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fitzpatrick's dermatology 9th edition (shammy) > 20 - 108 - BENIGN EPITHELIAL TUMORS, HAMARTOMAS, AND HYPERPLASIAS > Flashcards

20 - 108 - BENIGN EPITHELIAL TUMORS, HAMARTOMAS, AND HYPERPLASIAS Flashcards

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Dermatology Board Prep flashcards
1
Q

Hallmark histopathologic findings of seborrheic keratosis

A

acanthosis, papillomatosis, pseudo-horn cysts, and hyperkeratosis

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2
Q

most frequently detected mutations in seborrheic keratosis

A

The most frequently detected are activating mutations in the FGFR3 and PIK3CA genes.

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3
Q

Which of the following is true about lichen striatus?

a. It occurs most commonly on the trunk of children and adolescents.
b. The eruption is self-limited, with spontaneous regression within a few months to 2 years.
c. Histopathologically, it spares the eccrine coils
d. There is male predilection

A

B

A -Limb,

C. There is characteristic accentuation around eccrine coils,

D. There is female predilection

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4
Q

Which is the following are true about nevus sebaceous except?

a. Cases are typically sporadic.
b. More than 90% are believed to harbor an HRAS mutation.
c. These lesions can be verrucous at birth, later becoming more subtle under the hormonal influences of puberty.
d. Nevus sebaceous syndrome is also called Schimmelpenning syndrome.

A

C

Page 1809. These lesions can be subtle at birth, later becoming more elevated and verrucous under the hormonal influences of puberty.

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5
Q

What is the most common tumor found within a nevus sebaceous?

A

Trichoblastoma

Page 1810. The most common are trichoblastoma (7% of nevus sebaceous cases) and syringocystadenoma papilliferum (5%).

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6
Q

Which of the following syndromes is associated with ILVEN?

a. Proteus Syndrome (AKT1 mutation)
b. Type 2 Segmental Cowden Disease (PTEN mutation)
c. CHILD mutation (NSHDL mutation)
d. Schimmelpening syndrome (HRAS/KRAS mutation)

A

c. CHILD mutation (NSHDL mutation)

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7
Q

Syndromes associated with Keratinocytic epidermal nevi (nonepidermolytic)

A
  • HRAS/FGFR3 mutation
  • Proteus syndrome (AKT1 mutation)
  • Type 2 segmental Cowden disease (PTEN mutation)
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8
Q

Syndrome associated with ILVEN

A

CHILD syndrome (NSDHL mutation)

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9
Q

Syndromes associated with Nevus sebaceous

A
  • Schimmelpenning syndrome (HRAS/KRAS mutation)
  • Phakomatosis pigmentokeratotica (HRAS mutation)
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10
Q

Syndrome associated with Nevus comedonicus

A

Nevus comedonicus syndrome: ocular (eg, ipsilateral cataract, corneal erosion), skeletal, and CNS anomalies

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11
Q

Epidermoid cysts are lined by epithelium resembling the ____________

A

infundibulum of the hair follicle

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12
Q

Trichilemmal cysts arise from the _________

A

Outer root sheath

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13
Q

True on the histopathology of steatocystoma multiplex except

a. absent granular layer
b. eosinophilic cuticle on the luminal side
c. sebaceous glands are found within the cyst lumen
d. composed of stratified, squamous epithelium

A

c. sebaceous glands are found within the cyst lumen

Page 1816. Sebaceous glands are characteristically located in the cyst wall, and keratin, oil, and hairs are typically found in the lumen.

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14
Q

Branchial cysts located on the angle of the mandible arise from _______

A

first branchial cleft

angle of the mandible = first branchial cleft;

middle to lower third of the anterior border of the sternocleidomastoid = second branchial cleft

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15
Q

most common acquired benign epidermal tumor of the skin

A

SEBORRHEIC KERATOSIS

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16
Q
A

Seborrheic keratosis showing a rough surface and stuck-on appearance.

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17
Q
A
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18
Q
A
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19
Q
A
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20
Q
A

Dermatosis papulosa nigra.

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21
Q

drug associated with “pseudo-sign of Leser-Trélat”

A

Chemotherapeutic agents, such as cytarabine

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22
Q

most common associated malignancy in Leser-Trélat sign

A

The most common associated malignancy is gastrointestinal, followed by lymphoproliferative disease.

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23
Q
A

Figure 108-8 Lichen striatus. Linear configuration of slightly scaling papules in a blaschkoid distribution on the leg.

