5 dermatologic diseases Flashcards
is desquamative gingivitis a diagnosis?
no, it is a clinical term to describe a manifestation of what is usually lichen planus, mucous membrane pemphigoid, or pemphigus vulgaris
clinical features of desquamative gingivitis
- female predilection
- diffuse, erythematous gingiva
- involves attached tissues
- sensitive to spicy food
- may or may not be symptomatic
what is the most common lichenoid process condition we will see
idiopathic lichen planus
clinical features of lichen planus
- purple, polygonal, pruiritic papules, very itchy
- flexor surfaces of extremities like wrists and shins
reticular form of lichen planus
- more common than erosive or ulcerative
- asymptomatic
- posterior buccal mucosa
- lacy white striations (wickham striae)
lacy white striations (wickham striae)
reticular form of lichen planus
-spiderweb like
lichen planus is usually bilateral or unilateral?*
bilateral
lichen planus most common form
reticular form
erthematous form of lichen planus
- symptomatic
- striae at periphery of erythema
- may cause desquamative gingivitis (must be distinguished from MMP or pemphigus)
striae at periphery of erythema
erythematous form of lichen planus
least common form of lichen planus
ulcerative form
ulcerative form of lichen planus
- symptomatic
- white lesions, red lesions, + yellow lesions
- ulcers with striae and erythema
- may cause desquamative gingivitis (must be distinguished from MMP or pemphigus)
lichen planus is
idiopathic, don’t know why patients get them
lichen planus associated with what common medications
- NSAIDS
- anti-hypertensives
- antihyperglycemics
- cholesteral lowering agents
- anti-hypothyroidism agents
- antigout medications
- amalgam restorations
- cinnamon flavoring agents
- herbal remedies
what is the exception to the rule that lichen planus is never unilateral
amalgam restorations or cinnamon flavoring if they only chew on one side of the mouth
Is lichen planus immune mediated?
yes, but not autoimmune bc it’s a T cell process
histopathologic features of lichen planus
- orthokeratosis/parakeratosis that causes white wickam striae
- saw tooth rete ridges
- degeneration of basal cell layer
- colloid bodies (Degenerative aptotic cells
- band-like inflitrate of lymphocytes
easiest feature to recognize of lichen planus
squamitization of basal cell layer or bandlike infiltrate of lymphocytes
treatment of lichen planus
- just observe if asymptomatic (don’t have to treat)
- topical corticosteroids
topical corticosteroids for treating lichen planus
- fluocinonide or clobetasol gel (warn patient they should eat/drink for 30 min for longer contact time)
- dexamethasone elixir
- prophylactic antifungals in patients predisposed to candidiasis
malignant prognosis of lichen planus
- needs life long follow up
- 1-2 % increased risk of developing SCCA
- reported cases associated with erosive form
lichen planus
wrist as purple polygonal papules, has fine white lines (Wickham’s striae)
erosive lichen planus
ulceratoin of buccal mucosa shows peripheral radiating keratotic striae
erosive planus often appears as
desquamative gingivitis, producing erythema + tenderness
what is almost indistinguishable from lupus?
lichen planus
3 subtypes of lupus erythematous
(autoimmune disease)
1 systemic lupus erythematous (SLE)
2 chronic cutaneous lupus eryhtematous (CCLE)
3 subacute cutaneous lupus eryhtematous
systemic lupus erythematous
-multisystem disease with skin + oral lesions
predilection os systemic lupus erythematous
8-10 : 1 females
clinical features of systemic lupus erythematous
- fever, weight loss, arthritis, fatigue, malaise
- 40-50% butterfly rash over malar area + nose
- kidney involvement in 40-50%
- cardiact involvement
clinical features in the mouth of clinical features of systemic
- oral lesions in 5-40% of patients
- lichenoid lesions of palate, buccal mucosa, or gingiva (more likely than lichen planus to involve palate)
chronic cutaneous lupus erythematous oral clinical features
- oral lesions identical to lichen planus
- rarely without skin lesions
- erythematous zone
- surrounding fine white striae
- possible central stippling white dots
- may be sensitive to acidic or salty foods
subacute cutaneous lupus erythematous
features intermediate between SLE +CCLE
lupus
true autoimmune disease whereas lichen planus is not
ild SLE treatment
NSAIDs + antimalarials (antiinflammatory properties)
prognosis of SLE
95% 5 year survival
75% 15 year survival
renal failure= #1 cause of death
chronic cutaneous lupus eryhtematous skin lesions
scaling, atrophy, and pigmentary disturbances
-most evident on sun exposed skin
graft vs. host disease
-seen in allogeneic hematopoietic stem cell transplant recipients (8,000 annual transplants in USA)
milder graft. vs. host disease seen in patients with
- tend to do better
- better histocompatibility match
- younger age
- cord blood received
- female gender
oral lesions clinical features of graft vs. host disease
- sometimes, there’s only oral lesions
- 33- 75% of acute patients, 80% of chronic patients
- may be ONLY sign of disease
- wickham striae
- diffuse white papules on tongue, labial mucosa or buccal mucosa
- burning mucosa (must rule out candidiasis)
graft vs. host disease oral lesions
- ulcers last >2 weeks
- may relate to chemotherapy
- r/o HHV or bacterial infection
- xerostomia (tend to destry salivary glands)
- palatal mucoceles
people with graft vs. host disease have increased risk of
SCCA
direct immunofluorescence
