Skin and Mucosal Diseases II

Question 1

describe lichen planus

Answer 1

• common, chronic disease that affects skin and oral mucosa
• immune mediated mucocutaneous disorder
• medications may cause similar appearance: lichenoid mucositis

Question 2

what are the clinical features of lichen planus

Answer 2

• Most common in middle aged female adults
• skin lesions: purple, pruritic, polygonal, papules (4-Ps)
• often affect flexor surfaces of extremities

Question 3

what are the clinical features of reticular lichen planus

Answer 3

• most common
• involves buccal mucosa bilaterally
• interlacing white lines- Wickham striae
• wax and wane
• post inflammatory melanosis in darker skin patients
• usually asymptomatic

Question 4

what are the clinical features of erosive type lichen planus

Answer 4

• atrophic, erythematous areas with central ulceration
• patients often symptomatic
• periphery bordered by fine, white radiating striae
• atrophy and ulceration confined to gingiva: desquamative gingivitis

Question 5

what is the dx for lichen planus

Answer 5

• clinical, histopathology, direct immunofluorescence
• 10% formaling vs Michels solution

Question 6

when do you use michels solution

Answer 6

when you need to see direct immunofluorescence

Question 7

what is the tx for lichen planus

Answer 7

• reticular: usually asymptomatic, no tx needed
• erosive: topical corticosteroids

Question 8

describe erythema multiforme

Answer 8

• ulcerative mucocutaneous condition of uncertain etiology
• likely immune mediated
• 50% of cases: precipitating cause- infections such as herpes simplex, medications

Question 9

what are the clinical features of erythema multiforme

Answer 9

• often observed in young adults (20s and 30s)
• prodromal symptoms: fever, malaise, headache, cough
• slightly elevated, round, dusky-red patches on skin
• may appear as concentric circular erythematous rings- bullseye

Question 10

where are the lesions found in erythema multiforme

Answer 10

• oral cavity: most frequently involved mucosal site
• ocular, genitourinary, respiratory mucosa may be affected
• oral lesions: shallow erosions or ulcerations with irregular borders
• lips, labial mucosa, buccal mucosa, tongue, FOM, soft palate
• hemorrhagic crusting of the vermilion zone of lips

Question 11

what are the clinical features of erythema multiforme minor

Answer 11

• milder cases
• skin lesions and 1 mucosal site usually oral

Question 12

what are the clinical features of erythema multiforme major

Answer 12

• most severe
• widespread skin lesions and 2 or more mucosal sites
• severe ocular involvement: scarring (symblepharon formation)

Question 13

what is the tx for erythema multiforme

Answer 13

• usually self-limiting (2-6 weeks)
• systemic or topical corticosteroids

Question 14

what is Stevens-Johnson syndrome and toxic epidermal necrolysis

Answer 14

severe blistering diseases triggered by drug exposure

Question 15

what is the skin and mucosal involvement in SJS and TEN

Answer 15

• SJS: less than 10% skin and mucosal involvement
• TEN: greater than 30% skin and mucosal involvement

Question 16

what are the clinical features of SJS and TEN

Answer 16

• SJS: usually seen in younger patients
• TEN: usually patients above 60 years
• initially present with flu-like symptoms
• after a few days cutaneous lesions appear on trunk: erythematous macules, sloughing of the skin, flaccid bullae
• may have mucosal changes as well

Question 17

what is the tx for SJS and TEN

Answer 17

• identify, immediately discontinue offending drug
• management in burn unit of hospital
• mortality rate : SJS is 1-5% and TEN is 25-30%

Question 18

describe pemphigus vulgaris

Answer 18

• autoimmune disease
• immune attack on desmosomes- intra epithelial split
• 1-5 cases per million people
• blistering
• antibodies against desmosomes that hold epithelial cells together

Question 19

what are the clinical features of pemphigus vulgaris

Answer 19

• oral lesions often first sign of disease and most difficult to treat
• average diagnosis 50 years
• superficial, ragged erosions and ulcerations
• affect any oral mucosal location
• desquamative gingivitis
• skin lesions: flaccid vesicles and bullae that rupture quickly
• ocular lesions: conjunctivitis

Question 20

what are the clinica features of pemphigus vulgaris

Answer 20

• lesions persist and progress without treatment
• lesions are painful
• skin: positive Nikolsky sign: bulla appears with firm lateral pressure

Question 21

what is the dx with pemphigus vulgaris

Answer 21

• clinical, histopathology, direct immunofluorescence
• 10% formalin vs michels solution
• perilesional biopsy

