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Dermatology Board Prep > DERM (07) Disorders of Langerhans Cells and Macrophages > Histiocyotses > Flashcards

Histiocyotses Flashcards

1
Q

What do all histocytes come from?

A

A common CD34+ progenitor in the bone marrow

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2
Q

What are the different histocytes?

A

Langerhans cell, mononuclear cell/macrophage, derma dendrocyte/dendritic cells [two types: 1. Type 1 derma dendrocyte and type 2 dermal dendrocyte]

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3
Q

What distinguished a Langerhans cell?

A

Potent APC that migrates to and from the epidermis

Stains + for CD1a, S100, and Langerin (CD207, most specific because it stains Birbeck granules

Birbeck granules are pathognomonic on electron microscopy

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4
Q

What distinguishes a mononuclear cell/macrophage?

A

MIgrates to and from the dermis and has phagocytic and APC abilities

Stains + for: CD68 and HAM56

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5
Q

What distinguishes the dermal dendrocytes/dendritic cells?

A

Type I: dermal dendrocyte, versatile factor XIIIa+ cell. Resides in the papillary dermis, involved in antigen presentation, phagocytosis, collagen production and wound healing

Type II derma dendrocyte: Less known, CD34+, lives in the reticular dermis

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6
Q

What is the major morphological difference between the histiocytic proliferations?

A

There is a high degree of overlap within the specific entities but a significant difference between the larger groups (non-langerhans vs langerhans histocytoses)

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7
Q

What is the primary determinant of prognosis in Langerhans cell histiocytosis?

A

Degree of systemic involvement (which is why the new classification system classifies these based on systemic involvement)

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8
Q

Is progression from skin-only Langerhan’s cell histiocytosis to systemic common?

A

No

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9
Q

What are good prognositc indicators for systemic Langerhan’s cell histiocytosis?

A

>2 years old and no involvement of the liver, lung, spleen, or hematopoietic system (100% survival)

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10
Q

What are features of bag prognosis in Langerhans cell histiocytosis?

A

BRAF V600E mutation and failure to reponsd to tx by 6 weeks

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11
Q

What types of malignancy are most common with Langerhans cell histiocytosis?

A

AML, ALL, other hemotologic malignancies

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12
Q

What genetic mutations are seen in Langerhans cell histiocytosis?

A

BRAF V600E [60% of pts] (also melanoma and papillary throid cancer) and MAP2K1 mutations which lead to ERK activation

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13
Q

What stains are + in Langerhans cell histiocytosis?

A

S100+, CD1a+, Langerin+

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14
Q

What notable stains are negative in Langerhans cell histiocytosis?

A

Factor XIIIa, CD68, and HAM56

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15
Q

What is the histology of Langerhans cell histiocytosis?

A

Dense proliferation of Langerhans cells with reniform nuclei and eosinophils in papillary dermis, with single and nested Langerhans cells in the epidermis

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16
Q

What forms of Langerhans cell histiocytosis start before the age of 2?

A

Letterer-Siwe and Congenital self-healing reticulohistiocytosis

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17
Q

What is the clinical presentation of Letterer-Siwe Langerhans cell histiocytosis?

A

Acute, disseminated visceral, and cutaneous lesions

  • These are 1-2 mm pink seborrheic papules/pustules/vesicles on the scalp, flexural neck/axilla, perineum trunk
  • Petechiae, purpura, scale, crust, erosion, impetiginization and tinder fissures common
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18
Q

What is the prognosis of Letterer-Siwe Langerhans cell histiocytosis?

A

Poor

Extensive visceral and painful osteolytic bone lesions; thrombocytopenia and anemia all suggest poor prognosis

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19
Q

What is a clue to the diagnosis of Letterer-Siwe Langerhans cell histiocytosis as compared to sebborrheic dermatitis?

A

The papules in this are more descrete than in seb derm, also can see petechiae, and purpura which should not be seen commonly in seb derm

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20
Q

What is the typical age of onset for Letterer-Siwe Langerhans cell histiocytosis?

A

Always less than 2 y/o

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21
Q

What is the most common age for Hand-Schüller-Christian Langerhans cell histiocytosis?

A

2-6 y/o

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22
Q

What is the clinical presentation of Hand-Schüller-Christian Langerhans cell histiocytosis?

A

Mostly radiographic

There is a triad: diabetes insipidus, osteolytic bone lesions (cranium), exophthalmos (least common)

-Skin lesions happen in 30% and look similar to Letterer-Siwe

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23
Q

What is the management of Hand-Schüller-Christian Langerhans cell histiocytosis?

