectively. Read More

    Cutaneous Hematolymphoid and Histiocytic Proliferations in Children.
    Pediatr Dev Pathol 2018 Mar-Apr;21(2):208-251
    2 Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children's Hospital and Dermatopathology, Washington University Medical Center, St. Louis, Missouri.
    This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Read More

    Pulmonary hypertension in patients with interstitial lung disease.
    Pulm Pharmacol Ther 2018 Mar 29. Epub 2018 Mar 29.
    Section of Pulmonary, Critical Care & Sleep Medicine, Department of Internal Medicine, Yale School of Medicine, New Haven, CT, USA. Electronic address:
    Interstitial lung diseases (ILDs) comprise a broad and heterogeneous group of more than two hundred diseases with common functional characteristics. Their diagnosis and management require a multidisciplinary approach. This multidisciplinary approach involves the assessment of comorbid conditions including pulmonary hypertension (PH) that exerts a dramatic impact on survival. Read More

    An autopsy case report: Differences in radiological images correlate with histology in Erdheim-Chester disease.
    Pathol Int 2018 Mar 30. Epub 2018 Mar 30.
    Department of Pathology and Biological Responses, Nagoya University Graduate School of Medicine.
    p16 activation caused by oncogenic mutations may represent oncogene-induced senescence (OIS), a protective mechanism against oncogenic events. However, OIS can contribute to tumor development via tissue remodeling in some tumors. Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, is one such tumor. Read More

    Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment.
    Postepy Dermatol Alergol 2018 Feb 20;35(1):6-17. Epub 2018 Feb 20.
    Department of Dermatology, Venereology, and Allergology, Medical University of Gdansk, Gdansk, Poland.
    Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Read More

    Intensification of induction therapy and prolongation of maintenance therapy did not improve the outcome of pediatric Langerhans cell histiocytosis with single-system multifocal bone lesions: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study.
    Int J Hematol 2018 Mar 28. Epub 2018 Mar 28.
    Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji, Japan.
    Langerhans cell histiocytosis (LCH) with single-system (SS) multifocal bone (MFB) lesions is rarely fatal, but patients may experience relapses and develop LCH-associated sequelae. To evaluate effect on outcomes of pediatric multifocal LCH, we tested a treatment protocol modified from the Japan Langerhans Cell Histiocytosis Study Group (JLSG)-96 study. We assessed the outcomes of all consecutive newly diagnosed pediatric patients with LCH with SS-MFB lesions who were treated with JLSG-02 protocol in 2002-2009. Read More

    Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases.
    World J Surg Oncol 2018 Mar 27;16(1):72. Epub 2018 Mar 27.
    Department of Radiology, Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Room 203, Building 5, No.1665 Kongjiang Road, Shanghai City, 200092, Yangpu District, China.
    Background: We aimed to evaluate the clinical and imaging presentations of Langerhans cell histiocytosis (LCH) in the pediatric temporal bone.

    Methods: This retrospective study included 27 pediatric cases with pathological confirmed LCH of the temporal bone. The clinical and imaging features of the cases were analyzed. Read More

    Necrobiotic xanthogranuloma: a 30-year single-center experience.
    Ann Hematol 2018 Mar 22. Epub 2018 Mar 22.
    Division of Hematology/Oncology, Mayo Clinic, 5777 E. Mayo Boulevard, Phoenix, AZ, 85054, USA.
    To characterize the clinical features, associated disorders, and treatment of necrobiotic xanthogranuloma (NXG), a rare non-Langerhans cell histiocytosis, we conducted a retrospective review of pathologically confirmed NXG at Mayo Clinic Arizona from 1987 to June 2017. Data on clinical findings, laboratory findings, associated disorders, therapy, and response to therapy were extracted. Nineteen patients were identified. Read More

    and Langerhans Cell Histiocytosis.
    Iran J Pathol 2017 1;12(4):323-328. Epub 2017 Nov 1.
    Pediatric Infections Research Center, Research Institute for Children Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
    Background And Objective: Langerhans cell histiocytosis (LCH) is a rare histiocytic proliferative disorder of unknown etiology and mainly affects young children. The histological feature is granuloma-like proliferation of langerhans-type dendritic cells. Although the possible role of viruses such as (, ), (), () types 1 and 2 and (CMV, ) is suggested in the pathogenesis of LCH by some investigators, its exact pathophysiology has not been cleared yet. Read More

