Chapter 16 Flashcards

1
Q

What is the inherited condition in which two or more ectodermal derived structures fail to develop?

A

Ectodermal Dysplasia.

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2
Q

What is the best known form of ectodermal dysplasia and how does it present?

A

Hypohidrotic: X-linked male predominance, heat intolerance, fine sparse hair, periocular wrinkling with hyperpigmentation, brittle nails, absent salivary glands, missing teeth.

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3
Q

What is the likely dx?

A

White sponge nevus.

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4
Q

What is the defect seen in white sponge nevus?

A

Defect in keratins 4 and 13.

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5
Q

What is the likely Dx?

A

Warty dyskeratoma.

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6
Q

What is the likely Dx?

A

Peutz-Jeghers Syndrome.

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7
Q

What is the likely Dx?

A

Peutz-Jeghers syndrome.

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8
Q

What is a system complex characterized by mental retardation, seizure disorders, potato-like growths called tubers, and angiofibromas of the skin?

A

Tuberous sclerosis.

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9
Q

What are the characteristic skin lesions seen in tuberous sclerosis?

A

Shagreen patches—> Connective tissue hamartoma.

Ash-leaf spots—> Reverse pigment of cafe au let.

Facial Angiofibromas—> Multiple, smooth-surfaced papules occuring primarily on the nasolabial flod area.

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10
Q

What is the likely Dx?

A

Tuberous sclerosis.

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11
Q

What attachement disfunction is seen in pemphigus vs pemphigoid?

A

Pemphigus destroys desmosomes while pemphigoid destroys hemidesmosomes.

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12
Q

What are the 4 diseases of pemphigus?

A

Vulglaris, vegetans, erythematosus, and foliaceus.

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13
Q

What is the first sign of a pemphigus disease?

A

Oral lesions. “First to show and last to go.”

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14
Q

What affect does pemphigus have on the epithelium?

A

There is an intraepithelial split due to the loss of desmosomes.

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15
Q

What is a positive Nikolsky sign and what disease does it present in?

A

It is when a bulla can be induced on normal-appearing skin when firm lateral pressure is placed on the skin and pressure is exerted.

It is seen in pemphigus.

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16
Q

What term does acantholysis refer to and what disease is it seen in?

A

When cells of the epithelium “fall apart.”

Seen in pemphigus.

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17
Q

What disease will present with Tzank cells?

A

Pemphigus and herpes.

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18
Q

What is the purpose of direct and indirect immunoflourescence in pemphigus?

A

Direct will be positive for C3 and IgG within the epithelium while Indirect correlates with disease activity.

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19
Q

What is the likely Dx?

A

Pemphigus.

20
Q

What is the likely Dx?

A

Pemphigus.

21
Q

What is pathognomonic of mucous membrane pemphigoid?

A

An intraoral blood blister.

22
Q

What is the most significant complication of mucous membrane pemphigoid?

A

Ocular involvement.

23
Q

What are the adhesions that form in mucous membrane pemphigoid and what are their significance?

A

Symblepharons which can cause scarring of the eyelid leading to entropion (inward turning).

24
Q

What is the likely Dx?

A

Symblepharon of mucous membrane pemphigoid.

25
Q

What is the term for a blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis?

A

Erythema multiforme.

26
Q

What are the likely infections to cause erythema multiforme?

A

Herpes simplex and Mycoplasma pneumoniae.

27
Q

What is another name for erythema mulitforme major?

What are the characteristics of it?

A

Steven-Johnson syndrome.

Usually triggered by a drug, for this Dx there must be skin, oral mucosa, and either ocular or genital mucosa involvement.

28
Q

What is the likely Dx?

A

Erythema Multiforme.

29
Q

What is the term for a common, chronic dermatologic disease that can affect the oral mucosa?

A

Lichen Planus.

30
Q

How are the skin lesions of lichen planus described?

A

purple, pruritus, polygonal, and papules.

31
Q

What are the names of the thin, lacelike network of white lines seen in lichen planus>

A

Wickham’s striae

32
Q

What are the two forms of oral lesions in lichen planus and describe them.

A

Reticular: Much more common and asymptomatic.

Erosive: symptomatic

33
Q

What are some of the things that can induce Lichen Planus?

A

Stress
Trauma
Advil
Yeast

34
Q

What is the likely Dx?

A

Lichen Planus.

35
Q

What is the likely Dx?

A

Lichen Planus.

36
Q

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

A

Lupus erythematous.

37
Q

What are some of the common findings with systemic lupus erythmetous?

A

Fever, weight loss, arthritis, fatigue, malar rash.

38
Q

What is the characteristic rash of systemic lupus erythematous?

A

Malar rash: butterfly rash that develops in the malar area.

39
Q

What is the most signifcant factor of systemic lupus erythematous?

A

The complication may lead to kidney failure.

40
Q

What is the disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts?

A

systemic sclerosis.

41
Q

What is the first sign of systemic sclerosis?

A

Raynaud’s phenomenon: vasoconstrictive event triggered by emotional stress or exposure to cold.

42
Q

What is the mild variant of systemic sclerosis and describe it.

A

Termed localized scleroderma.

The lesions look like scars described as en coup de sabre.

43
Q

What is the likely Dx?

A

En coup de sabre from localized scleroderma.

44
Q

What are the characteristics of CREST syndrome?

A

Calcinosis cutis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, Telandiectasia.

45
Q

What is raynaud’s phenomenon?

What disease is it seen in?

A

When the extremeties are exposed to cold temps leading to blanching due to vasospasm and then turning blue due to venous stasis.

CREST syndrome and systemic sclerosis.

46
Q

What is telangiectasia and what disease is it associated with?

A

It is the numerous red papules from blood being trapped in capillaries close to the mucosal surface. It is seen in CREST syndrome.

47
Q

What is the likely Dx?

A

Telangiectasia of CREST syndrome