Skin and Mucosal Diseases II Flashcards
describe lichen planus
- common, chronic disease that affects skin and oral mucosa
- immune mediated mucocutaneous disorder
- medications may cause similar appearance: lichenoid mucositis
what are the clinical features of lichen planus
- Most common in middle aged female adults
- skin lesions: purple, pruritic, polygonal, papules (4-Ps)
- often affect flexor surfaces of extremities
what are the clinical features of reticular lichen planus
- most common
- involves buccal mucosa bilaterally
- interlacing white lines- Wickham striae
- wax and wane
- post inflammatory melanosis in darker skin patients
- usually asymptomatic
what are the clinical features of erosive type lichen planus
- atrophic, erythematous areas with central ulceration
- patients often symptomatic
- periphery bordered by fine, white radiating striae
- atrophy and ulceration confined to gingiva: desquamative gingivitis
what is the dx for lichen planus
- clinical, histopathology, direct immunofluorescence
- 10% formaling vs Michels solution
when do you use michels solution
when you need to see direct immunofluorescence
what is the tx for lichen planus
- reticular: usually asymptomatic, no tx needed
- erosive: topical corticosteroids
describe erythema multiforme
- ulcerative mucocutaneous condition of uncertain etiology
- likely immune mediated
- 50% of cases: precipitating cause- infections such as herpes simplex, medications
what are the clinical features of erythema multiforme
- 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
where are the lesions found in erythema multiforme
- 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
what are the clinical features of erythema multiforme minor
- milder cases
- skin lesions and 1 mucosal site usually oral
what are the clinical features of erythema multiforme major
- most severe
- widespread skin lesions and 2 or more mucosal sites
- severe ocular involvement: scarring (symblepharon formation)
what is the tx for erythema multiforme
- usually self-limiting (2-6 weeks)
- systemic or topical corticosteroids
what is Stevens-Johnson syndrome and toxic epidermal necrolysis
severe blistering diseases triggered by drug exposure
what is the skin and mucosal involvement in SJS and TEN
- SJS: less than 10% skin and mucosal involvement
- TEN: greater than 30% skin and mucosal involvement
what are the clinical features of SJS and TEN
- 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
what is the tx for SJS and TEN
- identify, immediately discontinue offending drug
- management in burn unit of hospital
- mortality rate : SJS is 1-5% and TEN is 25-30%
describe pemphigus vulgaris
- autoimmune disease
- immune attack on desmosomes- intra epithelial split
- 1-5 cases per million people
- blistering
- antibodies against desmosomes that hold epithelial cells together
what are the clinical features of pemphigus vulgaris
- 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
what are the clinica features of pemphigus vulgaris
- lesions persist and progress without treatment
- lesions are painful
- skin: positive Nikolsky sign: bulla appears with firm lateral pressure
what is the dx with pemphigus vulgaris
- clinical, histopathology, direct immunofluorescence
- 10% formalin vs michels solution
- perilesional biopsy
what is the tx for pemphigus vulgaris
- systemic corticosteroids
- immunosuppressive drugs
describe mucus membrane pemphigoid
- autoimmune disease
- chronic, blistering, mucocutaneous disease
- at least 2x more common compared to pemphigus
- tissue- bound autoantibodies against components of basement membrane - hemidesmosomes
what are the clinical features of mucus membrane pemphigoid
- 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
what is the most significant complication in mucus membrane pemphigoid
- ocular involvement - symblepharon formation
- may result in blindness
what is another name for mucus membrane pemphigoid
cicatricial pemphigoid
what is the dx for mucus membrane pemphigoid
- clinical, histopathology, direct immunofluorescence
- 10% formalin vs michels solution
- perilesional biopsy
what is the tx for mucus membrane pemphigoid
- if only oral lesions are present, may be controlled with topical corticosteroids
- patient should be refeerred to opthamologist
- OHI measures can improve gingival lesions
describe bullous pemphigoid
- autoimmune disease
- chronic, blistering, mucocutaneous disease
- tissue- bound autoantibodies against components of basement membrane - hemidesmosomes
what are the clinical features of bullous pemphigoid
- 75-80 years
- pruritis is often an early symptom
- bullae develop on skin- rupture after several days
- healing without scarring
describe systemic sclerosis
- may be immune mediated condition
- dense collagen deposited throughout the tissue
- most organs of the body affected
what are the clinical features of systemic sclerosis
- 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
describe raynaud phenomenon
vasoconstrictive event triggered by exposure to cold or stress- often the 1st sign of disease
what organs are involed in systemic sclerosis
- fibrosis of lungs, heart, GI tract
- may eventually lead to organ faulure
- pulmonary fibrosis -> pulmonary hypertension -> HF
what are the radiographic features of systemic sclerosis
- widening of PDL
- resorption of posterior mandibular ramus, condyle, coronoid process
what is the tx for systemic sclerosis
- prognosis is poor
- systemic medications: penicillamine
what are the components of crest syndrome
- calcinosis cutis
- raynaud phenomenon
- esophageal dysfunction
- sclerodactyly
- telliangiectasia
what is another name for CREST syndrome
limited scleroderma
what are the clinical featurs of CREST synromr
- 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
describe Crohn’s disease
- inflammatory bowel disease, immune related
- anywhere in GI tract- mouth to anus
- genetic factor implicated
- oral lesions may precede GI lesions
what are the clinical feautres of Crohn’s disease
- 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
what is the tx for crohn’s disease
- oral lesions tpyically clear with GI tx
- sulfasalazine, antibiotics, corticosteroids