A

The DI can be treated with vasopressin; bone lesions cured with curettage

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24
Q

What is diabetes insipidous seen in Hand-Schüller-Christian Langerhans cell histiocytosis?

A

Invasion into the posterior pituitary by histocytes

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25
Q

At what age does congenital self-healing reticulohistiocytosis occur?

A

Starts at birth or shortly therafter

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26
Q

What is the clinical presentation of congenital self-healing reticulohistiocytosis?

A

Skin-limited form

Rapidly self-healing; shows widespread (more than solitary) red or purple-brown papulonodules that can have erosion

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27
Q

What age does eosinophilic granuloma occur in?

A

Older children, 7-12 y/o

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28
Q

What is the clinical presentation of eosinophilic granuloma?

A

Localized from of langerhans cell histiocytosis. Usually solitary and asymptomatic bone lesions (cranium>ribs, spine, long bones)

-Very rare to involve the skin or mucosa

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29
Q

What is a common presenting symptom of eosinophilic granuloma?

A

Spontaneous fracture often the presenting sign

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30
Q

Treatment for eosinophilic granuloma?

A

Bone lesions can be treated with currette

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31
Q

What stains help distinguish non-Langerhans cell histiocytoses?

A

All are CD68+, +/- Factor XIIIa

All are negative for Langerin

S100 is negative in all except indeterminate cell histiocytoses and Rosai-Dorman

CD1a is negative in all except indeterminate cell histocytosis

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32
Q

What is the most common presenting ages for juvenile xanthogranuloma?

A

0-2 (15% at birth and 75% by first year)

33
Q

What is the prognosis of juvenile xanthogranuloma?

A

Spontaneous resolution in 3-6 years

34
Q

What are the most common locations for juvenile xanthogranuloma?

A

Head/neck>upper trunk

35
Q

What is the clinical apperence of juvenile xanthogranuloma?

A

One to few lesions>> numerous/widespread

  • Lesions are pink to red/yellow papules
36
Q

Mucosal involvement in juvenile xanthogranuloma?

A

Can occur rarely

37
Q

Ocular involvement in juvenile xanthogranuloma?

A

0.5% have involvement of unilateral eye (Iris most common) leading to hyphema, glaucoma and can lead to blindness

38
Q

What specialty should be consulted for new dx of juvenile xanthogranuloma?

A

Ophthalmology

39
Q

What are risk factors for ocular involement in juvenile xanthogranuloma?

A

Multiple lesions and children <2yrs

40
Q

What should be watched for if a pt has JXG and NF-1?

A

20x increased risk of JMML

41
Q

What is the histology of juvenile xanthogranuloma?

A

Well-circumscribed, dense dermal infiltrate of foamy lipidized histiocytes. Touton giant cells and eosionphils are seen; loss of rete ridges and can see ulceration

*CD68 +

42
Q

What is the age of onset of benign cephalic histiocytosis?

A

Infants < 1y/o usually

43
Q

What is the presentation of benign cephalic histiocytosis?

A

Numerous (more lesion than typical JXG) red-brown macules and papules of face/neck that may progress of upper torso

Probably a JXG variant

44
Q

What is the course of benign cephalic histiocytosis?

A

Self-limited, no internal or mucosal involvement

45
Q

What is the histology of benign cephalic histiocytosis?

A

Known for intracytoplasmic “comma-shaped/worm-like” bodies on electron microscopy, otherwise very similar to JXG but no Touton giant cells, minimal to no lipidized histiocytosis

46
Q

What is the age of onset for generalized eruptive histiocytosis?

A

Adults

20-50 y/o more than kids

47
Q

What is the clinical presentation of generalized eruptive histiocytosis?

A

Recurrent eruption of hundreds of small (<1cm) red-brown papules in axial distribution (trunk, proximal extremities> face)

Heal with hyperpigmentation

48
Q

What is the prognosis of generalized eruptive histiocytosis?

A

Self limited; no internal or mucosal involement

49
Q

What is the presentation of indeterminate cell histiocytosis?

A

Solitary and generalized variants exist

Eruption clinically and histologically indistiginguishable from benign cephalic histiocytosis and generalized eruptive histiocytosis (requires immunostains to distinguish)

50
Q

Prognosis of indeterminate cell histiocytosis?

A

Rare visceral and bone lesions with occasional fatal cases

51
Q

What stains help distinguish indeterminate cell histiocytosis?

A

S100+, CD1a+, Langerin negative (this is what distinguishes it from LCH)

52
Q

What is the age of presentation most commonly for necrotizing xanthogranuloma?

