Chapter 16: Dermatologic Diseases II

Question 1

mucous membrane pemphigoid may also be termed ___, and is twice as common as ___

Answer 1

• cicatricial pemphigoid (cicatrix means “scar”)
• twice as common as pemphigus

Question 2

what is the average age and gender predilection for someone with mucous membrane pemphigoid?

Answer 2

• 55
• F:M 2:1

Question 3

mucous membrane pemphigoid oral lesions begin as ___ or ___ which eventually rupture and leave ___

Answer 3

• vesicles or bullae
• ulcerated mucosa

Question 4

describe the oral lesions in mucous membrane pemphigoid

Answer 4

• intraoral blisters may be seen clinically (unlike with pemphigus)
  
  • an intraoral blood blister is virtually pathognomonic
• unlike other lesions in this disease, oral lesions usually don’t scar

Question 5

what is the most significant complication of mucous membrane pemphigoid?

Answer 5

ocular involvement (up to 25%)

Question 6

the earliest ocular change in mucous membrane pemphigoid can be detected via ___ examination by an ophthalmologist

Answer 6

slit-lamp

Question 7

as mucous membrane pemphigoid progresses, what can happen with the eyes?

Answer 7

• conjunctiva become inflamed and eroded, and attempts at healing lead to scarring
• adhesions, called symblepharons, result

Question 8

describe symblepharons associated with mucous membrane pemphigoid

Answer 8

• scarring can turn the eyelids inward (entropion), which causes the eyelashes to rub against the cornea
• scarring can close opening of lacrimal glands
• after all this happens, the cornea produces keratin as a protective mechanism
  
  • keratin is opaque, so this leads to blindness
• blindness can also occur by the upper and lower eyelids scarring together

Question 9

what are the histopathologic features of mucous membrane pemphigoid?

Answer 9

autoantibodies are directed against the basement membrane, leading to a subepithelial split (interepithelial)

Question 10

direct immunofluorescence of mucous membrane memphigoid shows ___ and ___ along the basement membrane

Answer 10

C3 and IgG

Question 11

what is the treatment for mucous membrane pemphigoid?

Answer 11

• first part of treatment is to refer to an ophthalmologist, regardless of if the patient has symptoms
• topical corticosteroids are used first; if they are unsuccessful, systemic agents may be used

Question 12

Answer 12

mucous membrane pemphigoid

Question 13

___ is a blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis, which is likely immunologically mediated

Answer 13

erythema multiforme

Question 14

in 50% of erythema multiforme cases, the clinician can identify a preceding infection or exposure to a medication. what is the infection? what are the medications?

Answer 14

• infection is usually herpes simplex or mycoplasma pneumoniae
• medications are usually antibiotics or analgesics

Question 15

describe the disease spectrum of erythema multiforme

Answer 15

• erythema multiforme minor
• erythema multiforme major - aka stevens-johnson syndrome
• toxic epidermal necrolysis - aka lyells disease

Question 16

is the onset of erythema multiforme acute or chronic? describe mild vs severe cases

Answer 16

• acute
• in mild cases, ulcerations affecting the oral mucosa develop
• in severe cases, the entire skin and mucosal surfaces may slough

Question 17

what patients are usually affected by erythema multiforme?

Answer 17

young adults in their 20s-30s, M>F

Question 18

what are the prodromal symptoms of erythema multiforme? how long before onset do they occur?

Answer 18

fever, headache, cough, sore throat occur 1 week before onset

Question 19

how long does erythema multiforme last? what % of cases have recurrent episodes?

Answer 19

• 2-6 weeks
• 20%

Question 20

what percent of patients with erythema multiforme develop skin lesions?

Answer 20

50%

Question 21

describe the skin lesions associated with erythema multiforme

Answer 21

• early lesions are typically flat, round, and red
• lesions become elevated and evolve into bulla with necrotic centers
• a highly characteristic skin lesion that develops is a target lesion
  
  • concentric circular erythematous rings resembling a target or bulls-eye

Question 22

describe the oral lesions associated with erythema multiforme

Answer 22

• erythematous patches that undergo epithelial necrosis and ulcerate
• entire oral cavity can be involved except the gingiva and hard palate

Question 23

what is a common presentation of the lips with someone who has erythema multiforme?

Answer 23

hemorrhagic crusting of the vermillion zone

Question 24

erythema multiforme lesions emerge quickly and are painful. what is a potential problems this poses for patients?

Answer 24

patients may become dehydrated due to an inability to ingest liquids

Question 25

stevens-johnson syndrome is also known as \_\_\_

Answer 25

erythema multiforme major

Question 26

which form of erythema multiforme is usually triggered by a drug and must have skin, oral mucosa, and either ocular or genital mucosa involvement to be diagnosed?

Answer 26

steven-johnson syndrome (EM major)

Question 27

which form of erythema multiforme is the most severe form, is almost always triggered by a drug, and involves diffuse sloughing of the skin and mucosa?

Answer 27

toxic epidermal necrolysis

Question 28

toxic epidermal necrolysis tends to occur in what age patient, and what gender more commonly?

Answer 28

* older people (EM minor and major are younger people) * F\>M (unlike EM minor)

Question 29

if a patient with toxic epidermal necrolysis survives, what happens to the cutaneous process?

