Skin & Mucosal Diseases- Part 2 Flashcards
Common chronic disease that affects skin and oral mucosa that is an immune-mediated mucocutaneous disorder:
lichen planus
What may cause a similar appearance to lichen planus? What is this called?
medications; lichenoid mucositis
List the clinical features of lichen planus:
- MC in middle-aged females
- skin lesions
Describe the skin lesions associated with lichen planus:
often affect flexor surfaces of extremities; 4 P’s
- purple
- pruritic
- polygonal
- papules
The 4 Ps to lichen planus skin lesions:
- purple
- pruritic
- polygonal
- papules
What type of lichen planus is the most common?
reticular type
Describe the clinical features of the RETICULAR TYPE of lichen planus:
- involves buccal mucosa bilaterally
- interlacing white lines (Wickham striae)
- wax & wane
- post-inflammatory melanosis
- usually asymptomatic
Describe the clinical features of the EROSIVE TYPE of lichen planus:
- atrophic erythematous areas with central ulceration
- patients often symptomatic
- periphery borders by fine, white radiating striae
- atrophy and ulceration confined to the gingiva (desquamative gingivitis)
How do you diagnose lichen planus?
clinical, histopathology, direct immunofluorescence
(10% formalin vs. Michels solution)
What treatment is indicated for lichen planus?
Reticular: usually asymptomatic no treatment needed
Erosive: topical corticosteroids
Diagnose this image:
lichen planus
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lichen planus
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lichen planus
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lichen planus
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lichen planus
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lichen planus
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lichen planus
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lichen planus
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lichen planus
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lichen planus
Diagnose the following histological image:
lichen planus
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lichen planus
Ulcerative mucocutaneous condition of uncertain etiology but likely an immune mediated process:
Erythema multiforme
50% of erythema multiform cases have a ___ cause. Give some examples:
precipitating- infections (HSV) and medications (infrequently)
List the clinical features of erythema multiform:
- observed in young adults (20s-30s)
- prodromal symptoms (fever, malaise, HA, cough)
- slightly elevated round, dusky-red patched on skin
- concentric circular erythematous rings (target lesion)
Where is the most frequently involved mucosal site for erythema multiforme?
oral cavity
Although the oral cavity is the most frequently involved oral site for erythema multiforme, other mucosa may be involved including:
- genitourinary
- ocular
- repsiratory
Describe the oral lesions associated with erythema multiforme:
shallow erosions or ulcerations with irregular borders, located on the lips, labial mucosa, buccal mucosa, tongue, FOM, and soft palate
Describe what can occur with the lips in a patient with erythema multiforme:
hemorrhagic crusting of vermillion zone
Describe the clinical features of erythema multiforme MINOR:
- milder cases
- skin lesions and 1 mucosal site (usually oral)
Describe the clinical features of erythema multiforme MAJOR:
- more severe
- widespread skin lesions and 2 or more mucosal sites
- severe ocular involvement (scarring & symblepheron formation)
What is the indicated treatment for erythema multiforme?
- usually self-limiting in 2-6 weeks but may use systemic or topical corticosteroids
Diagnose the following image:
erythema multiforme?
Diagnose the following image:
erythema multiforme?
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erythema multiforme?
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erythema multiforme?
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erythema multiforme?
Severe blistering diseases triggers by drug exposure include:
- Steven-Johnson Syndrome (SJS)
- Toxic epidermal necrolysis (TEN)
Describe Steven-Johnson Syndrome (SJS):
less than 10% of skin and mucosal involvement
Describe toxic epidermal necrolysis (TEN):
greater than 30% of skin and mucosal involvement
Clinical features of SJS are usually seen in:
younger patients
Clinical features of TEN are usually seen in:
patients above 60 years
Both SJS and TEN usually present with _____, and after a few days _____ appear on the ____
Flu-like symptoms; cutaneous lesions; trunk
Describe the cutaneous lesions seen in SJS and TEN. What may also occur?
erythematous macules; sloughing of skin (flaccid bullae); may have mucosal changes as well
What treatment is indicated for patients with SJS and TEN?
- identify & immediately discontinue offending drug
- management in burn unit of hospital
What is the mortality rate for the following?
SJS:
TEN:
SJS: 1-5%
TEN: 25-30%
Diagnose the following image:
SJS or TEN
Autoimmune disease caused by immune attack on desmosomes resulting in intraepithelial split:
pemphigus vulgaris
Pemphigus affects ____ per million people
1-5 cases
Pemphigus vulgaris is an autoimmune disease that attacks:
desmosomes (desmoglein 3)
List the clinical features of pemphigus vulgaris:
- Oral lesions (1st sign of disease & most difficult to treat)
- average diagnosis age 50 years old
- superficial ragged erosions & ulcerations
- affect any mucosal location
- desquamative gingivitis
- skin lesions (placid vesicles and bullae that rupture quickly)
- ocular lesions: conjunctivitis
Describe the lesions seen in pemphigus vulgaris:
- superficial ragged erosions & ulcerations
- lesions persist and progress without treatment
- lesions are painful
In pemphigus vulgaris cases what sign is positive?
Nikolsky sign; bulla appears with firm lateral pressure
How do you diagnose pemphigus vulgaris?