A

50’s

53
Q

Clinical presentation of necrotizing xanthogranuloma?

A

Destructive multisystem disease

Yellow xanthomatous plaques +/- ulceration

54
Q

What is the most common locations for necrotizing xanthogranuloma?

A

Periorbital>>>other face, trunk, extremities

55
Q

What are other manifestations of necrotizing xanthogranuloma?

A

50% have ophthalmic compliations, can also have hepatosplenomegaly, leukopenia and elevated ESR

56
Q

What gammapathy is associated with necrotizing xanthogranuloma?

A

IgGkappa monoclonal gammopathy (>80%), a/w plasma cell dyscrasia or multiple myeloma

57
Q

Histology of necrotizing xanthogranuloma?

A

Looks like palisading xanthogranuloma with cholesterol clefts

58
Q

What is the age range for reticulohistiocytosis (multicentric reticulohistiocytosis and solitary reticulohistiocytoma)?

A

30-40’s very rare in children

59
Q

What is the clinical presentation of multicentric reticulohistiocytosis?

A

F>M

Red-brown or yellow nodules

Acral sites are favored (head, dorsal hands > elbows)

50% have oral or nasopharyngeal lesions

60
Q

What disease is often seen with multicentric reticulohistiocytosis?

A

Destructive arthritis –> arthritis mutilans in 45%

61
Q

What is the presentation of the solitary form of solitary reticulohistiocytoma?

A

1 site is the head

Solitary, asymptomatic <1cm yellow-red nodule

62
Q

What systemic sx’s are associated with multicentric reticulohistiocytosis?

A

Elevated ESR, fever, anemia

63
Q

What important disease must be checked for in a pt with multicentric reticulohistiocytosis?

A

Solid organ maligancy, present in 30% of cases

64
Q

What is the “coral bead” sign seen in multicentric reticulohistiocytosis?

A

Papules line up along the periungual region

65
Q

What is the histology of multicentric reticulohistiocytosis?

A

Dermal infiltrate of mono- and multi-nucleated histiocytes with granular, pink-purple “ground glass” cytoplasm, often with a lacunae or empty white space

66
Q

What are the 5 main cutaneous articular syndromes?

A

RA, SLE, Sarcoid, multicentric reticulohistiocytosis, EED

67
Q

What is the most common age of onset for Rosai-Dorfman disease?

A

10-30 y/o

68
Q

What is the clinical presentation of Rosai-Dorfman disease?

A

Multisystem dz of children or young audlts; massive but asymptomatic bilateral cervical LAD

Can also have fever/night sweats/wieght loss, elevated ESR, polyclonal hypergammaglobulinemia

69
Q

What skin sites are most common in Rosai-Dorfman disease, and where do they occur?

A

1 sites are the eyelids and the malar cheek

Skin involvement happens in 10%

70
Q

What is the morphology of the cutaneous lesions in Rosai-Dorfman disease?

A

Multiple red-brown or xanthomatous papules/plaques

71
Q

Prognosis of Rosai-Dorfman disease?

A

Usually self-resolves

72
Q

What is the histology of Rosai-Dorfman disease?

A

Pan-dermal infiltrate of very large, very foamy S100+/CD68+ histiocytes with emperipolesis, and abundant plasma cells

73
Q

What is the most common age for xanthoma disseminatum?

A

<25 y/o

74
Q

What is the clinical presentation of xanthoma disseminatum?

A

Triad: cutaneous xanthomas, mucosal xanthomas (oral and upper airway most commonly) and diabetes insipidus

Lesions are red-brown or yellow papules that coalesce into oddly-patterned xanthoma-like plaques (hashtag like patterns)

75
Q

What anatomic areas are most affected by xanthoma disseminatum?

A

Symmetric flexural/intertriginous involvement

76
Q

What things is xanthoma disseminatum associated with?

A

Normal lipid levels

A/w monoclonal gammopathy, plasma cell dyscrasia

77
Q

What is the histology of xanthoma disseminatum?

A

Dense dermal infiltrates of many foam cells and occasional Touton giant cells

78
Q

What is Erdheim-Chester?

A

Fever, bone lesions, diabetes insipidus, exophthalmos, CNS, Multiple internal organs involved. Skin can be involved in 25% of cases, usually eyelids and upper half of body and shows up with red-brown to yellow indurated nodules/plaques

79
Q

What is the prognosis of Erdheim-Chester?

A

Bad, high mortality rate

DERM (07) Disorders of Langerhans Cells and Macrophages (4 decks)

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