Langerhans Cell Histiocytosis Presenting Research Articles

Presentation of pulmonary Langerhans cell histiocytosis before the development of lung cysts.

Pulmonary Langerhans cell histiocytosis (PLCH) affects mainly young, predominantly smoking adults with a peak at 20–40 years of age. Patients with PLCH often present with a nonproductive cough and/or dyspnea. High-resolution CT (HRCT) is the most important diagnostic modality in PLCH. The typical HRCT pattern combines small poorly limited nodules, cavitated nodules, and finally thick- and thin-walled cysts. In rare cases, HRCT enables PLCH to be diagnosed prior to the development of cysts.

Congenital Langerhans Cell Histiocytosis with Placental Involvement

Congenital presentations of Langerhans cell histiocytosis (LCH) are exceedingly rare, and concurrent placental parenchymal involvement has not been definitively documented in the literature. We present 2 cases of congenital multisystem LCH with placental involvement resembling chronic villitis. Placental examination may provide the initial diagnostic evidence of LCH and may significantly influence patient management and outcome; however, the prognostic implications remain unclear. In a clinical context suspicious for congenital LCH, the observation of chronic villitis should prompt consideration of placental involvement by LCH.

Langerhans Cell Histiocytosis Presenting as a Unifocal Eosinophilic Granuloma in the Body of the Mandible: A Rare Report

ABSTRACT Introduction Langerhans cell histiocytosis (LCH) is a relatively rare and unique benign disease characterized by an abnormal proliferation of immature dendritic cells which usually affects children and young adults. Jaws are involved in less than 10% of children with the disease. Objective To add on to the literature one more case of this rare and unique disease which accounts for less than 1% of all bone tumors and also to discuss its oral manifestation and management. Design Case report Setting Private maxillofacial surgery institute, Kolkata, West Bengal. Patient A 2 years and 8 months old male child with solitary swelling over the lower left jaw since one and half months. Lesion was fixed to underlying bone and there was an expansion of buccal cortical plate at the body of mandible. Results Enucleation of the lesion performed after assessing history, clinical and radiological dertails and also considering patient's age. Postoperative biopsy result came as LCH presenting as a unifocal eosinophil granuloma. Appropriate surgical intervention resulted in a dramatic improvement in patient's clinical condition. At present, patient is disease-free and asymptomatic. Conclusion Surgical enucleation or curettage is a preferred treatment option for maxillary and mandibular lesion. Potential for the unifocal disease to become multifocal should not be underestimated as the disease can be unpredictable. We recommend long-term follow-up due to uncertainty of the disease.

Langerhans cell histiocytosis presenting as a “blueberry muffin baby”

Langerhans cell histiocytosis presenting as a “blueberry muffin baby”

“Blueberry muffin” rash and large right thigh mass: a unique presentation of Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a clonal proliferation of bone marrow derived antigen-presenting cells that can involve a spectrum of cutaneous findings, with or without internal organ involvement. Neonatal LCH almost always presents with skin findings, usually petechial papules and/or erosions in a seborrheic distribution, with or without extracutaneous involvement. Previously described as varying entities, LCH is now considered a single disease process demonstrating a spectrum of clinical findings. We report a unique case of neonatal LCH presenting with a "blueberry muffin" rash in conjunction with a large soft tissue tumor.

Langerhans cell histiocytosis presenting with complicated pneumonia, a case report

We describe a 2 ½ year old boy presenting with fever, abdominal pain and splinter haemorrhages of the nails. On further examination there were signs of pneumonia with pleural effusion. This was treated with mini-thoracotomy, drainage and intravenous antibiotics. Further diagnostic workup for underlying causes showed diffuse cystic lung disease, suggestive of Langerhans cell histiocytosis. This was confirmed on pathology specimens, which showed Langerhans cells in lung tissue, nail bed and skin biopsy samples, indicating multisystem Langerhans cell histiocytosis. The patient was treated with Prednisone and Vinblastin according to the LCH-III guidelines. In this case report we give a brief description on cystic lung disease in children, Langerhans cell histiocytosis and associated nail abnormalities.

Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.

A Case of Langerhans Cell Histiocytosis Presenting with Bilateral Otorrhea

Langerhans cell histiocytosis (LCH) involves the proliferation and accumulation of Langerhans cells at various sites and presents with various symptoms, depending on the organ or organs invaded. A 2-year-old boy with LCH was treated at our hospital. Fever, head rash, bilateral chronic otorrhea unresponsive to antibiotics, and left exophthalmos were observed at our first examination. The CT scan showed destruction of the skull bone, zygomatic bone and vertebrae, and masses within these lesions. No bony defect was detectable in the middle ear, but a mass was present in the external auditory meatus. Skull X-rays revealed characteristic “punched-out” lesions. We confirmed the diagnosis of LCH with a biopsy of the head mass and the external auditory meatus mass. Immunohistology revealed positive staining of the lesional cells with CD1a and Langerin (CD207). The final diagnosis was multisystem LCH (MS-LCH). The child was placed on the chemotherapeutic regimen recommended by the Histiocyte Society.