    [Langerhans cell histiocytosis with orbital involvement in an infant: Case report].
    Arch Argent Pediatr 2018 Apr;116(2):e283-e287
    Hospital Nacional Edgardo Rebagliati Martins, Departamento de Anatomía Patológica.
    The Langerhans cell histiocytosis is a rare disease characterized by the clonal proliferation of CD1a + myeloid dendritic cells associated with a significant inflammatory component. The localized form of the disease is called eosinophilic granuloma. Bone involvement is common; in children, lytic lesions are most frequently found in the cranial dome being rare in the orbit. Read More

    Topical Imiquimod for the Treatment of Relapsed Cutaneous Langerhans Cell Histiocytosis after Chemotherapy in an Elderly Patient.
    Case Rep Dermatol Med 2018 3;2018:1680871. Epub 2018 Jan 3.
    Department of Pathology, Uji-Tokushukai Medical Center, Uji 611-0042, Japan.
    Diagnosis and treatment of Langerhans cell histiocytosis (LCH) in elderly patients are often difficult. We report here a 61-year-old female suffering from a refractory axillary ulcer for nearly a year, whose biopsy revealed LCH. It was also noted that the patient had other cutaneous papulovesicular eruptions of LCH as well as central diabetes insipidus. Read More

    [Langerhans cell sarcoma: a clinicopathologic analysis of four cases].
    Zhonghua Bing Li Xue Za Zhi 2018 Mar;47(3):163-167
    Department of Pathology, Fujian Provincial Hospital, Provincial Clinical Medical College of Fujian Medical University, Fuzhou 350001, China.
    To investigate the clinicopathological features, differential diagnosis, and genetic alteration of Langerhans cell sarcoma (LCS). Four cases of LCS were collected from Fujian Provincial Hospital and Fuzhou General Hospital of Nanjing Military Command of PLA from July 2013 to January 2017. Clinicopathological features and immunophenotype were retrospectively reviewed in four LCS cases combined with genetic mutation analysis of BRAF and ALK. Read More

    Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series.
    Pediatr Blood Cancer 2018 Mar 12. Epub 2018 Mar 12.
    Department of Dermatology, Hospital Universitari Arnau de Vilanova, Lleida, Spain.
    Langerhans cell histiocytosis (LCH) is a disorder characterized by accumulation of Langerhans-like cells in one or various organs. A correct staging work-up is essential since there are multiorgan presentations with a poor prognosis. We report three patients with LCH skin lesions mimicking molluscum contagiosum in association with both high and low risk organ involvement. Read More

    Molecular characterization of the histiocytoses: Neoplasia of dendritic cells and macrophages.
    Semin Cell Dev Biol 2018 Mar 16. Epub 2018 Mar 16.
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, United States; Human Oncology and Pathogenesis Program, Memorial Sloan Kettering Cancer Center, New York, NY, United States. Electronic address:
    The systemic histiocytoses encompass a clinically heterogeneous group of disorders leading to tissue damage secondary to the accumulation and infiltration of pathological cells thought to be derived from the dendritic or monocytic lineages with accompanying inflammation. For decades, whether or not the histiocytoses were inflammatory or neoplastic disorders was unclear, and their cellular origins have long been obscure and heavily debated. However, the rise of the molecular era led to the discovery of recurrent BRAF mutations in approximately 50% of patients with Langerhans cell and non-Langerhans cell histiocytoses, which provided the first convincing evidence that these are indeed histiocytic neoplasms. Read More

    Primary Langerhans Cell Histiocytosis of the Extrahepatic Bile Duct Occurring in an Adult Patient.
    Balkan Med J 2018 Mar 8. Epub 2018 Mar 8.
    Clinic of Pathology, Medstar Georgetown University Hospital, Washington, USA.
    Background: Langerhans cell histiocytosis is characterized by abnormal proliferation of neoplastic Langerhans cells. Langerhans cell histiocytosis commonly affects the pediatric population and presentation in adults remain a rare event. The presentation of langerhans cell histiocytosis is highly variable but skin, bone and lung involvement are very common. Read More

