28- Dermal Tumors Flashcards

1
Q

This type of vascular anomaly involute spontaneously

A

Infantile hemangioma

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2
Q

This type of vascular anomaly does not involute and are persistent

A

Vascular malformations

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3
Q

Cutaneous vascular anomaly that is characterized by an abnormal arrangement of tissues

A

Hamartomas

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4
Q

Hamartomas are usually contrasted to this condition because it is described as an increase in tissue present but in an β€œnormal” arrangement

A

Nevus

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5
Q

What are the 4 types of phakomatosis pigmentovascularis?

A

Cesioflammea (blue nevus, nevus flammeus)
Spirolosa (nevus spilus, pale pink vascular spot
Cesiomarmorata (blue spots, cutis marmorata)
Unclassifiable

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6
Q

Most common type of phakomatosis pigmentovascularis

A

Cesioflammea

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7
Q

Most commonly associated condition with phakomatosis cesioflammea

A

Sturge-Weber syndrome

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8
Q

Most commonly associated condition with phakomatosis spirulosa

A

Multiple granular cell tumors

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9
Q

Hamartoma that appears as a solitary nodular lesion on acral areas with pain and hyperhidrosis

A

Eccrine angiomatous hamartoma

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10
Q

Eccrine angiomatous hamartoma has been associated with these conditions

A

Spindle cell hemangioma
Verrucous hemangioma
AVM

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11
Q

Treatment for eccrine angiomatous hamartoma

A

Excision because of pain

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12
Q

Abnormal structures that result from an aberration in embryonic development or trauma

May be due to anatomic or functional alteration

A

Malformations

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13
Q

Types of anatomic malformations

A
Capillary
Venous
Arterial
Lymphatic 
Combined
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14
Q

Congenital disorder characterized by various shapes and size macules that are paler than surrounding skin

A

Nevus anemicus

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15
Q

Nevus anemicus resembles this disease but has normal amount of melanin

May occur in neck and trunk

A

Vitiligo

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16
Q

Underlying defect of nevus anemicus

A

Increased sensitivity of blood vessels to catecholamines

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17
Q

Patch of skin that is cooler than normal skin

A

Nevus oligemicus

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18
Q

Underlying defect in nevus oligemicus

A

Decreased blood flow due to vasoconstriction of deep blood vessels

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19
Q

Presence of purplish, reticulated, vascular network with segmental distribution- usually in extremities

A

Cutis marmorata telangiectasia congenita

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20
Q

Mottling is curis marmorata becomes more distinct during

A

Crying
Vigorous activity
Cold

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21
Q

Treatment for cutis marmorata

A

None- lesions usually improve by 2 years old

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22
Q

Differential in cutis marmorata where there is progressive development of large venous ectasias involving one limb

A

Bockenheimer syndrome

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23
Q

Congenital capillary malformation present in 25% of newborns

Pink red macule on posterior midline between occipital protruberance and C5

A

Nevus flammeus nuchae
β€œStork bite”
Port wine stain

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24
Q

Salmon patch type of nevus flammeus is located usually in

A

Glabellar region

One upper eyelid

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25
Q

Congenital capillary malformation that usually fades in childhood

A

Nevus flammeus

Port wine stain

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26
Q

Most common site of nevus flammeus

A

Unilateral distribution on the face

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27
Q

Nevus flammeus found in this area is associated with Sturge Weber syndrome

A

Ophthalmic division ( V1) of CN5

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28
Q

Leptomeningeal angiomatosis has this characteristic feature

A

Calcifications in the outer cortex

β€œTram tracks” that follow brain convolutions

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29
Q

Sturge Weber Syndrome usually results from the persistence of

A

primitive embryonal vascular plexus
-during the 6th fetal week around the cephalic neural tube and facial skin area

Usually regress by 9 weeks

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30
Q

Other syndrome associated with nevus flammeus (port wine stain) that has overgrowth of soft tissue and underlying bone in affected extremity

A

Klippel-Trenaunay- Parkes- Weber

Syndrome

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31
Q

This syndrome associated with nevus flammeus is characterized by port wine malformations

A

Klippel- Trenaunay

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32
Q

This syndrome associated with nevus flammeus is characterized by deep AVM

A

Parkes- Weber

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33
Q

Lesional skin in nevus flammeus demonstrates overgrowth of

A
Vascular endothelial growth factor (VEGF) 
VEGF receptor (VEGF-R2)
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34
Q

