scholarly journals Langerhans cell histiocytosis: Skin diseases and visceral lesions

2017 ◽  
Vol 89 (4) ◽  
pp. 115-120
Author(s):  
V D Elkin ◽  
T G Sedova ◽  
E A Kopytova ◽  
E V Plotnikova
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Skin Diseases ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Literature Analysis ◽  
Trigger Factors ◽  
Statistical Variations ◽  
The World

Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients. In addition to skin lesions, there is involvement of one or more visceral organs. Difficulties in diagnosing the disease lead to statistical variations of LCH in different countries of the world and require more attention by physicians of all specialties.

scholarly journals Advanced ulcerative tumefactive lesions of the mucocutaneous surface in previously unrecognized Langerhans Cell Histiocytosis

2021 ◽  
Vol 12 (4) ◽  
pp. 419-421
Author(s):  
Fatima Azzahra Elgaitibi ◽  
Soumaya Hamich ◽  
Noureddine Mahiou
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Rare Case ◽  
Clinical Presentation ◽  
Histopathological Examination ◽  
Systemic Disease ◽  
Skin Lesions ◽  
Clinical Suspicion ◽  
Langerhans Cell ◽  
Multiple Ulcers

Langerhans cell histiocytosis (LCH) is an uncommon systemic disease characterized by the infiltration of one organ or more by Langerhans cells. Its clinical presentation is heterogeneous and depends on the affected organs. We report the rare case of a 43-year-old female presenting herself with central diabetes insipidus and receiving nasal desmopressin spray. Later, the patient developed multiple papules and pustules progressing to multiple ulcers affecting the retroauricular and axillary folds, as well as the oral and genital mucosas. The skin lesions had been evolving for several months. A histopathological examination of a skin biopsy confirmed the clinical suspicion of LCH.

A Human Anti-CD1a Monoclonal Antibody for Targeted Immunotherapy in Langerhans Cell Histiocytosis and Hematologic Malignancies.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4815-4815
Author(s):  
Gitanjali Bechan ◽  
David W. Lee ◽  
R. Maarten Egeler ◽  
Robert J. Arceci
Keyword(s):  
Monoclonal Antibody ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Specific Binding ◽  
Human Monoclonal Antibody ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
Hematologic Malignancies ◽  
Langerhans Cell ◽  
Number Of Patients

Abstract Langerhans Cell Histiocytosis (LCH) is characterized by the clonal proliferative of immature Langerhans cells resulting in a disorder with a wide variety of clinical manifestations and outcomes. Current therapies are based on the use of chemotherapeutic and immunosuppressive agents, usually in combination. Treatments are relatively non-specific, associated with significant adverse side effects and are ineffective in a significant number of patients. More effective and less toxic treatments are therefore needed. The selective expression of the nonclassical MHC protein, CD1a, on the lesional Langerhans cells in LCH makes this antigen an excellent immunotherapeutic target. To this end, we have generated a completely human monoclonal antibody (Mab) that specifically binds to a native, external epitope of CD1a using a human spleen phage display library. Epitope binding regions were selected for recognition of external domains of native CD1a and sequences showing specific binding were cloned into a human IgG1 backbone. The anti-CD1a Mab, termed 2113, shows high affinity and specificity for CD1a and is internalized after binding to the surface of CD1a expressing cells. The 2113 Mab also demonstrates significant complement dependent lysis (CDC) and antibody dependent cell cytotoxicity (ADCC) against CD1a expressing cells. No direct antibody mediated apoptosis was observed for 2113 binding to CD1a. A preclinical murine CD1a positive tumor model is being used to test the 2113 Mab for selective localization and tumor cytotoxicity. This Mab should provide an excellent reagent for therapeutic targeting of LCH and CD1a positive hematologic malignancies as well as possibly having application in the treatment of autoimmune disorders, prevention of graft-versus-host disease and augmenting cancer vaccines through direct antigen delivery to Langerhans cells.

Disorders of Macrophages And Dendritic Cells

2017 ◽  
Author(s):  
Alexei A. Grom ◽  
Michael B Jordan ◽  
Jun Qin Mo
Keyword(s):  
Dendritic Cells ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Phagocytic Activity ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Skin Lesions ◽  
Dendritic Morphology ◽  
Langerhans Cell ◽  
Juvenile Xanthogranuloma ◽  
Specific Cell