Answer 29

typically resolves in 2-4 weeks

Question 30

describe the management of erythema multiforme

Answer 30

* discontinuation of the causative drug is paramount * if due to herpes, daily antivirals are indicated * patient is usually admitted to hospital for IV fluids * steroids are used in EM minor and major but are contraindicated in toxid epidermal necrolysis * TEN patients are treated in the burn unit

Question 31

what is the mortality rate for patients with erythema multiforme major?

Answer 31

up to 10%

Question 32

what is the mortality rate for patients with toxic epidermal necrolysis?

Answer 32

about 35%

Question 33

Answer 33

erythema multiforme

Question 34

what are two other names for erythema migrans?

Answer 34

geographic tongue and benign migratory glossitis

Question 35

erythema migrans is a common condition that primarily affects the \_\_\_, affects \_\_\_% of the population, and is two times more common in which gender?

Answer 35

* tongue * 3% * F:M 2:1

Question 36

erythema migrans may be related to what condition?

Answer 36

psoriasis

Question 37

erythema migrans lesions characteristically appear where on the tongue?

Answer 37

* anterior 2/3 of the dorsal tongue * concentrated on the tip and lateral borders

Question 38

erythema migrans may occur in places other than the tongue. what helps with identifying these lesions as erythema migrans?

Answer 38

they will have a serpentine border

Question 39

how do erythema migrans lesions develop?

Answer 39

* begin as multiple, small white patches which develop central erythematous atrophic zones and enlarge into well-demarcated zones of erythema * these areas are surrounded at least partially by a slightly elevated, yellow-white, serpentine or scalloped border * the lesions will heal in one area and then develop in a different area

Question 40

what other feature is associated with erythema migrans?

Answer 40

fissured tongue

Question 41

is erythema migrans symptomatic or asymptomatic?

Answer 41

asymptomatic, but some patients may experience burning when eating spicy foods

Question 42

what is the treatment for erythema migrans? is a biopsy required?

Answer 42

* no treatment necessary for asymptomatic erythema migrans * symptomatic patients should use topical corticosteroids * no biopsy required - it is a clinical diagnosis * patients should be assured they have a benign condition

Question 43

Answer 43

erythema migrans

Question 44

\_\_\_ is a common, chronic dermatologic disease that can affect the oral mucosa, and may be due to medications, amalgam, etc

Answer 44

* lichen planus * form that results from medications or amalgam is better known as "lichenoid mucositis"

Question 45

what age and gender is most common in patients affected by lichen planus? approximately what percent of the population is affected?

Answer 45

* middle-aged adults * F\>M * 1%

Question 46

describe lichen planus skin lesions (4 Ps)

Answer 46

* purple * pruritis * polygonal * papules

Question 47

do lichen planus skin lesions itch?

Answer 47

yes, but the patient doens't usually scratch because the lesions are painful

Question 48

lichen planus skin papules have a thin, lacelike network of white lines termed \_\_\_

Answer 48

wickham's striae

Question 49

what are the two forms of oral lichen planus?

Answer 49

reticular and erosive

Question 50

which form of lichen planus oral lesions are much more common and are asymptomatic?

Answer 50

reticular

Question 51

describe reticular oral lichen planus

Answer 51

* involves the posterior buccal mucosa bilaterally, seen as wickham's striae * if it occurs on the tongue, the lesions will be plaque-like * lesions tend to wax and wane

Question 52

which form of oral lichen planus is symptomatic?

Answer 52

erosive

Question 53

describe erosive oral lichen planus

Answer 53

* lesions are atrophic, erythematous areas with a central ulceration * periphery is usually bordered by fine, white, radiating striae * if confined to the gingiva, it is termed desquamative gingivitis * becomes indistinguishable from pemphigus and pemphigoid, which may also be limited to the gingiva

Question 54

how do drug-induced lichen planus lesions typically present?

Answer 54

found on the lower lip as non-healing ulcers

Question 55

what are the histopathologic features of lichen planus?

Answer 55

* pointed, "saw-toothed" rete ridges * destruction of the basal layer * band-like infiltrate of lymphocytes subjacent to the epithelium * degenerating keratinocytes within the epithelium (termed civatte bodies) * deposition of fibrinogen at the basement membrane via DIF

Question 56

is a biopsy needed for oral lichen planus lesions?

Answer 56

* not if lesions are bilateral AND asymptomatic * this is consistent with a clinical diagnosis of reticular oral lichen planus * biopsy is indicated if the lesions are symptomatic OR asymmetric

Question 57

what is the treatment for oral lichen planus?

Answer 57

* no treatment is necessary for bilateral AND asymptomatic lesions * if lesions are symptomatic OR asymmetric, oral topical steroids are used for treatment

Question 58

28% of women with erosive oral lichen planus also have ___ lesions

Answer 58

vaginal

Question 59

what 4 things can oral lichen planus lesions be induced by?

Answer 59

* stress * trauma * advil (any NSAID, acetaminophen is ok) * yeast

Question 60

Answer 60

reticular oral lichen planus

Question 61

Answer 61

erosive oral lichen planus

Question 62

Answer 62

lichen planus

Question 63

Answer 63

lichen planus

Question 64

Answer 64

lichen planus

Question 65

Answer 65

lichen planus wickham's striae

Question 66

Answer 66

lichen planus skin lesions