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.

Pulmonary Langerhans Cell Histiocytosis and Other Pulmonary Histiocytic Diseases: A Review

2008 ◽  
Vol 132 (7) ◽  
pp. 1171-1181 ◽  
Author(s):  
Timothy Craig Allen
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Correct Diagnosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
Data Sources ◽  
Primary Material ◽  
Pulmonary Langerhans Cell Histiocytosis

Abstract Context.—Pulmonary Langerhans cell histiocytosis is the most common and best known pulmonary histocytic lesion; however, the realm of pulmonary histiocytic lesions also includes an assortment of uncommon diseases that may exhibit pulmonary involvement. Objective.—To review pulmonary Langerhans cell histiocytosis and other pulmonary histiocytoses to better ensure correct diagnosis and optimal assessment of prognosis and treatment. Data Sources.—Literature review and primary material from the author's institution. Conclusions.—This review discusses the most common pulmonary histocytosis, pulmonary Langerhans cell histiocytosis, and also reviews the uncommon pulmonary histiocytic lesions, which are distinct from pulmonary Langerhans cell histiocytosis.

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.

An unusual co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: report of a case and review of the literature

2013 ◽  
Vol 53 (5) ◽  
pp. 558-563 ◽  
Author(s):  
Eran Cohen-Barak ◽  
Dganit Rozenman ◽  
Jan Schafer ◽  
Judith Krausz ◽  
Roni Dodiuk-Gad ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Review Of The Literature ◽  
Rosai Dorfman Disease

scholarly journals Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
K. Earlam ◽  
C. A. Souza ◽  
R. Glikstein ◽  
M. M. Gomes ◽  
S. Pakhalé
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pulmonary Involvement ◽  
Langerhans Cell ◽  
Cytotoxic Agents ◽  
Current Cigarette Smoking ◽  
System Disease ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Multisystem Involvement ◽  
Organ Systems ◽  
Current Cigarette

Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

scholarly journals Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Alessandra Rizzoli ◽  
Simona Giancristoforo ◽  
Cristina Haass ◽  
Rita De Vito ◽  
Stefania Gaspari ◽  
...  
Keyword(s):  
Oral Mucosa ◽  
Langerhans Cell Histiocytosis ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Self Healing ◽  
Review Of The Literature ◽  
Cutaneous Lesions ◽  
Cutaneous Manifestations ◽  
Case Presentation

Abstract Background Congenital self-healing reticulohistiocytosis (CSHRH), also called Hashimoto-Pritzker disease, is a rare and benign variant of Langerhans cell histiocytosis, characterized by cutaneous lesions without extracutaneous involvement. Case presentation We present a case of CSHRH with diffuse skin lesions and erosions in the oral mucosa, present since birth and lasting for 2 months, and we perform a review of the literature on Pubmed in the last 10 years. Conclusions Our case confirm that lesions on oral mucosa, actually underestimated, may be present in patients with CSHRH. Patients affected by CSHRH require a close follow-up until the first years of life, due to the unpredictable course of Langerhans cell histiocytosis, in order to avoid missing diagnosis of more aggressive types of this disorder.

scholarly journals Pulmonary Langerhans cell histiocytosis with Skin involvement; an amazing case report

2019 ◽  
Vol 7 (1) ◽  
pp. 32-36
Author(s):  
Sara Saniee ◽  
Niloofar Afshari ◽  
Saeed Kashefi
Keyword(s):  
Case Report ◽  
Langerhans Cell Histiocytosis ◽  
Pulmonary Involvement ◽  
Medical Problem ◽  
Langerhans Cell ◽  
Pathology Report ◽  
Skin Involvement ◽  
Recurrent Pneumothorax ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Progressive Dyspnea

Introduction: Langerhans cell histiocytosis (LCH) is a rare disease characterized by infiltration of large mononuclear or dendritic cells in one or more organs. Lung can be involved alone or with other organs in this complication. Case Report: We report a 33-year-old smoker man with LCH in whom both the lung and the skin were involved. He was referred to our department due to malodourous, crusted and scaly erythematous patches, plaques, and pustules involved scalp and flexural area. His medical problem started with pulmonary involvement. He experienced spontaneous pneumothorax, and was suffering progressive dyspnea for years. Cryo-transbronchial lung biopsy previously had been done. Pathology report was pulmonary LCH (PLCH). Weid skin biopsy. Histologic analysis of skin with haemotoxylin and eosin (H&E) staining and immunohistochemistry (IHC) exam with S100 and CDa1 confirmed the diagnosis of LCH. Conclusion: It is important to consider PLCH in the setting of recurrent pneumothorax and progressive dyspnea in middle-aged smoker patient. PLCH may be associated with skin involvement.

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