Disease & Mucosa Disease- Part 2 Flashcards
Common, chronic disease that affects skin & 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 plaque:
- MC in middle-aged females
- skin lesions
Describe the skin lesions associated with lichen planus:
-often affect flexor surfaces of extremities
(4 P’s)
1. purple
2. pruritic
3. polygonal
4. papules
4 P’s 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 bordered by fine, white radiating striae
- atrophy & ulceration confined to the gingiva (desquamative gingivitis)
How do you diagnose lichen planus?
Clincal, histopathology, direct immunofluorescence
10% Formalin vs. Michels solution
What treatment is indicated for lichen planus?
Reticular: usually asymptomatic no treatment is needed
Erosive: topical corticosteroids
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following image:
Lichen planus
Diagnose the following based on the histological image:
Lichen planus
Diagnose the following based on the histological image:
Lichen planus
Ulcerative mucocutaenous condition of uncertain etiology but likely an immune mediated process:
Erythema multiforme
50% of erythema multiform causes have a ____ cause. Give some examples
Precipitating cause- infections (HSV) & mediations (infrequently)
List the clinical features of erythema multiform:
- observed in young adults (20s-30s)
- Prodromal symptoms (fever, malaise, headache, cough)
- slightly elevated. round, dusky-red patches on skin
- concentric, circular, erythematous rings (target lesion)
Where is the most frequently involved mucosal site for erythema multiform?
oral cavity
Although the oral cavity is the most frequently involved mucosal site for erythema multiforme, other mucosa may be involved including:
- genitourinary
- ocular
- respiratory
Describe the oral lesions associated with erythema multiforme:
shallow erosions or ulcerations with irregular borders located on the lips, labial & buccal mucosa, tongue, FOM & 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 & 1 mucosal site (usually oral)
Describe the clinical features of erythema multiforme MAJOR:
-more severe
-widespread skin lesions & 2 or more mucosal sites
-severe ocular involvement (scarring & symblepharon 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
Diagnose the following image:
erythema multiforme
Diagnose the following image:
erythema multiforme
Diagnose the following image:
erythema multiforme
Severe blistering diseases triggered by drug exposure include:
- Steven-Johnson syndrome (SJS)
- Toxic epidermal necrolysis (TEN)
Describe Steven-Johnson syndrome (SJS):
Less than 10% of skin & mucosal involvement
Describe Toxic epidermal necrolysis (TEN):
Greater than 30% of skin & 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 & TEN initially present with ____ and after a few days ______ appear on the ____
flu-like symptoms; cutaneous lesions; trunk
Describe the cutaneous lesions seen in SJS & TEN. What may also occur?
Erythematous macules; sliuhginh of skin (flaccid bullae); may have mucosal changes as well
What treatement is indicated for patients with SJS & 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/TEN
Autoimmune disease caused immune attack on desmosomes resulting in intra-epithelial split:
Pemphigus vulgaris
Pemphigus vulgaris effects ___ 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 (first 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 (flaccid vesicles & bullae that rupture quickly)
- ocular lesions: conjunctivitis
Describe the lesion seen in pemphigus vulgaris:
-superficial ragged erosions & ulcerations
-lesions persist & progress without treatment
-lesions are painful
In pemphigus vulgaris cases, what sign is positive?
Nikolsky signs; bullae appears with firm lateral pressrue
How do you diagnose pemphigus vulgaris?
- clinical, histopathology & direct immunofluorescence
- 10% Formalin vs. Michels solution
- Perilesional biopsy
What treatment is indicated with pemphigus vulgaris?
