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OMF Pathology > 16 Dermatologic Diseases > Flashcards

16 Dermatologic Diseases Flashcards

1
Q

What is the best known form of ectodermal dyslpasia?

A

Hypohidrotic Ectodermal Dysplasia

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2
Q

What are distinguishing traits of people with Hypohidrotic ectodermal dysplasia?

A
  • fine, sparse hair
  • brittle nails
  • hypoplastic/absent salivary glands
  • abnormal dental crowns
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3
Q

Patient also has fine hair and high heat intolerance. What do they likely have?

A

Hypohidrotic ectodermal dysplasia

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4
Q

Hypohidrotic E.D. has a predeominance for which sex, and is linked with which sex chromosome?

A

Male predominance, X-linked

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5
Q

What defect in keratins presents as white, corrugated plaques bilaterally on buccal mucosa?

A

White sponge nevus

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6
Q

what is a genetically determined skin disorder?

A

Genodermatosis

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7
Q

White sponge nevus is recessive or dominant?

A

Autosomal dominant

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8
Q

What does this suggest? (hint: bilateral presentation)

A

white sponge nevus

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9
Q

When did these lesions most likely first appear? (hint: presents bilaterally and autosomal dominant)

A

birth or early childhood

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10
Q

What presents intraorally as a white lesion with a warty or roughened surface?

A

Warty Dyskeratoma

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11
Q

Warty dyskeratomas histopathologically identical to what disease?

A

Darier disease

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12
Q

Pt is over 40 and presents with this asymptomatic, rough-surfaced lesion on keratinized mucosa. What is it?

A

Warty Dyskeratoma

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13
Q

Peutz-Jeghers Syndrome is characterized by what?

A
  1. freckle-like lesions on skin around hands, mouth, and intraorally
  2. intestinal polyposis
  3. predisposition to develop cancer
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14
Q

What is Peutz-Jegher’s inherited as?

A

Autosomal dominant

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15
Q

What is unique about the freckles seen with Peutz-Jegher’s Syndrome?

A

They don’t change darkness with sun exposure like regular freckles

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16
Q

Peutz-Jegher’s patients are __ times more likely to develop cancer in their lifetime.

A

18 times more likely

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17
Q

Why do Peutz-Jegher’s patients commonly experience intestinal obstruction?

A

Intussusception, which is telescoping of the proximal bowel into the distal portion

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18
Q

If Peutz-Jegher’s Syndrome, what else might they present with?

A

Perioral and intraoral freckling

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19
Q

Also has intestinal polyps. What do they have?

A

Peutz-Jeghers Syndrome

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20
Q

What are some characteristics of tuberous sclerosis?

A
  • mental retardation
  • potato-like growths (tubers)
  • epilepsy
  • shagreen patches
  • ash-leaf spots
  • angiofibromas of skin
  • ungual fibromas
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21
Q

A patient with tuberous sclerosis might show facial angiofibromas where on the face?

A

nasolabial fold area

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22
Q

What is a shagreen spot?

A

A connective tissue hamartoma (benign neoplasm that grows at same rate as tissue around it)

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23
Q

What is an ash-leaf spot?

A

an ovoid area of hypopigmentation

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24
Q

What heart condition is associated with tuberous sclerosis?

A

Rhabdomyoma

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25
Q

What are these facial angiofibromas characteristic of?

A

tuberous sclerosis

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26
Q

What is this called? What pathology is it a characteristic of?

A
  1. ash-leaf spot
  2. tuberous sclerosis
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27
Q

What are two oral manifestations seen with tuberous sclerosis?

A
  1. facial enamel pitting of permant anterior teeth
  2. multiple fibrous papules
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28
Q

What are four types of Epidermolysis Bullosa?

A
  1. simplex
  2. junctional
  3. dystrophic
  4. hemidesmosomal
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29
Q

What are four things to know about simplex-type epidermolysis bullosa?

A
  • blistering of hands and feet
  • mucosal involvement uncommon
  • no scarring
  • good prognosis
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30
Q

Junctional-type epidermolysis bullosa is characterized by?

A
  1. severe blistering at birth
  2. granulation tissue around mouth
  3. oral involvement common
  4. often fatal
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31
Q

What form of dystrophic epidermolysis bullosa is not typically life threatening?

A

Dominant form

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32
Q

What form of dystrophic epidermolysis bullosa is severely debilitating?

A

Recessive form

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33
Q

Describe dominant form of dystrophic epidermolysis bullosa.

A
  • blistering and scarring in areas of chronic trauma
  • mild oral manifestations
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34
Q

Describe recessive form of dystrophic epidermolysis bullosa.

A
  • blisters and scarring in areas of minor trauma
  • secondary infections of blisters
  • hands scarred into “mittens”
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35
Q

What type of epidermolysis bullosa is this? (hint: good prognosis, no scarring)

A

simplex

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36
Q

What is the most common pemphigus-related disease?