Treatment for port wine stain

A

Laser therapy- recurrence common

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35
Q

Best record of safety and efficacy in the treatment of nevus flammeus

A

Flashlamp pulsed dye laser

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36
Q

Present as rounded, bright red or deep purple spongy nodules that occur chiefly on the head and neck and may also involve mucous membranes

Generally asymptomatic

A

Cavernous venous malformation

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37
Q

Syndrome associated with venous malformation:

Dyschondroplasia with hemagiomata
Uneven bone growth, unilateral distribution

A

Maffucci syndrome

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38
Q

Syndrome associated with venous malformation:

Enchondromatosis is present without cutaneous abnormalities

A

Ollier disease

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39
Q

Human enchondromatosis has been associated with this protein and this gene

A

Parathyroid hormone related protein (PTHrP)

Indian hedgehog gene (IHH)

40
Q

PTHrP does this to the proliferating chondrocytes

A

Delays differentiation

41
Q

What is the effect of IHH to chondrocytes?

A

Promotes proliferation

42
Q

Syndrome associated with venous malformation:

Characterized by cutaneous and GI venous malformations (can rupture)

Lesions are bluish with soft nipple like center that can be emptied by pressure

A

Blue rubber bleb nevus syndrome

43
Q

Syndrome associated with venous malformation:

Cutaneous and osseus venous and lymphatic malformations
associated with massive osteolysis β€œdisappearing bones”

A

Gorham’s disease

Gorham’s sign

44
Q

Vascular malformation that presents in adults as a blush purple nodule on trunk or breast

Lobular mass with dilated interconnected vascular channels filled with blood

A

Sinusoidal hemangioma

45
Q

Venous malformation should be distinguished from

A

Glomuvenous malformation

Glomangioma

46
Q

VM or GM?

Painful if compressed

A

GM

47
Q

VM or GM?

Shrinks with pressure

A

VM

48
Q

VM or GM?

Painful in the morning due to congestion

A

VM

49
Q

VM or GM?

Inherited

A

GM

50
Q

VM or GM?

Sporadic

A

VM

51
Q

VM or GM?

Sclerotherapy more effective

A

VM

52
Q

VM or GM?

Extremity involvement more common

A

GM

53
Q

VM or GM?

Isolated blue lesion that may involve muscle

A

VM

54
Q

VM or GM?

Increased pain with menstruation, pregnancy, OCP

A

VM

55
Q

Triad of Klippel- Trenaunay syndrome

A

Nevus flammeus
Venous and lymphatic malformation
Soft tissue hypertrophy of affected extremity

(When you clip the tray- wine stains, malformed Venus, lots of tissues)

56
Q

What condition is seen that warrants KTS diagnosis to be appended to Parkes-Weber?

A

AV fistula

57
Q

Klippel- Trenaunay Syndrome (KTS) is usually seen in what body part

A

Lower limb (95%)

58
Q

Earliest and most common presenting sign in KTS

A

Nevus flammeus that is confined to the skin of an extremity

59
Q

Diagnostics for KTS

A

Color duplex UTZ- venous patency
MRI- muscle and bone
Arteriography- if AV fistula is suspected
Xray- extremities

60
Q

Treatment of KTS

A

Flashlamp pumped pulsed dye laser- nevus flammeus
Varicosities- microfoam sclerosis, thermal ablation or surgical stripping
Edema- elevation diuretics compression stockings

61
Q

Congenital AV fistulas mostly occur on which body part

A

Extremities

62
Q

Congenital AV fistulas is usually a component of this disease

A

Osler-Weber- Rendu

63
Q

Reddish or purple nodules observed in AV fistulas may resemble this

A

Kaposi sarcoma (Stewart- Bluefarb syndrome)

64
Q

Congenital AV fistulas usually occur during what age

A

2nd-3rd decade of life

65
Q

Uncommon congenital AV fistulas of scalp or face

Pulsating mass on skin and may penetrate cranium or

Solitary blue or red papule in mid adult

A

Cirsoid aneurysm (angioma arteriale racemosum)

66
Q

Diagnosis of AV fistulas

A

Plethysmography
Thermography
O2 saturation of venous blood
Arteriography

67
Q

Treatment for AV fistula

A

Traumatic- excision

Congenital- doppler guided sclerotherapy

68
Q

Deep seated vesicle like papules resembling frog spawn at birth

Yellowish and exude a clear liquid

Arranged irregularly in groups but localized to one region

A

Superficial lymphatic malformation

Lymphangioma circumscriptum

69
Q

Superficial lymphatic malformation may also be seen adjacent to this lesion that may represent twin spotting phenomenon