systemic corticosteroids & immunosuppressive drugs
Diagnose the following image:
Pemphigus vulgaris
Diagnose the following image:
Pemphigus vulgaris
Diagnose the following image:
Pemphigus vulgaris
Diagnose the following image:
Pemphigus vulgaris
Diagnose the following image:
Pemphigus vulgaris
Diagnose the following image:
Pemphigus vulgaris
Diagnose the following histological image:
Pemphigus vulgaris
Diagnose the following histological image:
Pemphigus vulgaris
Diagnose the following histological image:
Pemphigus vulgaris
An autoimmune disease characterized by chronic blistering due to tissue-bound autoantibodies against components of the basement membrane (hemidesmosomes):
Mucous membrane pemphigoid (Cicatricial pemphigoid)
Mucous membrane pemphigoid is a ____ & ____ disease and results in _____
autoimmune & mucocutaneous; 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)
Clinical features of mucous membrane pemphigoid (cicatricial pemphigoid):
- 50-60 years of age
- MC mucosal site is the oral cavity
- can also be seen: ocular, nasal, esophageal, laryngeal & vaginal mucosa
- oral vesicles or bullae
- painful lesions
- desquamative gingivitis
The oral lesions (vesicles or bullae) in mucous membrane pemphigoid may:
rupture causing large erosions & uclerations
What is the most significant complication of mucous membrane pemphigoid?
Ocular involvment- symblepharon formation - may result in blindness
How do you diagnose mucous membrane pemphigoid?
- Clinical, histopathology, direct immunofluorescence
- 10% Formalin vs. Michels solution
- Periolesional biopsy
What treatment is indicated in mucous membrane pemphigoid?
- if oral lesions present may be controlled with topical corticosteroids
- patients should be referred to opthalmologist
- OHI measures for gingival lesions
Diagnose the following image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following histological image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Diagnose the following histological image:
Mucous membrane pemphigoid (cicatricial pemphigoid)
Autoimmune disease characterized by chronic blistering caused by tissue-bound antibodies against components of the basement membrane (mucocutaenous 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
- pruritis is often an early symptom
- bullae develop on skin & 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 sclerosis (scleroderma)
List the clinical features of systemic sclerosis (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)
- microstomia develops with perioral involvement
- tongue becomes stiff
- dysphagia
- xerostomia
- nasal Ala becomes atrophied (pinched appearance)
- Raynaud phenomenaum
- Resorption of terminal phalanges (claw-like fingers)
Vasoconstrictive event triggered by exposure to cold or stress often the 1st sign of 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 the PDL
- resorption 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 may also be called:
limited scleroderma
Describe the components of CREST syndrome:
- Calcinosis cutis
- Raynaud phenomenon
- Esophageal dysfunction
- Scerlodactyly
- Teliangiectasia
Describe the clinical features of CREST syndrome:
- Calcinosis cutis
- Raynaud phenomenon
- Esophageal dysfunction
- Scerlodactyly
- Teliangiectasia
- Calcinosis cutis - movable subcutaneous nodules
- Raynaud phenomenon- severe vasospasm in fingers/toes
- Esophageal dysfunction- abnormal collagen deposition
- Scerlodactyly- fingers become stiff; skin is smooth & shiny
- Telangiectasia- superficial dilated capillaries
CREST syndrome is most commonly seen in:
females 6th-7th decade
Diagnose the following image:
CREST syndrome
Diagnose the following image:
CREST syndrome
Diagnose the following image:
CREST syndrome
Diagnose the following image:
CREST syndrome
Diagnose the following histological image:
CREST syndrome
Immune related inflammatory bowel disease:
Crohn’s disease
A ____ factor may be implicated in Crohn’s disease
genetic factor
Crohn’s disease can occur anywhere in the:
GI tract (mouth to anus)
In Crohn’s disease, what may precede the GI lesions?
Oral lesions
List the clinical features of Crohn’s disease:
- MC diagnosed in 2nd decade
- abdominal cramping, pain, nausea, diarrhea
- weight loss & malnutrition
- anemia
- decreased growth
- diffuse, nodular oral swelling & oral ulcers
Describe the oral manifestations of Crohn’s disease:
-diffuse, nodular oral swelling
-oral ulcers
-cobblestone appearance
-erythematous macules & plaques
What treatment is indicated in cases of Crohn’s disease:
Oral lesions typically clear with GI treatment of Sulfasalazine, antibiotics & corticosteroids
Diagnose the following image:
Crohn’s disease
Diagnose the following image:
Crohn’s disease