- clinical, histopathology & direct immunofluorescence
- 10% formalin vs. Michels solution
- Periolesional biopsy
What treatment is indicated for pemphigus vulgaris?
systemic corticosteroids, and immunosupressive drugs
Diagnose the following image:
pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
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pemphigus vulgaris
An autoimmune disease characterized by chornic blistering due to tissue-bound autoantibodies against components of the basement membrane (i.e. hemidesmosomes)
mucous membrane pemphigoid (Cicatricial pemphigoid)
Mucous membrane pemphigoid is a ___ and ___ disease and results in ____.
autoimmune; mucocutaneous disease; chronic blistering
What is more common between the following?
- pemphigus vulgaris
- mucous membrane pemphigoid
mucous membrane pemphigoid (cicatricial pemphigoid) is atleast 2x more common
Mucous membrane pemphigoid is an autoimmune disease that attacks the:
components of the basement membrane (hemidesmosomes)
List the clinical features of mucous membrane pemphigoid (cicatricial pemphigoid):
- 50-60 years of age
- most common mucosal site is oral cavity
- can also be seen: ocular, nasal, esophageal, laryngeal, and vaginal mucosa
- oral vesicles or bullae
- painful
- desquamative gingivitis
The oral lesions (vesicles or bullae) in mucous membrane pemphigoid may:
rupture causing large erosions & ulcerations
What is the most significant complication of mucous membrane pemphigoid?
ocular involvement (symblepharon formation)- may result in blindness
How do you diagnose mucous membrane pemphigoid?
- clinical, histopathology, direct immunofluorescence
- 10% formalin vs. Michels solution
- perilesional biopsy
What treatment is indicated in mucous membrane pemphigoid?
- if oral lesions present, may be controlled with topical corticosteroids
- patient should be referred to ophthamologist
- OHI measures for gingival lesions
Diagnose the following image:
mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following image:
mucous membrane pemphigoid (cicatricial pemphigoid)
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mucous membrane pemphigoid (cicatricial pemphigoid)
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mucous membrane pemphigoid (cicatricial pemphigoid)
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mucous membrane pemphigoid (cicatricial pemphigoid)
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mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following histological slide:
mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following histological slide:
mucous membrane pemphigoid (cicatricial pemphigoid)
Autoimmune disease; chronic blistering blistering caused by tissue-bound antibodies against components of the basement membrane (mucocutaneous disease):
Bullous pemphigoid
Give an example of a basement membrane component that may be attacked in bullous pemphigoid:
hemidesmosomes
List the clinical features of bullous pemphigoid:
- 75-80 years old
- pruritic is often an early symptoms
- bullae develop on skin and rupture several days after
- healing without scarring
Diagnose the following image:
bullous pemphigoid
Possibly an immune-mediated condition in which dense collagen is deposited throughout the tissue. Most organs of the body affected:
systemic scerosis (scleroderma)
List the clinical features of systemic scerosis (scleroderma):
- female predominance (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 appearance)
- microstoma deuvels with perioral involvment
- tongue becomes stiff
- dysphagia
- xerostoma
- nasal Ala becomes atrophied (pinched appearance)
- Raynaud phenomenon
- resorption of terminal phalanges (claylike fingers)
Vasoconstrictive event triggered by exposure to cold or stress (often the 1st sign of disease in systemic sclerosis)
Raynaud phenomenon
Systemic sclerosis may involve other organs including:
- fibrosis of lungs
- fibrosis of heart
- fibrosis of GI tract
- pulmonary fibrosis
(all of these may lead to organ failure)
List the radiographic features of systemic sclerosis:
- widening of PDL
- resoprtion of the posterior mandibular ramus
- resorption of condyle
- resorption of coronoid process
What treatment is indicated for systemic sclerosis?
systemic medications like penicillamine however prognosis is poor
CREST syndrome:
limited sceleroderma
Describe CREST syndrome:
- Calcinosis cutis
- Raynaud phenomenon
- Esophageal dysfunction
- Sclerodactyly
- Teliangiectasia
Describe the clinical features of CREST syndrome:
- Calcinosis cutis
- Raynaud phenomenon
- Esophageal dysfunction
- Sclerodactyly
- Teliangiectasia
- Calcinosis cutis- moveable subcutaneous nodules
- Raynaud phenomenon- severe vasospasm in fingers/toes
- Esophageal dysfunction- abnormal collagen deposition
- Sclerodactyly- fingers become stiff; skin is smooth and shiny
- Teliangiectasia- superficial dilated capillaries
CREST syndrome is most commonly seen in:
females in the 6th -7th decade
Diagnose the following image:
CREST syndrome
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CREST syndrome
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CREST syndrome
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CREST syndrome
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CREST syndrome
Immune related inflammatory bowel disease:
crohns disease
A ____ factor may be implicated in crohns disease:
genetic
Chrohns disease can occur anywhere in the:
GI tract (mouth to anus)
In crohns disease, what lesions may precede the GI lesions?
oral lesions
List the clinical features of crohns disease:
- Most commonly diagnosed in 2nd decade
- abdominal cramping, pain, nausea, and diarrhea
- weightless & malnutrition
- anemia
- decreased growth
- diffuse nodular oral swelling and oral ulcers
Describe the oral manifestations of Crohn’s disease:
- diffuse nodular oral swelling
- oral ulcers
- cobblestone appearance
- erythematous macules & plaques
Treatment indicated for Crohn’s disease:
oral lesions typically clear with GI treatment of sulfasalazine, antibiotics and corticosteroids
Diagnose the following image:
Crohns disease
Diagnose the following image:
Crohns disease