A

Pemphigus vulgaris

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37
Q

Oral lesions of pemphigus vulgaris are described with what clever phrase?

A

“first to show, last to go”

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38
Q

In pemphigus vulgaris, autoantibodies are directed toward what cellular protein?

A

desmosomes

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39
Q

How is the histological presentation of epithelium described in pemphigus vulgaris?

A

As intraepithelial split

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40
Q

What is a characteristic feature of pemphigus vulgaris where bulla apear on normal-appearing skin with firm pressure?

A

Positive Nikolsky Sign

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41
Q

Give two histological characteristics of pemphigus vulgaris.

A
  1. acantholysis (intraepithelial split)
  2. Tzanck cells
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42
Q

Epithelium just falls off due to acantholysis. What is this?

A

pemphigus vulgaris

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43
Q

Tzanck cells are seen on histology. What is this?

A

Pemphigus vulgaris

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44
Q

Patient has positive Nikolsky sign. What is this?

A

Pemphigus vulgaris

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45
Q

These lesions are “first to show, last to go”. What is this?

A

Pemphigus vulgaris

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46
Q

This is pemphigus vulgaris. Describe what is happening here.

A

Autoantibodies are attacking desmosomes of epithelial cells, causing “intraepithelial split”

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47
Q

Intraoral blood blister are virtually pathognomonic for what disease?

A

Mucous membrane pemphigoid

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48
Q

Unlike with pemphigus, what might be seen intraorally with pemphigoid?

A

blisters

49
Q

What happens to the eyes in mucous membrane pemphigoid?

A

Entropion (eyelids turn inward) and keratin deposition on the cornea; both leading to blindness

50
Q

What is it called in mucous membrane pemphigoid causes adhesion of the eyelid to the eye?

A

Symblepharons

51
Q

What is this called?

A

symblepharon

52
Q

What is this called? What disease is it seen with?

A
  1. Entropion
  2. mucous membrane pemphigoid
53
Q

In pemphigoid, autoantibodies are attacking what?

A

the basement membrane (causing interepithelial split)

54
Q

Seen with pemphigoid, what is this showing?

A

Autoantibodies attacking the basement membrane

55
Q

A virtually pathognomonic sign for what disease?

A

mucous membrane pemphigoid

56
Q

Patient shows intraoral blisters and symblepharons. What is this?

A

mucous membrane pemphigoid

57
Q

What are two common infections seen with erythema multiforme?

A

Herpes simplex, and Mycoplasma pneumoniae

58
Q

erythema multiforme major is also known as what?

A

Stevens-Johnson Syndrome

59
Q

What is a highly characteristic skin lesion that develops in erythema multiforme?

A

Target lesions

60
Q

T or F: Erythema multiforme presents with prodrome symptoms 7 days before onset.

A

True. Prodrome (fever, headache, cough, sore throat) is usually about a week.

61
Q

What intraoral surfaces are involved in erythema multiforme?

A

All surfaces except gingiva and hard palate

62
Q

Also has target lesions on body. What is this diagnosis?

A

Erythema multiforme

63
Q

What is this?

A

target lesions (seen with erythema multiforme)

64
Q

What is the most severe form of erythema multiforme that occurs in older people and is almost always triggered by a drug?

A

Toxic epidermal necrolysis

65
Q

To diagnose Stevens-Johnson Syndrome, what criteria must be met?

A

skin/oral mucosal involvement

+

ocular/genital mucosal involvement

66
Q

What are two other names for erythema migrans?

A
  1. geographic tongue
  2. benign migratory glossitis
67
Q

Where intraorally does erythema migrans present?

A

anterior 2/3 of tongue

68
Q

Describe the lesions of erythema migrans.

A

Irregularly shaped zones of erythema with a raised, yellow to white border

69
Q

Erythema migrans is associated with what other condition?

A

fissured tongue

70
Q

Pt complains of burning when they eat spicy foods. What is this?

A

Benign migratory glossitis

71
Q

What is this?

A

Erythema migrans (geographic tongue)

72
Q

Skin lesions of lichen planus present with what characteristics?

A
  • purple
  • pruritic
  • polygonal
  • papules
73
Q

Skin papules of lichen planus also present with what?

A

Wickham’s striae

74
Q

Describe reticular lichen planus.

A

net or web-like

75
Q

describe erosive lichen planus

A

ulcerative

76
Q

Which type of lichen planus is more common, asyptomatic, and involves posterior buccal mucosa as wickham striae?

A

Reticular lichen planus

77
Q

Which type of lichen planus is symptomatic, and shows erythema with central ulceration?

A

Erosive lichen planus

78
Q

What is this?

A

reticular lichen planus

79
Q

What is this?

