Chapter 16 Flashcards
What are a group of inherited conditions in which two or more ectodermal derived anatomic strictures fail to develop, usually involve the skin, hair, nails, teeth, sweat glands? What is the best known form of this that would have issues with Heat intolerance, fine/sparse hair, and teeth are markedly reduced in number and have abnormal crown shapes? Who is most predominant, what type of genetic linked trait is this?
- Ectodermal Dysplasia
- Hypohidrotic ectodermal dysplasia
- Male predominant, X-Linked
What is an autosomal dominant, defect in keratins (keratins 4, 13), lesions appear at birth or early childhood, symmetrical, thickened, white, corrugated, diffuse plaques on the bilateral buccal mucosa? This belongs to a category of genetically determined skin disorders called?
- White Sponge Nevus
- Genodermatosis
A system complex characterized by freckle-like lesions of the hands/perioral skin (resemble freckles, but don’t change with sun increase or decrease)/and oral mucosa, has Intestinal Polyposis, where patients have a problem with intestinal obstruction due to Intussusception,
and a predisposition for affected patients to develop cancer (18x more likely)?
- Peutz-Jeghers Syndrome
What is a system complex characterized by: CNS manifestations, Mental retardation (in 40%), Seizure disorders (in 75%), Potato-like growths (“tubers” in 85%), Angiofibromas of the skin (mostly on the face), Angiomyolipomas are found on the kidney, Ungual (or periungual) fibromas and Characteristic skin lesions? What are the two types of skin lesions? A rare tumor of the heart muscle, termed what is also common? What effect does this have on the teeth?
- Tuberous Sclerosis
- Shagreen patches and Ash-leaf spots
- Cardiac Rhabdomyoma
- Enamel pitting on the facial aspect of the anterior permanent teeth and Multiple fibrous papule.
What 4 disease are related to Pemphigus? Which is the most common of these, the oral lesions are usually the first to show and the last to go, autoantibodies are directed against the desmosomes (cell to cell attachment), there is a split within the epithelium (intraepithelial split), patients complain of oral pain and will exhibit multiple erosions throughout on intraoral examination, patients rarely report vesicle or bulla formation intraorally (due to early rupture because of the thin roof of the blisters), before steroids 90% of patients died and histologically has floating Tzanck cells? What is a Bulla that can be induced on normal-appearing skin if firm lateral pressure is exerted that is associated with this? What is a localized version of Pemphigus Vulgaris?
o Pemphigus vulgaris o Pemphigus vegetans o Pemphigus erythematosus o Pemphigus foliaceus - Pemphigus vulgaris - Nikolsky sign - Haley-Haley Syndrome
What is almost twice as common as pemphigus, are oral lesions begin as vesicles or bullae which eventually rupture and leave ulcerated mucosa, intraoral blisters (blood blisters) may be seen clinically, and most significant complication is the ocular involvement (can be identified through a slit-lamp examination through an Ophthalmologist)?
What is another name for this?
This can also cause adhesions of the eye called? Scarring from this which causes the eyelid to go inward is called what?
- Benign Mucous Membrane Pemphigoid
- Cicatricial Pemphigoid
- Symblepharons and Entropion
Blistering ulcerative mucocutaneous condition of uncertain etiopathogenesis, In 50% of cases the clinician can identify a preceding infection or exposure to a medication, Infection is usually herpes simplex or Mycoplasma pneumoniae; medications are usually antibiotics or analgesics, Prodromal symptoms (fever, headache, cough, sore throat) occur 1 week before onset, Disease lasts 2-6 weeks; 20% have recurrent episodes, and lesions become elevated and evolve into bulla with necrotic centers and a highly characteristic skin lesion that develops is a Target Lesion?
There are three forms of this, what are they and what makes them different?
- Erythema Multiforme
- EM minor, EM major (AKA Stevens-Johnson syndrome, usually triggered by a drug) and Toxic Epidermal Necrolysis (AKA Lyell’s disease, most severe form and always triggered by a drug).
What is a common condition primarily affecting the tongue, characteristically the lesions appear on the anterior 2/3 of the dorsal tongue, the lesions will heal in one area and then develop in a different area, there is an association with fissured tongue, most patients are asymptomatic but may experience burning when eating spicy foods?
What are two other names for this?
- Erythema Migrans
- Geographic tongue and Benign migratory glossitis
Chronic dermatologic disease that can affect the oral mucosa, may be due to medications/amalgam, skin lesions itch but the patient doesn’t usually scratch because they will hurt, Drug-induced LP lesions are typically found on the lower lip as non-healing ulcers?
Skin lesions are the 4 Ps what are they?
What are the two forms of oral lesions involved in this and what are the differences?
Lesions can be induced by what 4 things?
- Lichen Planus
- o Purple
o Pruritus
o Polygonal
o Papules - o Reticular: Much more common, Asymptomatic, usually seen as seen as Wickham’s striae.
o Erosive: Patients are symptomatic, lesions are atrophic, erythematous areas with a central ulceration. If confined to the gingiva, it is termed Desquamative Gingivitis. - S – Stress
- T – Trauma
- A – Advil (any NSAIDs, acetaminophen is ok)
- Y – Yeast
What is an example of an immunologically mediated condition which is the Most common Collagen Vascular or Connective Tissue disease in the US? What are the 3 types and common findings with each?
- Lupus Erythematous
- o Systemic lupus erythematous (SLE):
- Malar rash – 50% (butterfly rash)
- Kidney Failure*
- Heart complications
- Libman-Sacks Endocarditis: Associated with heart complications.
- Oral Lesions
o Chronic cutaneous lupus erythematous (CCLE)
o Subacute cutaneous lupus erythematous (SCLE)
A disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts, experience resorption of the terminal phalanges and flexion contractures to produce shortened, clawlike fingers/fingertip ulcerations, Nasal alae become atrophied resulting in a pinched appearance, if it gets to organs (Lung, Heart , Kidney, GI tract) which can lead to fibrosis and Microstomia?
What is the first sign of this disease, what is seen and what is it?
What is a a mild variant of systemic sclerosis that exists and Affects a solitary patch of skin, Lesions look like scars so the name en coup de sabre (“strike of the sword”) is used to describe them, Condition is typically purely cosmetic?
- Systemic Sclerosis
- Raynaud’s phenomenon: Vasoconstrictive event triggered by emotional distress or exposure to cold.
- Localized scleroderma
What is CREST syndrome and what are it’s characteristics?
- A system complex that is characterized by: C – Calcinosis cutis R – Raynaud’s phenomenon E – Esophageal dysfunction S – Sclerodactyly T – Telangiectasia