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24
Q
A
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25
Q

Solitary, shiny, erythematous or orange to brown, blanching, well-demarcated papule or nodule with collarette of scale

A

Clear cell acanthoma presents as a solitary, shiny, erythematous to brown, well-demarcated papule or nodule that blanches almost fully with pressure

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26
Q

Clear cell acanthoma is composed of distinctive, _______-rich keratinocytes

A

glycogen-rich​

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27
Q
A
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28
Q

chronic tender nodule that develops most commonly on the helix of the ear in men older than 50 years of age

A

CHRONDRODERMATITIS NODULARIS HELICIS

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29
Q

■ Solitary focus of acantholytic dyskeratosis

■ Skin-colored, umbilicated papule on the head or neck of middle-aged and older individuals

A

WARTY DYSKERATOMA

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30
Q
A

ACANTHOMA FISSURATUM (GRANULOMA FISSURATUM)

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31
Q

benign hamartomatous proliferations of the epithelium and adnexae that present at birth or early childhood.

A

Epidermal nevi

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32
Q

The most common variant of epidermal nevi, usually presenting at birth or before adolescence

A

KERATINOCYTIC EPIDERMAL NEVUS

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33
Q
A
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34
Q

unilateral subtype of systematized Keratinocytic epidermal nevus

A

nevus unius lateris

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35
Q

bilateral subtype of Keratinocytic epidermal nevus

A

ichthyosis hystrix

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36
Q

variant of keratinocytic epidermal nevus that clinically appears psoriasiform (erythematous with thick scale) and can be very pruritic

A
37
Q
A
38
Q
A

NEVUS SEBACEOUS

39
Q
A
40
Q
A
41
Q
A
42
Q

term collectively describing several distinct syndromes that all demonstrate an epidermal nevus occurring in the context of other developmental abnormalities

A

EPIDERMAL NEVUS SYNDROME

43
Q

The most common extracutaneous features of epidermal nevus syndrome involve what organs

A

ocular, neurologic, and skeletal systems

44
Q
A
45
Q

derived from the infundibular portion of hair follicle and thus show flattened surface epithelium and keratohyaline granules

A

Epidermoid cysts

46
Q

ining resembles the isthmic portion of hair follicle and thus show a scalloped surface epithelium lacking a granular layer

A

Trichilemmal cyst (Pilar Cyst)

47
Q

thought to be the result of plugging of a vellus hair follicle or eccrine sweat duct; resemble small epidermal cysts

A

Milia

48
Q

a sebaceous duct cyst characterized by a waxy, eosinophilic cuticle lining the cyst and characteristic sebaceous glands in the cyst walls

A

Steatocystoma

49
Q

: collections of epidermis located along embryologic fusion planes, most commonly on the forehead, lateral eye, or neck

A

Dermoid cysts

50
Q

asymptomatic cysts caused by occlusion of branchial cleft sinuses that are located along the angle of the mandible if arising from the first branchial cleft and the middle to lower third of the anterior border of the sternocleidomastoid in cases arising from the second branchial cleft

A

Branchial cysts

51
Q

epithelial invaginations located in the preauricular area that arise from the incomplete fusion of the first and second branchial arches in the preauricular area

A

Preauricular cysts or sinuses

52
Q

What HPV subtypes have been detected in palmoplantar epidermoid cysts

A

HPV types 57 and 60 DNA

53
Q

milia on the palate

A

Epstein pearls

54
Q

Steacystoma can be found in what syndromes

A

Alagille syndrome and pachyonychia congenita type II

55
Q

Eruptive lesions of seborrheic keratosis may be a sign of internal malignancy , what is this sign called?

A

Leser-Trélat sign

56
Q

hallmark histologic findings of seborrheic keratosis

A
  • hyperkeratosis
  • acanthosis
  • papillomatosis
  • pseudohorn cyst
57
Q

This medication may cause inflammation of preexisting SKs, which then become more clinically apparent (“pseudo-sign of Leser-Trélat”).

A

Chemotherapeutic agents, such as cytarabine

58
Q

The most frequently detected Genetic Alteration Reported in Association with Seborrheic Keratosis

A

FGFR3 and PIK3CA

59
Q

site of predilection of stucco keratosis

A

distal extremities, particularly the lower legs

60
Q

multiple small 1- to 3-mm white to tan keratotic papules with predilection for the distal extremities, particularly the lower legs

A

STUCCO KERATOSIS

61
Q

histologic term used to describe intraepidermal whorls of bland keratinocytes

A

CLONAL SEBORRHEIC KERATOSIS

62
Q

Irritated seborrheic keratosis with surrounding eczematous changes is called?