A

Cafe au lait macules

70
Q

Treatment of superficial lymphatic malformation

A

MRI to evaluate extent of deep involvement

CO2 laser- deeper component absent
Pulsed dye, intense pulse light, sclerosants and electrosurgery

71
Q

Circumsribed painless soft tissue masses that occur in neck, axilla or groin

Associated with Turner syndrome and orher aneuploidy syndromes

A

Cystic hygroma

72
Q

This syndrome is characterized by lymphangiomatosis and chylous effusions with osteolytic changes resulting in β€œvanishing bones”

A

Gorham-Stout syndrome

73
Q

This lesion can occur on face and neck
Children: back of hands, forearms, face

Palmar erythema
Also occurs in pregnant and liver disease

A

Spider angioma

74
Q

Spider angiomas occurs in children, pregnant and those with hepatic dysfunction because

A

Elevated estrogen levels in blood

75
Q

Treatment for spider angioma

A

Involute in children and pregnant (upon delivery)

Active therapy- electrodessication of central punctum or laser

76
Q

Small, dark blue slightly elevated blebs that are easily compressed

Face, neck, ears, lips forearms, back of hands

Manifestations of chronic sun damage

A

Venous lakes (phlebectases)

77
Q

Treatment of venous lakes

A
Electrocautery
Laser ablation
Fulguration
Infrared coagulation 
Cryotherapy
78
Q

Capillary aneurysms are clinically indistinguishable from

A

Malignant melanoma

79
Q

Flesh colored solitary lesions that resemble intradermal nevus that may thrombose and grow darker and larger

Surrounded by zone of erythema

A

Capillary aneurysms

80
Q

Fine, linear vessels coursing on the skin surface are collectively called

A

Telangiectasia

81
Q

Cuticular telangiectasias are indicative of

A

Collagen vascular disease ( LE, scleroderma, dermatomyositis(

Tortuous- LE
Dilated- scleroderma, DM

82
Q

Treatment for telangiectasia

A

Electrodessication

Laser ablation- pulse stacking

83
Q

Bleeding disease that affects blood vessels of skin and mucous membranes

Mottled skin with redness that blanch on pressure. Normal blood coagulation

Differs from generalized telangiectasia due to hemorrhagic tendency

A

Universal angiomatosis

84
Q

Fine threadlike telangiectasia in a unilateral or dermatomal distribution

Acquired- increased estrogen

A

Unilateral nevoid telangiectasia

85
Q

Unilateral nevoid telangiectasia is usually found in

A

Trigeminal

C3 and C4 areas

86
Q

Telangiectases that have overlying hyperkeratotic surface

A

Angiokeratomas

87
Q

Also called telangiectatic warts

Dull red or purplish black verrucous, rounded papules usually on dorsum of fingers and toes, elbows and knees

Cold cyanotic hands and feet

A

Angiokeratoma of Mibelli

88
Q

Multiple small vascular papules jn the scrotum or vulva

Diffuse redness of area
Histo: communicating lacunae in subpapillary layer

A

Angiokeratoma of Fordyce

89
Q

Treatment for Fordyce angiokeratoma

A
Reassurance
Laser ablation
Shave excision
Cautery
Fulguration
90
Q

Red brown, dome shaped dermal papules or nodules usually in head and neck ( retroauricular, scalp)

Underlying AV shunt present
Do not spontaneously regress
May recur if AV shunt not excised
Female preponderance

A

Angiolymphoid hyperplasia with eosinophilia (ALHE)

91
Q

ALHE must be differentiated from this disease

A

Kimura disease

92
Q

Inflammatory disease that present as massive subcutaneous swelling in preauricular and submandibular area in Asian men

Associated with allergic conditions

A

Kimura disease

93
Q

Small, eruptive solitary sessile/pedunculated friable papule often seen in exposed surface and on gingiva

Bleed easily on slightest trauma
Secondary staph colonization common

A

Pyogenic granuloma

94
Q

Treatment of pyogenic granuloma

A

Curettage or shave excision

Fulguration/ silver nitrate- destroy base

95
Q

Minute, copper colored to bright red angiomatous puncta that tend to become papular

Occur in groups, forms new points in periphery while center fades
No purpura- netlike/diffuse erythema
Mostly in LE
Involution never complete

A

Angioma serpiginosum

96
Q

Angioma serpiginosum must be differentiated from

A

Progressive pigmentary disease of Schamberg

- pinpoint areas if purpura (cayenne pepper spots)

97
Q

Most important histo finding in angioma serpiginosum

A

Dilated and tortuous capillaries in dermal papillae

No inflammatory infiltrate or extravasation of RBC