    Treatment of Langerhans cell histiocytosis with a modified risk-adapted protocol-experience from a tertiary cancer institute in India.
    Pediatr Blood Cancer 2018 Mar 7. Epub 2018 Mar 7.
    Pediatric Hematolymphoid Disease Management Group, Department of Medical Oncology, Tata Memorial Center, Mumbai, India.
    Background: Involvement of risk-organs (RO+) in Langerhans cell histiocytosis (LCH) and inadequate early response identifies patients at high risk for relapse and mortality requiring intensive salvage therapy including stem cell transplant, adding cost and toxicity. To mitigate this, we used a standard induction, augmented with metronomic etoposide, and prolonged maintenance-similarly augmented for RO+, and retrospectively analyzed its impact.

    Procedure: LCH patients from 2009 through 2014 were included. Read More

    Population-based incidence of conjunctival tumours in Olmsted County, Minnesota.
    Br J Ophthalmol 2018 Mar 6. Epub 2018 Mar 6.
    Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
    Aim: To determine population-based incidence of conjunctival tumours in Olmsted County, Minnesota.

    Methods: The Rochester Epidemiology Project medical record linkage system was used to identify patients with conjunctival tumours in Olmsted County (1 January 1980 to 31 December 2015). Records were reviewed for demographics, types of tumours, histopathology, treatment and clinical course. Read More

    Clinical features and treatment outcomes of Langerhans cell histiocytosis of the spine.
    Spine J 2018 Mar 2. Epub 2018 Mar 2.
    Orthopaedic Department, Peking University Third Hospital, Haidian District, Beijing, 100191, China. Electronic address:
    Background Context: Langerhans cell histiocytosis (LCH) of the spine is a relatively rare condition with unknown etiology. The diagnosis and treatment protocols for spine LCH remain controversial.

    Purpose: In this study, we evaluated the efficacy and safety of our proposed diagnosis and treatment protocol introduced in 2009. Read More

    Scabies Surrepticius: Scabies Masquerading as Pityriasis Rosea.
    Cureus 2017 Dec 19;9(12):e1961. Epub 2017 Dec 19.
    Department of Dermatology, University of California, San Diego.
    Scabies, a mite infestation caused by 'Sarcoptes scabiei', most commonly presents as pruritic linear burrows where the mite has invaded the skin. Scabies variant such as bullous, crusted, hidden, incognito, nodular and scalp-mimic the other conditions. In addition, atypical presentations of scabies can masquerade as dermatitis herpetiformis, ecchymosis, Langerhans cell histiocytosis, systemic lupus erythematosus, urticaria, and urticaria pigmentosa. Read More

    Langerhans Cell Histiocytosis - A Challenge for the Dental Professional.
    J Cutan Aesthet Surg 2017 Oct-Dec;10(4):215-218
    Department of Periodontics, JCD Dental College, Sirsa, Haryana, India.
    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. Read More

    Clinical implications of oncogenic mutations in pulmonary Langerhans cell histiocytosis.
    Curr Opin Pulm Med 2018 May;24(3):281-286
    Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
    Purpose Of Review: Langerhans cell histiocytosis (LCH) is a neoplasm of dendritic cells with a wide clinical spectrum. Localized pulmonary LCH occurs in young adults with a history of smoking and can either resolve spontaneously or lead to progressive decline in pulmonary function. Young children can also present with localized disease - frequently bone or skin - or with multifocal or multisystem disease. Read More

    Erdheim-Chester disease: description of two illustrative cases involving the lung.
    Histopathology 2018 Feb 22. Epub 2018 Feb 22.
    Pathology Unit, Ospedale di Terni, University of Perugia, Perugia, Italy.
    Aim: Erdheim-Chester disease represents a clonal systemic proliferation of histiocytes. Bone is the most common site of involvement, although almost any organ, including the lungs, can be affected.