Question 22

what is the tx for pemphigus vulgaris

Answer 22

• systemic corticosteroids
• immunosuppressive drugs

Question 23

describe mucus membrane pemphigoid

Answer 23

• autoimmune disease
• chronic, blistering, mucocutaneous disease
• at least 2x more common compared to pemphigus
• tissue- bound autoantibodies against components of basement membrane - hemidesmosomes

Question 24

what are the clinical features of mucus membrane pemphigoid

Answer 24

• 50-60 years of age
• most common mucosal site: oral cavity
• ocular, nasal, esophageal, laryngeal, vaginal mucosa
• oral lesions: vesicles or bullae
• may rupture: large erosions and ulcerations
• lesions are painful
• may be observed in any intraoral site
• desquamative gingivitis

Question 25

what is the most significant complication in mucus membrane pemphigoid

Answer 25

- ocular involvement - symblepharon formation - may result in blindness

Question 26

what is another name for mucus membrane pemphigoid

Answer 26

cicatricial pemphigoid

Question 27

what is the dx for mucus membrane pemphigoid

Answer 27

- clinical, histopathology, direct immunofluorescence - 10% formalin vs michels solution - perilesional biopsy

Question 28

what is the tx for mucus membrane pemphigoid

Answer 28

- if only oral lesions are present, may be controlled with topical corticosteroids - patient should be refeerred to opthamologist - OHI measures can improve gingival lesions

Question 29

describe bullous pemphigoid

Answer 29

- autoimmune disease - chronic, blistering, mucocutaneous disease - tissue- bound autoantibodies against components of basement membrane - hemidesmosomes

Question 30

what are the clinical features of bullous pemphigoid

Answer 30

- 75-80 years - pruritis is often an early symptom - bullae develop on skin- rupture after several days - healing without scarring

Question 31

describe systemic sclerosis

Answer 31

- may be immune mediated condition - dense collagen deposited throughout the tissue - most organs of the body affected

Question 32

what are the clinical features of systemic sclerosis

Answer 32

- females: 2-3x more common - mainly observed in adults - often first noticed by cutaneous changes - skin develops diffuse, hard texture - surface is usually smooth - involvement of facial skin: smooth, taut, mask-like apperance - microstomia - tongue becomes stiff, dysphagia may develop - xerostomia - nasal ala decomes atrophied- pinched appearance - raynaud phenomenon - resorption of terminal phalanges -> shortened clawlike fingers

Question 33

describe raynaud phenomenon

Answer 33

vasoconstrictive event triggered by exposure to cold or stress- often the 1st sign of disease

Question 34

what organs are involed in systemic sclerosis

Answer 34

- fibrosis of lungs, heart, GI tract - may eventually lead to organ faulure - pulmonary fibrosis -> pulmonary hypertension -> HF

Question 35

what are the radiographic features of systemic sclerosis

Answer 35

- widening of PDL - resorption of posterior mandibular ramus, condyle, coronoid process

Question 36

what is the tx for systemic sclerosis

Answer 36

- prognosis is poor - systemic medications: penicillamine

Question 37

what are the components of crest syndrome

Answer 37

- calcinosis cutis - raynaud phenomenon - esophageal dysfunction - sclerodactyly - telliangiectasia

Question 38

what is another name for CREST syndrome

Answer 38

limited scleroderma

Question 39

what are the clinical featurs of CREST synromr

Answer 39

- most common in females 6-7th decade of life - calcinosis cutis: movable, subcutaneous ndules - raynaud phenomenon: severe vasopasm in fingers/toes - esophageal dysfunction: abnormal collagen deposition - sclerodactyly: fingers become stiff, skin - smooth, shiny appearance - telangiectasia: superficial dilated capillaries

Question 40

describe Crohn's disease

Answer 40

- inflammatory bowel disease, immune related - anywhere in GI tract- mouth to anus - genetic factor implicated - oral lesions may precede GI lesions

Question 41

what are the clinical feautres of Crohn's disease

Answer 41

- most common dx in 2nd decade - abdominal cramping, pain, nausea, diarrhea - weight loss and malnutrition may develop -> anemia, decreased growth - oral: diffuse, nodular swelling, ulcers - cobblestone appearance - erythematous macules and plaques

Question 42

what is the tx for crohn's disease

Answer 42

- oral lesions tpyically clear with GI tx - sulfasalazine, antibiotics, corticosteroids