Colonic Langerhans Cell Histiocytosis Presenting as an Isolated Polyp

Correspondence to Koon Hee Han, M.D. Department of Internal Medicine, Gangneung Asan Hospital, 38 Bangdong-gil, Sacheon-myeon, Gangneung 210-711, Korea Tel: +82-33-610-3139, Fax: +82-33-610-8130, E-mail: gi@gnah.co.kr Copyrightc 2013 The Korean Association of Internal Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 대장 용종에 국한되어 발생한 랑게르한스 세포 조직구증 1예

A Case of Langerhans Cell Histiocytosis Presenting with Bilateral Otorrhea

A Case of Langerhans Cell Histiocytosis Presenting with Bilateral Otorrhea

Multisystem Langerhans cell histiocytosis presenting as an oral lesion

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which the pathologic Langerhans cells infiltrate and destroy the tissues. Patients with LCH present varied clinical manifestations. Cutaneous lesions in LCH manifest as vesiculopapular eruptions that often mimic various infectious diseases particularly in infants. We present a case of a female infant with an ulcerative lesion intraorally. The baby was asymptomatic otherwise. A detailed history revealed the presence of cutaneous lesions that was overlooked by her parents.Conclusion:This report tries to briefly discuss the current concepts regarding the etiology of LCH. An attempt has been made to emphasis the need for a through systemic examination. The protocol of investigative procedures to be adopted in LCH is also discussed.

Rapidly Fatal Multiorgan Langerhans Cell Histiocytosis in a Neonate

Multiorgan Langerhans cell histiocytosis (LCH) presenting in the neonatal period is associated with a poor prognosis. We report a 5-day-old neonate who presented at birth with diffuse cutaneous nodules and respiratory failure who received a postmortem diagnosis of LCH. We emphasize the importance of recognizing the cutaneous findings of this rare disease.

Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy

Langerhans cell histiocytosis (LCH) in adults first presenting in the skin is rare. Guidelines for staging, treatment and follow-up are lacking. To better define staging procedures, treatment results and clinical course in adult patients with LCH first presenting in the skin. Eighteen adult patients with LCH first presenting in the skin were collected from five centres collaborating in the Dutch Cutaneous Lymphoma Group. Clinical records and (skin) biopsy specimens were reviewed and follow-up data were obtained. A literature search on adult patients with LCH presenting in the skin was performed. Staging procedures showed extracutaneous disease in three of 16 patients who were adequately staged. One patient had a histologically confirmed lytic LCH bone lesion, while two patients had a myelodysplastic syndrome. During follow-up two of 18 patients developed extracutaneous localizations of LCH. Five patients developed a second haematological malignancy, including (myelo)monocytic leukaemia (two cases), histiocytic sarcoma (one case), diffuse large B-cell lymphoma (one case) and peripheral T-cell lymphoma (one case). Review of the literature revealed six other adult patients with a second haematological malignancy preceding or following a diagnosis of LCH. The results of the present study suggest an increased risk of a second haematological malignancy in adult patients with LCH presenting in the skin. Extensive staging at presentation and long-term follow-up are therefore warranted in such patients.

Pulmonary Langerhans Cell Histiocytosis Presenting as Recurrent Spontaneous Pneumothorax in an Adolescent Male

Pulmonary Langerhans Cell Histiocytosis Presenting as Recurrent Spontaneous Pneumothorax in an Adolescent Male

Langerhans cell histiocytosis presenting as a multi‐system disorder in an infant

Langerhans cell histiocytosis presenting as a multi‐system disorder in an infant

Clinical Profile of Langerhans Cell Histiocytosis at a Tertiary Centre: A Prospective Study

To study the varied presentations of Langerhans Cell Histiocytosis (LCH), the differential diagnosis of the varied presentations and the time lag in achieving the diagnosis. Prospective analysis of children diagnosed to have LCH over a period of 51 mo was done. A complete history and physical examination was undertaken in all patients, followed by relevant laboratory and radiological evaluation. Biopsy of the appropriate specimen was done. The extent of the disease was documented, accordingly treated and followed up. There were 16 children with LCH from October 2005 through December 2009. The age ranged from 8 mo to 72 mo. Diagnosis was confirmed by CD1a/S 100 in 15 children (93.75%). The mean time to arrive at the diagnosis was 9.9 mo. Multisystem disease was documented in 11 (68.75%) children and there were 4 (25.0%) cases of pulmonary LCH. The mean time of follow-up was 14.4 mo (range, 1 mo to 50.6 mo). Most common referral diagnoses in LCH patients was recurrent pneumonia and immunodeficiency. There is a need for high index of suspicion for diagnosis of LCH; misdiagnosis is frequent. Pulmonary involvement in children with LCH appears common. It is possibly still underdiagnosed. Nail changes are uncommon, but may act as a marker for multisystem disease. In addition to survival data and analysis of prognostic factors, the prospective collection of data on diverse presentations is essential, along with a high index of suspicion for the diagnosis of LCH.