identifies autoantibodies bound to patient’s tissue
indirect immunofluorescence
identifies autoantibodies circulating in patient’s blood
when considering vesiculo
- always include intact epithelium
- submit separate biopsies in 10% fomalin for H+ E or michel medium for DIF
pemphigus related diseases
- pemphigus vulgaris
- pemphigus vegetans
pemphigus vulgaris
most common, if untreated, can be threatening
pemphigus vegetans
rare, sometimes oral lesions
pemphigus vulgaris- antibodies to
antibodies to desmosomes
pemphigus vulgaris is a potentially life threatening
skin and mucous membrane disease
clinical features of pemphigus vulgaris
-painful chronic oral sores, dysphagia, weight loss
oral lesions of pemphigus vulgaris
- 50% show oral lesions first, up to 1 yr before skin lesions
- almost all show oral lesions eventually
- erosions and ragged ulcerations of palate, gingiva, buccal, or labial mucosa
- oral lesions often last to resolve
- positive nikolsky sign
histopathologic features of pemphigus vulgaris
- perilesional tissue required for diagnosis
- intraepithelial separation above basal layer (SUPRA basilar cleavage)
- acantholysis (splitting apart) of spinous layer
- generally positive with DIF IIF
rounded tzanck cells
histologic features of pemphigus vulgaris
-keratinocytes lose some of their structure when they have acantholysis of spinous layer
PV can be thought of like abrick wall
bricks precariously stay together, but with force they will fall apart
tx of pemphigus vulgaris
- corticosteroids
- azathioprine
- immunoglobulin therapy
- untreated, mortality approaches 80%
paraneoplastic autoimmune multiorgan syndrome (PAMS)
- vesiculobullous lesions on skin + oral mucosa (patient can’t eat or swallow)
- palmar + plantar bullae distinguish from pemphigus
- crusted lips mimic erythema multiform
- 70% have eye scarring
crusted lips mimic erythema multiforme
paraneoplastic pemphigus autoimmune multiorgan syndrome
paraneoplastic autoimmune multiorgan syndrome (PAMS) histopathologic features
sub-epithelial or intraepithelial clefting
mucous membrane pemphigoid
autoimmune disorder with autoantibodies to hemidesmosomes, NOT DESMOSOMES***
what is 2x as common as pemphigus vulgaris
mucous membrane pemphigoid
average age of mucous membrane pemphigoid
50-60 years
-female predilection 2:1
mucous membrane pemphigoid
- only ocular involvement
- only oral cavity involvement
- mucosal + cutaneous lesions
- multiple mucosal surfaces, no significant skin disease
mucous membrane pemphigoid
postiive nikolsky sign **
clinical features of mucous membrane pemphigoid
oral lesions seen in most, but conjunctiva, nose, esophagus, larynx, and vagina may be invovled
25% with oral lesions in mucous membrane pemphigoid may develop
eye lesions
mucous membrane pemphigoid histopathologic features
sub-epithelial separation below basal layer (SUB basilar cleavage)
mucous membrane pemphigoid treatment
- topical steroids for oral lesions
- refer to opthalmologist
trichiasis
eyelashes rubbing aginst the eye itself
-upper eyelid turns inward
bullous pemphigoid
similar to MMP (mucous membrane pemphigoid) but with very rare oral lesions
epidermolysis bullosa acquisita
50% of patients have oral lesions (most with cutaneous lesions)
linear IgA disease
50% have mucosal involvement
erythema multiforme is triggered by
cross reactivity to herpes simplex virus
- rapid onset
- immune mediated condition
erythema multiforme most cases are secondary to
infection; typically HSV or mycoplamsa pneumoniae
predilection for erythema multiforme
male predilection; mostly ages 20-40
erythema multiforme is one of few conditions that can present with
rash on palms of hand (papilloma fabe syndrome does as well)
clinical features of erythema multiforme
- erythematous mucosal patches
- shallow ulcers (very painful)
- hemorrhagic crusts of lips**
- entire perimeter of tongue may be affected
- targeteoid cutaneous lesoins
targeteoid cutaneous lesoins
looks like a bulleye
**common clinical features of erythema multiforme
hemorrhagic crusts of lips + targetoid cutaneous lesions
erythema multiforme treatment
systemic and or topical steroids
stevens-johnson syndrome
- milder form of toxic epidermal necrosis
- usually result of medication
- mucocutaneous disease often confused with erythema multiforme
- stevens-johnson syndrome invovles head and trunk and is triggered by a drug reaction rather than an infection
stevens-johnson syndrome affects
ocular or genital mucosa involved in conjunction with oral and skin lesions
toxic epidermal necrolysis aka
lyell disease
toxic epidermal necrolysis
severe form of stevens-johnson syndrome which involves more than 30% of total body surface area
age of most patients with toxic epidermal necrolysis
> 60 years
toxic epidermal necrolysis diffuse sloughing of
a significant portion of skin and mucosal surfaces, predisposing to dehydration and infection
toxic epidermal necrolysis management
as for burn patients
toxic epidermal necrolysis mortality rate
25-30%
oral manifestations of dermatologic diseases lichenoid diseases
- idiopathic lichen planus
- lichenoid drug eruptions
- lupus erythematosus
- graft vs. host disease
oral manifestations of dermatologic diseases- autoimmune diseases
- pemphigus vulgaris
- paraneoplastic autoimmune multiorgan syndrome
- mucous membrane pemphigoid
oral manifestations of dermatologic diseases-immune mediated diseases
- erythema multiforme
- stevens-johnson syndrome/toxic epidermal necrolysis
