16 Dermatologic Diseases Flashcards
What is the best known form of ectodermal dyslpasia?
Hypohidrotic Ectodermal Dysplasia
What are distinguishing traits of people with Hypohidrotic ectodermal dysplasia?
- fine, sparse hair
- brittle nails
- hypoplastic/absent salivary glands
- abnormal dental crowns
Patient also has fine hair and high heat intolerance. What do they likely have?
Hypohidrotic ectodermal dysplasia
Hypohidrotic E.D. has a predeominance for which sex, and is linked with which sex chromosome?
Male predominance, X-linked
What defect in keratins presents as white, corrugated plaques bilaterally on buccal mucosa?
White sponge nevus
what is a genetically determined skin disorder?
Genodermatosis
White sponge nevus is recessive or dominant?
Autosomal dominant
What does this suggest? (hint: bilateral presentation)
white sponge nevus
When did these lesions most likely first appear? (hint: presents bilaterally and autosomal dominant)
birth or early childhood
What presents intraorally as a white lesion with a warty or roughened surface?
Warty Dyskeratoma
Warty dyskeratomas histopathologically identical to what disease?
Darier disease
Pt is over 40 and presents with this asymptomatic, rough-surfaced lesion on keratinized mucosa. What is it?
Warty Dyskeratoma
Peutz-Jeghers Syndrome is characterized by what?
- freckle-like lesions on skin around hands, mouth, and intraorally
- intestinal polyposis
- predisposition to develop cancer
What is Peutz-Jegher’s inherited as?
Autosomal dominant
What is unique about the freckles seen with Peutz-Jegher’s Syndrome?
They don’t change darkness with sun exposure like regular freckles
Peutz-Jegher’s patients are __ times more likely to develop cancer in their lifetime.
18 times more likely
Why do Peutz-Jegher’s patients commonly experience intestinal obstruction?
Intussusception, which is telescoping of the proximal bowel into the distal portion
If Peutz-Jegher’s Syndrome, what else might they present with?
Perioral and intraoral freckling
Also has intestinal polyps. What do they have?
Peutz-Jeghers Syndrome
What are some characteristics of tuberous sclerosis?
- mental retardation
- potato-like growths (tubers)
- epilepsy
- shagreen patches
- ash-leaf spots
- angiofibromas of skin
- ungual fibromas
A patient with tuberous sclerosis might show facial angiofibromas where on the face?
nasolabial fold area
What is a shagreen spot?
A connective tissue hamartoma (benign neoplasm that grows at same rate as tissue around it)
What is an ash-leaf spot?
an ovoid area of hypopigmentation
What heart condition is associated with tuberous sclerosis?
Rhabdomyoma
What are these facial angiofibromas characteristic of?
tuberous sclerosis
What is this called? What pathology is it a characteristic of?
- ash-leaf spot
- tuberous sclerosis
What are two oral manifestations seen with tuberous sclerosis?
- facial enamel pitting of permant anterior teeth
- multiple fibrous papules
What are four types of Epidermolysis Bullosa?
- simplex
- junctional
- dystrophic
- hemidesmosomal
What are four things to know about simplex-type epidermolysis bullosa?
- blistering of hands and feet
- mucosal involvement uncommon
- no scarring
- good prognosis
Junctional-type epidermolysis bullosa is characterized by?
- severe blistering at birth
- granulation tissue around mouth
- oral involvement common
- often fatal
What form of dystrophic epidermolysis bullosa is not typically life threatening?
Dominant form
What form of dystrophic epidermolysis bullosa is severely debilitating?
Recessive form
Describe dominant form of dystrophic epidermolysis bullosa.
- blistering and scarring in areas of chronic trauma
- mild oral manifestations
Describe recessive form of dystrophic epidermolysis bullosa.
- blisters and scarring in areas of minor trauma
- secondary infections of blisters
- hands scarred into “mittens”
What type of epidermolysis bullosa is this? (hint: good prognosis, no scarring)
simplex
What is the most common pemphigus-related disease?