Langerhans Cell Histiocytosis Presenting with Headache and Sellar Mass in an Adult

Introduction Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by aberrant proliferation of a specific dendritic (Langerhans) cell belonging to the monocyte macrophage system. 1 These cells can infiltrate virtually any organ without inducing dysfunction. 2 LCH is encountered more often in children, with a peak age range of 1-3 years and an incidence of 3-5 cases per million per year and a male to female ratio of 2:1. 3 LCH is rare in adults and the incidence may be underestimated due to the fact that many cases likely go undiagnosed. In order of decreasing frequency, the presenting symptoms are skin rash, dyspnea or tachypnea, polyuria and polydipsia, bone pain, lymphadenopathy, weight loss, fever, gingival hypertrophy, ataxia, and memory problems. 4 Regarding the endocrine system, LCH has a particular predilection for involvement of the hypothalamo–pituitary axis (HPA), leading to diabetes insipidus (DI) in up to 50% of cases. In a recent analysis, DI was the most common and permanent consequence of LCH, occurring in 24% of patients. 5 Other endocrine deficiencies can develop in up to 20% of patients. 5,6 Endocrine manifestations include DI followed by growth hormone deficiency with a median latency of about one year, followed by gonadotropin deficiency with a median latency of about seven years from the diagnosis of DI. ACTH and TSH deficiency also have been described. 7

Langerhans Cell Histiocytosis

The cutaneous presentation of Langerhans cell histiocytosis (LCH) is very rare and can be highly variable among individuals, which can often lead to a delay in diagnosis. To discuss a case report and literature review of important clinical indicators, histology, diagnosis, evaluation, and treatment guidelines. Case report and literature review. Skin biopsies positive for CD1a and/or langerin are diagnostic for LCH. A thorough review of systems, baseline laboratory tests, and imaging studies can determine the extent of LCH. Treatment of cutaneous disease is largely based on case report and small case studies, but baseline treatment should generally begin with oral or topical steroids. When patients have more severe disease that requires a systemic approach, the efficacy of therapy should be assessed 6 weeks into therapy, with subsequent treatment intensification in patients with limited response. Owing to the rarity of this condition, there are no specific guidelines for treatment of LCH, but guidelines put forth by the Histiocyte Society assist in categorization and basic treatment approaches for patients with systemic disease.

Langerhans cell histiocytosis: Two clinical presentations in the same patient

Langerhans cell histiocytosis (LCH) is a heterogeneous group of diseases characterized by a pathological proliferation of cells phenotypically similar to Langerhans cells. The disease course is variable, alternating between resolving and potentially fatal forms. The diagnosis is based on clinical appearance and confirmed by CD1a positivity and / or immunohistochemistry. We report the case of a male child of 3 months with two different presentations of Langerhans cell-histiocytosis (LCH) at different times. The first presentation was classified as a self-healing LCH (formerly known as Hashimoto-Pritzker). The last presentation, although clinically suggestive of Letterer-Siwe (former designation), was not associated with systemic disease. This emphasizes that LCH cannot be compartmentalized into four groups, but considered a single disease with a wide spectrum of clinical presentations. This case underscores the importance of frequent and long-term follow-up of these patients.

Congenital self-healing histiocytosis presenting as blueberry muffin baby: A case report and literature review

Congenital self-healing Langerhans cell histiocytosis (CSHLCH), also called as Hashimoto-Pritzker disease, is a rare, benign variant of histiocytosis. Despite the initial dramatic clinical presentation, affected infants are otherwise healthy and skin lesions disappear spontaneously within several weeks to months. We present a case of CSHLCH presenting as blueberry muffin baby. The lesions appeared in the first week of life and lasted 6 months. The follow-up period was 24 months, without any signs of relapse. At the pediatric dermatology unit of our clinic, during the last 20 years, we had 10 children with Langerhans cell histiocytosis and among them only one with CSHLCH. In the literature, we found only 5 newborns with Langerhans cell histiocytosis presenting as blueberry muffin baby, among them only 4 with self-healing CSHLCH. The early recognition of CSHLCH may spare children from redundant and potentially toxic systemic treatment.