The mononuclear phagocytic system consists of dendritic cells (DCs) and monocytes/macrophages, historically referred to as histiocytes. The macrophages exhibit varying degrees of phagocytic activity. DCs typically have dendritic morphology. Their phagocytic activity is limited. Instead, they play a key role in antigen presentation to lymphocytes. The various populations of macrophages and DCs (such as Langerhans cells [LCs] and dermal dendrocytes) are usually distinguished based on characteristic morphology and patterns of expression of specific cell surface and intracellular markers. Abnormal accumulation and behavior of these cells may lead to the development of a spectrum of diseases collectively known as the histiocytoses. Clinically, histiocytic disorders comprise a wide variety of conditions that affect both children and adults and range from benign skin lesions to rapidly progressive life-threatening systemic disorders. LCs play a pivotal role in the development of Langerhans cell histiocytosis, dermal dendrocytes are the predominant cell population in the lesions of juvenile xanthogranuloma, and macrophages are central to the pathogenesis of hemophagocytic lymphohistiocytosis and related disorders. This review contains 5 figures, 3 tables, and 77 references. Key words: dendritic cells, dermal dendrocytes, hemophagocytic lymphohistiocytosis, histiocytes, juvenile xanthogranuloma, Langerhans cell histiocytosis, Langerhans cells, macrophage activation syndrome, macrophages 

Pulmonary Langerhans Cell Histiocytosis

2020 ◽  
Vol 41 (02) ◽  
pp. 269-279
Author(s):  
Brian Shaw ◽  
Michael Borchers ◽  
Dani Zander ◽  
Nishant Gupta
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Myeloid Neoplasm ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Mitogen Activated Protein ◽  
The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

scholarly journals Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Rashid AL Umairi ◽  
Danielle Blunt ◽  
Wedad Hana ◽  
Matthew Cheung ◽  
Anastasia Oikonomou
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Corticosteroid Treatment ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Sinus Histiocytosis ◽  
Review Of The Literature ◽  
Massive Lymphadenopathy ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

Clonal proliferation of Langerhans cells in Langerhans cell histiocytosis

The Lancet ◽  
1994 ◽  
Vol 343 (8900) ◽  
pp. 767-768 ◽  
Author(s):  
R.C. Yu ◽  
A.C. Chu ◽  
C. Chu ◽  
L. Buluwela
Keyword(s):  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Clonal Proliferation

Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

2001 ◽  
Vol 5 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Kelli W. Morgan ◽  
Jeffrey P. Callen
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Rare Condition ◽  
Signs And Symptoms ◽  
Male Infant ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Self Healing ◽  
Systemic Involvement ◽  
S 100 ◽  
Simplex Virus

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.

Gene expression analysis of dendritic/Langerhans cells and Langerhans cell histiocytosis

2006 ◽  
Vol 209 (4) ◽  
pp. 474-483 ◽  
Author(s):  
R Rust ◽  
J Kluiver ◽  
L Visser ◽  
G Harms ◽  
T Blokzijl ◽  
...  
Keyword(s):  
Gene Expression ◽  
Expression Analysis ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Gene Expression Analysis ◽  
Langerhans Cell

scholarly journals A Case Report of Pediatric Langerhans Cell Histiocytosis: Current Approach and Diagnostic Challenges for Dermatologist

2020 ◽  
Vol 12 (3) ◽  
pp. 79-86
Author(s):  
Irwan Junawanto ◽  
Khairuddin Djawad ◽  
Sri Rimayani ◽  
Farida Tabri ◽  
Nurelly N. Waspodo ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Poor Prognosis ◽  
Clinical Manifestations ◽  
Current Approach ◽  
Myeloproliferative Disorder ◽  
Langerhans Cell ◽  
Histopathologic Examination ◽  
Chemotherapy Treatment ◽  
Cutaneous Manifestations ◽  
Patient Organ

Abstract Langerhans Cell Histiocytosis (LCH) is a chronic and rare myeloproliferative disorder caused by disorders in Lang-erhans cell proliferation in various organs and tissues. LCH has a wide variety of clinical manifestations, making it difficult to diagnose. Cutaneous manifestations are polymorphic in the form of purpura, papule, vesicles and pustules. LCH can involve vital organs such as the liver and lungs as well as the hematopoiesis system that usually gives a poor prognosis. The prognosis is also influenced by the age of patient, organ dysfunction and response to the first 6 weeks of chemotherapy treatment. A 3-year-old girl reported a major complaint of an abscess-like lesion in the region of neck accompanied by an extensive purpura of scalp, neck and inguinal areas accompanied by vulvar erosions. The immunohistochemical and histopathologic examination support LCH and the clinical improvement after intravenous administration of intravenous 3 mg/m2 Vinblastine chemotherapy, 75 mg/m2 etoposide, oral 40 mg/m2 per prednisone. After the 6th cycle of chemotherapy, the patient died.

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