Pemphigus vulgaris
Oral lesions of pemphigus vulgaris are described with what clever phrase?
“first to show, last to go”
In pemphigus vulgaris, autoantibodies are directed toward what cellular protein?
desmosomes
How is the histological presentation of epithelium described in pemphigus vulgaris?
As intraepithelial split
What is a characteristic feature of pemphigus vulgaris where bulla apear on normal-appearing skin with firm pressure?
Positive Nikolsky Sign
Give two histological characteristics of pemphigus vulgaris.
- acantholysis (intraepithelial split)
- Tzanck cells
Epithelium just falls off due to acantholysis. What is this?
pemphigus vulgaris
Tzanck cells are seen on histology. What is this?
Pemphigus vulgaris
Patient has positive Nikolsky sign. What is this?
Pemphigus vulgaris
These lesions are “first to show, last to go”. What is this?
Pemphigus vulgaris
This is pemphigus vulgaris. Describe what is happening here.
Autoantibodies are attacking desmosomes of epithelial cells, causing “intraepithelial split”
Intraoral blood blister are virtually pathognomonic for what disease?
Mucous membrane pemphigoid
Unlike with pemphigus, what might be seen intraorally with pemphigoid?
blisters
What happens to the eyes in mucous membrane pemphigoid?
Entropion (eyelids turn inward) and keratin deposition on the cornea; both leading to blindness
What is it called in mucous membrane pemphigoid causes adhesion of the eyelid to the eye?
Symblepharons
What is this called?
symblepharon
What is this called? What disease is it seen with?
- Entropion
- mucous membrane pemphigoid
In pemphigoid, autoantibodies are attacking what?
the basement membrane (causing interepithelial split)
Seen with pemphigoid, what is this showing?
Autoantibodies attacking the basement membrane
A virtually pathognomonic sign for what disease?
mucous membrane pemphigoid
Patient shows intraoral blisters and symblepharons. What is this?
mucous membrane pemphigoid
What are two common infections seen with erythema multiforme?
Herpes simplex, and Mycoplasma pneumoniae
erythema multiforme major is also known as what?
Stevens-Johnson Syndrome
What is a highly characteristic skin lesion that develops in erythema multiforme?
Target lesions
T or F: Erythema multiforme presents with prodrome symptoms 7 days before onset.
True. Prodrome (fever, headache, cough, sore throat) is usually about a week.
What intraoral surfaces are involved in erythema multiforme?
All surfaces except gingiva and hard palate
Also has target lesions on body. What is this diagnosis?
Erythema multiforme
What is this?
target lesions (seen with erythema multiforme)
What is the most severe form of erythema multiforme that occurs in older people and is almost always triggered by a drug?
Toxic epidermal necrolysis
To diagnose Stevens-Johnson Syndrome, what criteria must be met?
skin/oral mucosal involvement
+
ocular/genital mucosal involvement
What are two other names for erythema migrans?
- geographic tongue
- benign migratory glossitis
Where intraorally does erythema migrans present?
anterior 2/3 of tongue
Describe the lesions of erythema migrans.
Irregularly shaped zones of erythema with a raised, yellow to white border
Erythema migrans is associated with what other condition?
fissured tongue
Pt complains of burning when they eat spicy foods. What is this?
Benign migratory glossitis
What is this?
Erythema migrans (geographic tongue)
Skin lesions of lichen planus present with what characteristics?
- purple
- pruritic
- polygonal
- papules
Skin papules of lichen planus also present with what?
Wickham’s striae
Describe reticular lichen planus.
net or web-like
describe erosive lichen planus
ulcerative
Which type of lichen planus is more common, asyptomatic, and involves posterior buccal mucosa as wickham striae?
Reticular lichen planus
Which type of lichen planus is symptomatic, and shows erythema with central ulceration?
Erosive lichen planus
What is this?
reticular lichen planus
What is this?
