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Dermatology
Histiocytoses
Built from Dermatology, 5th Edition
What’s inside
8 sections · 129 slides
Overview
- What this topic covers
Overview and Classification
What a histiocytosis is, how it develops, and how the group is organized
- What is a histiocytosis
- Three histiocytic cells of the skin
- How the disease actually starts
- The growth signal that drives histiocytoses
- Five-group classification of histiocytoses
- The five histiocytosis groups
- A field guide before the details
- Clinical features of the histiocytoses.
- Clinical features of the histiocytoses. (continued)
- Clinical features of the histiocytoses. (continued)
- Clinical features of the histiocytoses. (continued)
- Clinical features of the histiocytoses. (continued)
- Clinical features of the histiocytoses. (continued)
- Telling the cells apart under the microscope
- Classic macrophage and dermal dendrocyte immunostains
- Antigenic markers of the histiocytoses.
- Antigenic markers of the histiocytoses. (continued)
L Group: Langerhans-Related Histiocytoses
Langerhans cell histiocytosis, indeterminate cell histiocytosis, and Erdheim-Chester disease
- Langerhans cell histiocytosis: key features
- A history of confusing names
- How common is LCH
- The BRAF mutation behind LCH
- The spectrum of LCH severity
- Skin findings in LCH
- Langerhans cell histiocytosis: clinical spectrum
- Acute multisystem LCH (Letterer-Siwe pattern)
- Congenital self-healing LCH (Hashimoto-Pritzker disease)
- Congenital self-healing Langerhans cell histiocytosis
- Red-purple papule of congenital self-healing LCH
- Chronic LCH: the classic triad
- Bone disease and diabetes insipidus in LCH
- LCH in adults
- LCH and malignancy: two different patterns
- Prognosis in LCH
- LCH under the microscope
- Langerhans cell histiocytosis: histopathologic features
- Confirming the diagnosis: LCH's molecular fingerprint
- Birbeck granules on electron microscopy
- Telling LCH apart from its look-alikes
- A four-immunostain panel distinguishing LCH from non-LCH
- Working up a patient with LCH
- Systemic and targeted therapy for LCH
- Indeterminate cell histiocytosis: key features
- Indeterminate cell histiocytosis: background and cause
- How indeterminate cell histiocytosis looks and behaves
- Indeterminate cell histiocytosis on the leg
- Pathology and workup of indeterminate cell histiocytosis
- Erdheim-Chester disease: key features
- Erdheim-Chester disease: history and how common it is
- The bone and fibrosis findings of Erdheim-Chester disease
- Skin lesions of Erdheim-Chester disease
- Xanthelasma-like eyelid plaques in Erdheim-Chester disease
- Erdheim-Chester disease under the microscope, and its mimics
C Group: Cutaneous and Mucocutaneous Histiocytoses
Non-Langerhans cell histiocytoses of the skin, from benign cephalic histiocytosis to Rosai-Dorfman mimics
- Benign cephalic histiocytosis: key features
- Benign cephalic histiocytosis: presentation and course
- Benign cephalic histiocytosis: pathology and diagnosis
- Generalized eruptive histiocytoma: key features
- Generalized eruptive histiocytoma: presentation
- Generalized eruptive histiocytomas in a young child
- Generalized eruptive histiocytoma: pathology and care
- Juvenile xanthogranuloma: key features
- Juvenile xanthogranuloma: naming history
- Juvenile xanthogranuloma: how common, and why it happens
- Two clinical patterns of juvenile xanthogranuloma
- Juvenile xanthogranuloma: clinical spectrum from pink to yellow
- Juvenile xanthogranuloma nodule with central yellow color
- Juvenile xanthogranuloma with a yellow-red color
- Eye involvement: the one JXG complication to catch early
- JXG, neurofibromatosis, and leukemia risk
- Juvenile xanthogranuloma under the microscope
- Juvenile xanthogranuloma: early cellular infiltrate to Touton giant cells
- Juvenile xanthogranuloma: telling it apart, and treatment
- Progressive nodular histiocytosis: key features
- Progressive nodular histiocytosis: presentation and diagnosis
- Progressive nodular histiocytosis: pathology and treatment
- Xanthoma disseminatum: key features
- Xanthoma disseminatum: how common, and why it happens
- Xanthoma disseminatum: presentation
- Xanthoma disseminatum: sclerotic form with scarring
- Xanthoma disseminatum: flexural involvement of the axilla
- Xanthoma disseminatum: involvement of the major flexures
- Xanthoma disseminatum: sclerotic form in a patient with multiple myeloma
- Three clinical courses of xanthoma disseminatum
- Xanthoma disseminatum: pathology, mimics, and treatment
- Progressive mucinous histiocytosis: key features
- Progressive mucinous histiocytosis: presentation and care
- Necrobiotic xanthogranuloma: key features
- Necrobiotic xanthogranuloma: presentation
- Necrobiotic xanthogranuloma: the paraprotein connection
- Necrobiotic xanthogranuloma under the microscope
- Necrobiotic xanthogranuloma: cholesterol clefts and giant cells
- Necrobiotic xanthogranuloma: mimics and treatment
- Multicentric reticulohistiocytosis: key features
- Multicentric reticulohistiocytosis: how common, and why it happens
- Multicentric reticulohistiocytosis: presentation
- Multicentric reticulohistiocytosis: papules and arthritis mutilans
- Multicentric reticulohistiocytosis under the microscope
- Multicentric reticulohistiocytosis: "ground glass" giant cells
- Multicentric reticulohistiocytosis: mimics and treatment
R Group: Rosai-Dorfman Disease
Massive lymphadenopathy, extranodal disease, and the histologic hallmark of emperipolesis
- Rosai-Dorfman disease: key features
- Rosai-Dorfman disease: history and how common it is
- What drives Rosai-Dorfman disease
- Rosai-Dorfman disease: systemic features
- Cutaneous Rosai-Dorfman disease
- Rosai-Dorfman disease: papules and plaques of the cheek and chin
- Rosai-Dorfman disease: nodules on the buttock
- Rosai-Dorfman disease under the microscope: emperipolesis
- Rosai-Dorfman disease: emperipolesis on immunostaining
- Rosai-Dorfman disease: mimics and treatment
M Group and Other Rare Histiocytoses
Malignant histiocytoses and ALK-positive histiocytosis
- Malignant histiocytoses
- ALK-positive histiocytosis
- ALK-positive histiocytosis: markers and treatment
Summary
The through-line that connects every histiocytosis in this chapter
- Key takeaways
- How to approach a suspected histiocytosis
References
Source citations from the chapter
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- Dermatology, 5th Edition (2-Volume Set)