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

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.

Download Full-text

Langerhans cell histiocytosis: Skin diseases and visceral lesions

Terapevticheskii arkhiv ◽

10.17116/terarkh2017894115-120 ◽

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.

Download Full-text

Langerhans Cell Histiocytosis Involving the Gastrointestinal Tract

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2014-0290-cc ◽

2014 ◽

Vol 138 (10) ◽

pp. 1350-1352 ◽

Cited By ~ 6

Author(s):

Amir Behdad ◽

Scott R. Owens

Keyword(s):

Gastrointestinal Tract ◽

Langerhans Cell Histiocytosis ◽

Clinical Presentation ◽

Pediatric Population ◽

Systemic Disease ◽

Langerhans Cell ◽

Braf V600e ◽

Molecular Testing ◽

The Past ◽

Variable Clinical Presentation

Langerhans cell histiocytosis is a rare disease with a variable clinical presentation, and its prognosis and treatment depend on the extent and severity of disease. Although the pathogenesis of Langerhans cell histiocytosis has been debated in the past, recent evidence suggests that it is a neoplastic proliferation potentially derived from a myeloid-lineage precursor. Involvement of the gastrointestinal tract in Langerhans cell histiocytosis is exceedingly rare and is most often encountered in the pediatric population, in the setting of systemic disease. This is illustrated using a case of Langerhans cell histiocytosis involving the esophagus in a 59-year-old woman who presented with dysphagia, in which molecular testing documented a BRAF V600E mutation. We review the features and implications of this diagnosis.

Download Full-text

A neonate with Langerhans cell histiocytosis presenting as blueberry muffin rash: Case report and review of the literature

SAGE Open Medical Case Reports ◽

10.1177/2050313x20919616 ◽

2020 ◽

Vol 8 ◽

pp. 2050313X2091961

Author(s):

Janelle Cyr ◽

Annie Langley ◽

Dina El Demellawy ◽

Michele Ramien

Keyword(s):

Case Report ◽

Site Selection ◽

Langerhans Cells ◽

Langerhans Cell Histiocytosis ◽

Clinical Suspicion ◽

Langerhans Cell ◽

Imaging Findings ◽

Review Of The Literature ◽

Biopsy Site ◽

High Degree

In our case report, we discuss a 1-day-old boy presenting with blueberry muffin syndrome diagnosed with Langerhans cell histiocytosis. The diagnosis complicated by an initial difficult-to-interpret biopsy showing only a hint of perifollicular CD1a-positive cells; however, given our team’s strong clinical suspicion of Langerhans cell histiocytosis, a second biopsy of a more mature lesion was done and showed typical histopathology. This case introduces the possibility of perifollicular Langerhans cells early in this condition, demonstrates the importance of appropriate biopsy site selection, and highlights the importance of maintaining a high degree of suspicion when there is poor clinicopathologic correlation. Our case report contains a comprehensive table which reviews the systemic and cutaneous clinical features, as well as the laboratory, pathology, and imaging findings for the differential diagnoses of blueberry muffin baby.

Download Full-text

Disorders of Macrophages And Dendritic Cells

10.2310/im.1311 ◽

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

Download Full-text