Reticular lichen planus
What kind of lichen planus is this?
erosive
What kind of lichen planus can cause this?
erosive
Drug-induced lichen planus lesions are typically found on the _____ ____ as non-healing ulcers.
lower lip
What three histologic features are seen in lichen planus?
- saw-tooth rete pegs
- Civatte bodies
- fibrinogen deposition in basement membrane
28% of women with ELP have ______ ______.
vaginal lesions
Lesions can be induced with what four things? (hint: STAY)
S - stress
T- trauma
A - advil (any NSAID though)
Y - yeast
What can occur in recipients of allogeneic bone marrow transplantations?
Graft Versus Host Disease (GVHD)
What happens in GVHD?
The grafted cells start attacking the host tissues because they’re freaked out they’re not in their own home.
What type of GVHD is usually seen within a month of procedure, but can be any time before 100 days?
Acute GVHD
What kind of GVHD either presents after 100 days from the procedure or is a continuation of acute GVHD?
chronic GVHD
Acute GVHD can mimic what other disease?
Toxic Epidermal Necrolysis
Chronic GVHD can mimic what two other diseases?
Systemic Lupus Erythematosis or Sjogren’s Syndrome
Acute GVHD develops in __% of patients, and chronic develops in __%.
acute = 50%
chronic = 70%
What is the most common collagen vascular or connective tissue disease in the US?
Lupus Erythematous
What sex has a higher incidence for systemic lupus erythematous?
Females 10:1 compared to males
What two heart conditions can affect SLE patients?
Pericarditis (most common)
Libman-Sacks Endocarditis (warty vegetations on valves)
Based on the characteristic Malar rash on this patient’s face, what does she have?
systemic lupus erythematosus
Intraoral lichenoid lesions are seen with what systemic disease?
systemic lupus erythematosus
Patient also has fatigue, arthritis, and malar rash. What is your diagnosis?
systemic lupus erythematosus
What is the most common cause of death in patients with SLE?
Renal failure
What presents with Raynaud’s phenomenon, nasal alar atrophy, and clawlike fingers?
Systemic sclerosis
In systemic sclerosis, most organ systems have excess fibrin deposition in them. What is the main cause of death in patients with Systemic Sclerosis?
Heart failure due to Pulmonary HTN (from fibrosis)
Since fibrosis occurs across the whole body in systemic sclerosis, would you expect to see a patient have microstomia?
Yes, because there will be fibrosis of perioral tissues
Related to systemic slcerosis, what is it called if theres fibrosis to only a localized patch of skin?
Localized scleroderma
En coupe de sabre (“strike of the sword”) is characteristic of waht disease?
Localized scleroderma
What does CREST stand for?
C – Calcinosis cutis
R – Raynaud’s phenomenon
E – Esophageal dysfunction
S – Sclerodactyly
T – Telangiectasia
What are multiple, movable, nontender, subcutaneous, nodular calcifications called? (hint: the C in crest)
Calcinosis Cutis
This blanching is called what? (hint: happens due to stress or cold)
Raynaud’s phenomenon
What causes esophageal dysfunction in CREST syndrome?
abnormal collagen deposition in esophageal submucosa
What is it called when fingers undergo permanent flexure, and skin becomes smooth and shiny?
Sclerodactyly
What is this condition called? What two diseases is it seen with?
- Sclerodactyly
- Systemic sclerosis
- CREST Syndrome
What is the name for numerous scattered red papules most frequently found on the vermilion zone of lips & facial skin?
Telangiectasia
Do telangiectasias blanch?
Yes, it indicates the red color is due to blood contained within blood vessels
What is this? (hint: it is the T in CREST)
telangiectasia
What disease is characterized by the development of a velvety, brownish alteration of the skin?
Acanthosis Nigricans
Benign acanthosis nigricans caused by obesity is termed what?
Pseudoacanthosis Nigricans
Acanthosis nigricans is seen in conjuction with what pathology?
GI cancer
What is this?
Acanthosis Nigricans
What can this be in conjunction with?
GI cancer (pt shows acanthosis nigricans)