A

Meyerson phenomenon

63
Q

rare, idiopathic, papular eruption that usually resolves in 1 to 2 years

A

Lichen Striatus

The cause is unknown, and the disease is self-limiting.

64
Q

lichen striatus usually occurs in what population and gender?

A

It occurs most commonly on the limbs of children and adolescents (5 - 15 years old) with a female predilection.

65
Q

lichen striatus has been associated with what condition?

A

An association with atopic dermatitis has also been suggested

66
Q

refers to a more extensively distributed variant of keratinocytic epidermal nevi

A

systematized epidermal nevus

67
Q

More than 90% of nevus sebaceus are believed to harbor an what mutation

A

HRAS mutation

68
Q

nevus sebaceous syndrome AKA

A

Schimmelpenning syndrome

69
Q

most common benign neoplasm associated with nevus sebaceus

A

Trichoblastoma

70
Q

most common malignant neoplasm associated with nevus sebaceus

A

Basal cell carcinoma

71
Q

Nevus comedonicus syndrome is the association of nevus comedonicus with noncutaneous findings such as

A
  • skeletal defects,
  • cerebral abnormalities, and
  • cataracts
72
Q

a potential regulator of follicular homeostasis, has been postulated to be defective in nevus comedonicus

A

NEK9 Kinase

73
Q

characterized by a segmental smooth muscle hamartoma in association with musculoskeletal defects such as ipsilateral breast hypoplasia

A

Becker nevus syndrome

74
Q

consists of an epidermal hamartoma with long, fine white hair in addition to ocular, neurologic, and skeletal deficiencies

A

Angora hair nevus syndrome

75
Q

ILVEN is associated with what syndrome

A

CHILD syndrome (NSDHL mutation): ipsilateral bone, visceral, and neurologic defects; epidermal nevi lateralized with flexural affinity

76
Q

Nevus sebaceous is associated with what syndromes

A

Schimmelpenning syndrome (HRAS/KRAS mutation): ocular (eg, lipodermoid, coloboma), cerebral, and skeletal anomalies

Phakomatosis pigmentokeratotica (HRAS mutation): CNS anomalies; speckled lentiginous nevi and epidermal nevi following checkerboard pattern

77
Q

Keratinocytic epidermal nevi (nonepidermolytic) is associated with what syndromes?

A

Proteus syndrome (AKT1 mutation): palmoplantar cerebriform hyperplasia, asymmetrical macrodactyly, soft tissue overgrowth; epidermal nevi characteristically soft and flat

Type 2 segmental Cowden disease (PTEN mutation): focal segmental glomerulosclerosis, soft tissue overgrowth, colon polyps; epidermal nevi characteristically thick and papillomatous (also called linear Cowden nevi)

78
Q
A
79
Q

branchial cyst located along the angle of the mandible arises from the?

A

first branchial cleft

80
Q

branchial cyst located on the middle to lower third of the anterior border of the sternocleidomastoid arises from the?

A

second branchial cleft

81
Q

branchial cyst arising from the first branchial cleft will be located where?

A

along the angle of the mandible

82
Q

branchial cyst arising from the second branchial cleft will be located where?

A

middle to lower third of the anterior border of the sternocleidomastoid

83
Q

What HPV types have been detected in palmoplantar epidermoid cysts?

A

HPV types 57 and 60 DNA

84
Q

These genetic disorders may predispose individuals to having epidermoid cyst.

A
  • Gorlin syndrome (nevoid BCC syndrome)
  • Pachyonychia congenita type 2 (Jackson-Lawler type),
  • Gardner syndrome
85
Q

Arise from the epithelium located between the orifice of the sebaceous gland and the arrector pili muscle. This squamous epithelium undergoes rapid keratinization, resulting in a cyst wall without a granular layer.

A

TRICHILEMMAL CYST (PILAR CYST)

86
Q

Trichilemmal cysts arise from what part of the hair follicle ?

A

outer root sheath

87
Q

plaque-type, inflammatory variant of milia that is commonly located on the ear

A

Milia en plaque

88
Q

Steatocystoma multiplex if familial, is inherited in what mode of inheritance?

A

Autosomal Dominant

89
Q

epithelial-lined cysts containing various appendageal structures resulting from retained epithelium along embryonic fusion planes. These cysts are either congenital or develop in childhood

A

DERMOID CYST

fitzpatrick's dermatology 9th edition (shammy) (122 decks)

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