    Methods And Results: The diagnosis of Erdheim-Chester disease can be difficult, owing to its rarity and protean presentation. Read More

    Indeterminate cell histiocytosis in a Chinese patient with progressive and extensive nodular lesions and mixed indeterminate cell and macrophage-monocyte lineage.
    J Cutan Pathol 2018 Feb 22. Epub 2018 Feb 22.
    Department of Dermatology, National Skin Centre, Singapore.
    Indeterminate cell histiocytosis (ICH) is an extremely rare cutaneous neoplastic disorder. It has the immunophenotypic features of both Langerhans and non-Langerhans cell histiocytosis. We report here a case of a healthy young Chinese woman who presented with disfiguring, thick, infiltrated cutaneous nodules on the face, trunk and extremities which appeared progressively over a period of 4 years. Read More

    The multiple faces of Langerhans cell histiocytosis in childhood: A gentle reminder.
    Mol Clin Oncol 2018 Mar 18;8(3):489-492. Epub 2017 Dec 18.
    Fourth Department of Pediatrics, Aristotle University of Thessaloniki, Papageorgiou General Hospital, 56403 Thessaloniki, Greece.
    Langerhans cell histiocytosis (LCH) is a rare hematologic disorder that results from the clonal multiplication and accumulation of immature dendritic Langerhans cells. Its reported incidence rate varies, but is considered to be 2.6-8. Read More

    A 12-Month-Old Healthy Girl with a New Oral Ulcer and Chronic Diaper Rash.
    Dermatopathology (Basel) 2017 Jan-Dec;4(1-4):24-30. Epub 2017 Nov 16.
    Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.
    A 12-month-old healthy girl presented with a chronic diaper rash. Physical examination demonstrated crusting of the scalp, erythematous papules with surrounding petechiae on the lower abdomen, and an intraoral palatal ulcer. Further imaging demonstrated bone involvement. Read More

    Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma.
    Rom J Ophthalmol 2017 Jul-Sep;61(3):229-236
    Opthalmology Clinic, "Sf. Spiridon" University Hospital, Iași, Romania.
    Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. Read More

    Congenital Langerhans cell histiocytosis presenting in a 27-week-gestation neonate.
    Pediatr Dermatol 2018 Mar 13;35(2):e140-e141. Epub 2018 Feb 13.
    Section of Dermatology, University of Chicago, Chicago, IL, USA.
    Langerhans cell histiocytosis is exceedingly rare in premature infants, and the few cases reported suggest a poor prognosis with systemic involvement. We present a case of Langerhans cell histiocytosis limited to a single cutaneous lesion, presenting in a 27-week-gestation infant, which is the youngest gestational age of reported Langerhans cell histiocytosis cases. The lesion showed spontaneous resolution by 41 weeks corrected gestational age, and systemic involvement was absent, demonstrating a mild course of skin-only Langerhans cell histiocytosis in a premature infant. Read More

    Solving a Mystery . . . 8 Years Later.
    J Investig Med High Impact Case Rep 2018 Jan-Dec;6:2324709617752962. Epub 2018 Jan 24.
    Mayo Clinic, Rochester, MN, USA.
    Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis with multisystem involvement and insidious symptoms. In this article, we describe an interesting case of Erdheim-Chester disease that was eventually diagnosed 8 years after symptoms initially started. Read More

    Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature.
    Leuk Res 2018 Apr 2;67:39-44. Epub 2018 Feb 2.
    Cancer Research Foundation of New York, USA. Electronic address:
    Background: There is mounting evidence that Langerhans cell histiocytosis (LCH) and acute myeloid leukemia (AML) are hematopoietic neoplasms that arise from the same myeloid precursor cell. In addition, studies suggest a relationship between LCH and primary idiopathic myelofibrosis (MF). Furthermore familial LCH, AML, and MF have each been reported. Read More

