Skin and Mucosal Diseases part 2 Flashcards
What is 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?
- MC in middle-aged female adults
- Skin lesions: purple, pruritic, polygonal, papules (4-P’s)
- Often affect flexor surfaces of extremities
What are the clinical features of reticular type (most common) of lichen planus?
- Involves buccal mucosa bilaterally
- Interlacing white lines – Wickham striae
- Wax and wane
- Post-inflammatory melanosis
- Usually asymptomatic
What are the clinical features of erosive type of 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 are the two types of lichen planus?
- reticular type (most common)
- erosive type
How is lichen planus diganosed?
- Clinical, histopathology, direct immunofluorescence
- 10% Formalin vs Michels solution
What is the treatment for lichen planus?
- Reticular: usually asymptomatic, no tx needed
- Erosive: topical corticosteroids
lichen planus
reticular
lichen planus
erosive
What are the areas of dark purple of this histology of lichen planus?
inflammatory cells
What technique is used here and for what disease?
direct immunofluorescence
- lichen planus
What is erythema multiforme?
- Ulcerative mucocutaneous condition of uncertain etiology
- Likely an immune mediated process
- 50% of cases: precipitating cause – infections (ie: herpes simplex), medications (infrequently)
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 – target lesion
What are the clinical sites of erythema multiforme?
- Oral cavity: most frequently involved mucosal site
- Ocular, genitourinary, respiratory mucosa may be affected
— Severe ocular involvement: scarring (symblepharon formation) - 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 types of erythema multiforme?
- Erythema multiforme minor: milder cases
— Skin lesions and 1 mucosal site (usually oral) - Erythema multiforme major: more severe
— Widespread skin lesions and 2 or more mucosal sites
What is the treatment for erythema multiforme?
- Usually self-limiting (2-6 weeks)
- Systemic or topical corticosteroids
erythema multiforme
bullseye/target lesion
erythema multiforme
What is Stevens-Johnson syndrome and toxic epidermal necrolysis?
- Severe blistering diseases triggered by drug exposure
- SJS: <10% skin and mucosal involvement
- TEN: >30% skin and mucosal involvement
What are the clinical features of stevens-johnson syndrome?
- SJS: usually seen in younger patients
- 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 are the clinical features of toxic epidermal necrolysis?
- 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 treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis?
- Identify, immediately discontinue offending drug
- Management in burn unit of hospital
- Mortality rate: SJS – 1-5% , TEN – 25-30%
Stevens-Johnson syndrome and toxic epidermal necrolysis
What is pemphigus vulgaris?
- Autoimmune disease
- Immune attack on desmosomes – intra-epithelial split
- 1-5 cases per million people
What are the clinical features of pemphigus vulgaris?
- Oral lesions often first sign of disease, and most difficult to treat
- Avg diagnosis age: 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
- Lesions persist and progress without treatment
- Lesions are painful
- Skin: positive Nikolsky sign: bulla appears with firm lateral pressure
How do you diagnose pemphigus vulgaris?
- Clinical, histopathology, direct immunofluorescence
-10% Formalin vs Michels solution - Perilesional biopsy
What is the treatment for pemphigus vulgaris?
Systemic corticosteroids, immunosuppressive drugs
pemphigus vulgaris
What is unique in this histology and what disease is it associated with?
- epithelium is “unzipping”
pemphigus vulgaris
What is unique in this histology and what disease is it associated with?
- tombstoning
pemphigus vulgaris
What technique is this used to identify pemphigus vulgaris and what is unique about it?
direct immunofluorescence
- chicken wire appearance
What is mucus membrane pemphigoid (cicatricial pemphigoid)?
- Autoimmune disease
- Chronic, blistering, mucocutaneous disease
- At least 2x more common compared to pemphigus
- Tissue-bound autoantibodies against components of basement membrane (ie: hemidesmosomes)
What are the clinical features of mucus membrane pemphigoid (cicatricial pemphigoid)?
- 50-60 years of age
- MC 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
- Most significant complication: ocular involvement – symblepharon formation
— May result in blindness
How do you diagnose mucus membrane pemphigoid (cicatricial pemphigoid)?
- Clinical, histopathology, direct immunofluorescence
- 10% Formalin vs Michels solution
- Perilesional biopsy
What is the treatment for mucus membrane pemphigoid (cicatricial pemphigoid)?
- If only oral lesions present, may be controlled with topical corticosteroids
- Patient should be referred to ophthalmologist
- OHI measures for gingival lesions
mucus membrane pemphigoid (cicatricial pemphigoid)
What is a perilesional biopsy?
a biopsy near the lesion but not on the lesion
- if you do a biopsy on the lesion there might not be any epithelium
What is unique in this histology and what disease is it associated with?
split of the basal cell layer from the basement membrane
- mucus membrane pemphigoid
What is bullous pemphigoid?
- Autoimmune disease
- Chronic, blistering, mucocutaneous disease
- Tissue-bound autoantibodies against components of basement membrane (ie: hemidesmosomes)
mostly involves the skin
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
Bullous pemphigoid
What is systemic sclerosis (scleroderma)?
- 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 appearance
- Microstomia develops with perioral involvement
- Tongue becomes stiff, dysphagia may develop
- Xerostomia may be present
- Nasal ala becomes atrophied – pinched appearance
- Raynaud phenomenon: vasoconstrictive event triggered by exposure to cold or stress – often 1st sign of disease (fingers and toes)
- Resorption of terminal phalanges → shortened clawlike fingers
- Involvement of other organs: fibrosis of lungs, heart, GI tract (can lead to organ failure)
What is the raynaud phenomenon associated with systemic sclerosis?
vasoconstrictive event triggered by exposure to cold or stress – often 1st sign of disease (fingers and toes)
What are the radiographic features of systemic sclerosis?
- Widening of the PDL
- Resorption of posterior mandibular ramus, condyle, coronoid process
What is the treatment for systemic sclerosis?
prognosis is poor
- Systemic medications: penicillamine (doesn’t work too well)
What is CREST syndrome?
- limited version of scleroderma
- Calcinosis cutis, Raynaud phenomenon, Esophageal dysfunction, Sclerodactyly, Teliangiectasia
What are the clinical features of CREST syndrome?
- MC female, 6th– 7th decade
- Calcinosis cutis: movable, subcutaneous nodules
- Raynaud phenomenon: severe vasospasm in fingers/toes
- Esophageal dysfunction: abnormal collagen deposition
- Sclerodactyly: finger becomes stiff, skin – smooth, shiny appearance
- Telangiectasia: superficial dilated capillaries
scleroderma
What phenomenon is this and for what disease?
Raynaud phenomenon
- Systemic sclerosis
very dense collagen
- systemic sclerosis
What is 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 features of Crohn’s disease?
-MC 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 treatment for Crohn’s disease?
- Oral lesions typically clear with GI treatment
- Sulfasalazine, antibiotics, corticosteroids
Crohn’s disease