A

Reticular lichen planus

80
Q

What kind of lichen planus is this?

A

erosive

81
Q

What kind of lichen planus can cause this?

A

erosive

82
Q

Drug-induced lichen planus lesions are typically found on the _____ ____ as non-healing ulcers.

A

lower lip

83
Q

What three histologic features are seen in lichen planus?

A
  1. saw-tooth rete pegs
  2. Civatte bodies
  3. fibrinogen deposition in basement membrane
84
Q

28% of women with ELP have ______ ______.

A

vaginal lesions

85
Q

Lesions can be induced with what four things? (hint: STAY)

A

S - stress

T- trauma

A - advil (any NSAID though)

Y - yeast

86
Q

What can occur in recipients of allogeneic bone marrow transplantations?

A

Graft Versus Host Disease (GVHD)

87
Q

What happens in GVHD?

A

The grafted cells start attacking the host tissues because they’re freaked out they’re not in their own home.

88
Q

What type of GVHD is usually seen within a month of procedure, but can be any time before 100 days?

A

Acute GVHD

89
Q

What kind of GVHD either presents after 100 days from the procedure or is a continuation of acute GVHD?

A

chronic GVHD

90
Q

Acute GVHD can mimic what other disease?

A

Toxic Epidermal Necrolysis

91
Q

Chronic GVHD can mimic what two other diseases?

A

Systemic Lupus Erythematosis or Sjogren’s Syndrome

92
Q

Acute GVHD develops in __% of patients, and chronic develops in __%.

A

acute = 50%

chronic = 70%

93
Q

What is the most common collagen vascular or connective tissue disease in the US?

A

Lupus Erythematous

94
Q

What sex has a higher incidence for systemic lupus erythematous?

A

Females 10:1 compared to males

95
Q

What two heart conditions can affect SLE patients?

A

Pericarditis (most common)

Libman-Sacks Endocarditis (warty vegetations on valves)

96
Q

Based on the characteristic Malar rash on this patient’s face, what does she have?

A

systemic lupus erythematosus

97
Q

Intraoral lichenoid lesions are seen with what systemic disease?

A

systemic lupus erythematosus

98
Q

Patient also has fatigue, arthritis, and malar rash. What is your diagnosis?

A

systemic lupus erythematosus

99
Q

What is the most common cause of death in patients with SLE?

A

Renal failure

100
Q

What presents with Raynaud’s phenomenon, nasal alar atrophy, and clawlike fingers?

A

Systemic sclerosis

101
Q

In systemic sclerosis, most organ systems have excess fibrin deposition in them. What is the main cause of death in patients with Systemic Sclerosis?

A

Heart failure due to Pulmonary HTN (from fibrosis)

102
Q

Since fibrosis occurs across the whole body in systemic sclerosis, would you expect to see a patient have microstomia?

A

Yes, because there will be fibrosis of perioral tissues

103
Q

Related to systemic slcerosis, what is it called if theres fibrosis to only a localized patch of skin?

A

Localized scleroderma

104
Q

En coupe de sabre (“strike of the sword”) is characteristic of waht disease?

A

Localized scleroderma

105
Q

What does CREST stand for?

A

C – Calcinosis cutis

R – Raynaud’s phenomenon

E – Esophageal dysfunction

S – Sclerodactyly

T – Telangiectasia

106
Q

What are multiple, movable, nontender, subcutaneous, nodular calcifications called? (hint: the C in crest)

A

Calcinosis Cutis

107
Q

This blanching is called what? (hint: happens due to stress or cold)

A

Raynaud’s phenomenon

108
Q

What causes esophageal dysfunction in CREST syndrome?

A

abnormal collagen deposition in esophageal submucosa

109
Q

What is it called when fingers undergo permanent flexure, and skin becomes smooth and shiny?

A

Sclerodactyly

110
Q

What is this condition called? What two diseases is it seen with?

A
  1. Sclerodactyly
  2. Systemic sclerosis
  3. CREST Syndrome
111
Q

What is the name for numerous scattered red papules most frequently found on the vermilion zone of lips & facial skin?

A

Telangiectasia

112
Q

Do telangiectasias blanch?

A

Yes, it indicates the red color is due to blood contained within blood vessels

113
Q

What is this? (hint: it is the T in CREST)

A

telangiectasia

114
Q

What disease is characterized by the development of a velvety, brownish alteration of the skin?

A

Acanthosis Nigricans

115
Q

Benign acanthosis nigricans caused by obesity is termed what?

A

Pseudoacanthosis Nigricans

116
Q

Acanthosis nigricans is seen in conjuction with what pathology?

A

GI cancer

117
Q

What is this?

A

Acanthosis Nigricans

118
Q

What can this be in conjunction with?

A

GI cancer (pt shows acanthosis nigricans)

OMF Pathology (21 decks)

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