    Multidisciplinary approach in a case of Hand-Schüller-Christian disease with maxillary involvement.
    Spec Care Dentist 2018 Mar 8;38(2):107-111. Epub 2018 Feb 8.
    Department of Clinical and Experimental Medicine, University of Foggia, Foggia, Italy.
    Langerhans cell histiocytosis (LCH) is a childhood pathology with a peak of incidence ranging from 1 to 4 years of age, though diagnosis is often made in adult age. LCH is clinically classified into three types: eosinophilic granuloma, Hand-Schuller-Christian disease and Abt-Letterer-Siwe disease. We report a case of Hand-Schüller-Christian disease with diabetes insipidus, skull and maxillary involvement in a 16-year-old boy referred to our observation for gradual increase in mobility of the teeth and subsequent gradual loss of the second premolars and the first molars of the upper jaw. Read More

    Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement.
    Int J Radiat Oncol Biol Phys 2018 Mar 6;100(3):670-678. Epub 2017 Nov 6.
    Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, New York. Electronic address:
    Purpose: To characterize the efficacy and safety of radiation therapy in a contemporary Langerhans cell histiocytosis (LCH) cohort and to explore whether there are sites at higher risk for local recurrence.

    Patients And Methods: Between 1995 and 2015 we identified 39 consecutive LCH patients who were treated primarily with radiation therapy. Patients were staged by single/multisystem involvement and established risk organ criteria. Read More

    Langerhans cell histiocytosis as an unusual cause of back pain in a child: A case report and review of literature.
    J Craniovertebr Junction Spine 2017 Oct-Dec;8(4):384-386
    Department of Pediatrics, Division of Pediatric Hemato Oncology, Sri Ramachandra University, Chennai, Tamil Nadu, India.
    Low back pain in children and adolescents are usually attributed to mechanical causes and faulty positions. Although most of them are self-limiting, physicians should be aware of the red flag signs that warrant complete evaluation to rule out malignant causes of back pain. As delay in the diagnosis of vertebral lytic lesion may have sequelae in the growing children, pain disproportionate to the signs should have low threshold levels for evaluation. Read More

    [Emphysema, did you say emphysema?]
    Rev Mal Respir 2018 Jan 3;35(1):83-87. Epub 2018 Feb 3.
    Service de pneumologie, hôpital Pasteur, universite Côte-d'Azur, FHU OncoAge, centre hospitalier universitaire de Nice, 06000 Nice, France.
    Introduction: Chronic obstructive pulmonary disease (COPD) is a common condition that may initially look simple but may conceal other diseases capable of accelerating its natural history or even simulating it. We describe four cases presenting as COPD with emphysema that were reclassified on the basis of certain clinical characteristics and the radiological pattern.

    Case Reports: A 52 year old never smoking woman presenting with emphysema was eventually diagnosed as having lymphangioleiomyomatosis on the basis of an abdominal CT scan showing kidney angiomyolipomas. Read More

    Xanthoma Disseminatum Presenting with Hoarseness.
    Iran J Otorhinolaryngol 2017 Nov;29(95):365-368
    Department of Pathology, JIPMER, Puducherry, India.
    Introduction: Xanthoma disseminatum (XD) is a rare, benign, non-Langerhans cell histiocytic disorder with unknown etio-pathology. It manifests with multiple, grouped, red-brown to yellow papules and nodules involving the skin, mucous membranes, and internal organs with a predilection for flexures and the face.

    Case Report: We report a patient who presented with disseminated xanthomatous papules and nodules involving the face, neck, trunk, axilla, groin, and oral cavity, along with hoarseness of voice. Read More

    Emergent presentation of Langerhans cell histiocytosis in a pediatric patient: Acute cerebellar involvement causing obstructive hydrocephalus requiring posterior fossa decompression.
    Indian J Radiol Imaging 2017 Oct-Dec;27(4):432-435
    Department of Radiology, University of Florida, Gainesville, Florida, USA.
    Langerhans cell histiocytosis (LCH) is a disorder of the monocyte-macrophage system that can be unifocal or systemic. Here, we present a pediatric case who initially presented with osseous LCH but again presented 6 years later emergently with cerebellar symptoms, cerebellar mass and obstructive hydrocephalus. Patient underwent biopsy of the cerebellum which was path proven intracranial LCH. Read More

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