Chapter 16 Dermatologic Diseases Flashcards
What is a group of inherited conditions in which two or more ectodermal derived anatomic structures fail to develop?
-Ectodermal dysplasia
What are some common things that fail to develop in ectodermal dysplasia?
- Skin
- Hair
- Nails
- Teeth
- Sweta glands
What is the best known type of Ectodermal Dysplasia?
-Hypohidrotic ectodermal dysplasia
T/F Hypohidrotic ectodermal dysplasia is an X-linked female predominant disease
False
-Male predominant
What type of hair does a person with hyphohidrotic ectodermal dysplasia?
- Fine
- Sparse hair
What are the shape of teeth in someone with ectodermal dysplasia?
-Conical shaped
What is white sponge nevus?
-Genodermatosis a genetically determined skin disorder
White sponge nevus has a defect in what keratins?
- 4
- 13
T/F White sponge nevus is autosomal recessive
False
-Autosomal dominant
What age do white sponge nevus lesions appear first?
-Birth or early childhood
What does a white sponge nevus look like?
-Symmetrical thickened white corrugated diffuse plaque on the bilateral buccal mucosa
Where do you see a Warty Dyskeratoma?
-Skin or oral mucosa
What is Peutz-Jeghers Syndrome characterized by?
- Freckle-like lesions of the hands, perioral skin and oral mucosa
- Intestinal polyposis
- Predisposition for affected patients to develop cancer
If the intestinal polyps are premalignant what syndrome is that associated with?
-Gardner’s syndrome
Patients with Peutz-Jeghers syndrome have a problem with intestinal obstruction due to what?
-Intussusception
T/F Pts are about 18x more likely to have a malignancy in their lifetime if diagnosed with Peutz-Jeghers syndrome
True
What are the oral lesions of Peutz-Jeghers syndrome?
-1 to 4 mm brown macules
where do the the oral lesions of Peutz-Jeghers occur?
- Labial mucosa
- Buccal mucosa
- Tongue
What are the CNS manifestations of Tuberous Sclerosis?
- Mental retardation
- Seizure disorders
- Potato-like growths called tubers
Besides the CNS manifestations what else do you see in Tuberous Sclerosis?
- Angiofribromas of the skin
- Ungual fibromas
- Shagreen patches
- Ash-leaf spot
What are facial angiofibromas?
-Smooth surfaced papules occuring primarily on the nasolabial fold area
What are ASh-leaf spots?
-Ovoid areas of hypopigmentation
What is a rare tumor of the heart muscle that is also associated with Tuberous Sclerosis?
-Rhabdomyoma
What are the oral manifestations with Tuberous Sclerosis?
- Developmental enamel pitting on the facial aspect of anterior permanent teeth
- Multiple fibrous papules
What are the four diseases of Pemphigus?
- Pemphigus vulgaris
- Pemphigus vegetans
- Pemphigus erythematosus
- Pemphigus foliaceus
What is the first sign of pemphigus vulgaris?
-Oral lesions
What are the autoantibodies directed against in pemphigus vulgaris (autoimmune)?
- Desmosomes (Desmoglome 3)
- Causes an intraepithelial split
What is a characteristic feature of pemphigus vulgaris?
-Positive Nikolsky sign
What is a nikolsky sign?
-You can induce a Bulla if firm lateral pressure is exerted
What are the loose cells called in Pemphigus vulgaris?
-Tzanck cells (these are also found in herpes)
The cells of the epithelium fall apart in pemphigus vulgaris what is that termed?
-Acantholysis
If you do a direct immunofluorescence in pemphigus vulgaris what is it positive for?
- C3
- IgG (both found within the epithelium
What do you use to treat Pemphigus vulgaris?
-Systemic corticosteroid
What is Epidermolysis bullosa acquisita associated with?
Type VII collagen of anchoring fibrils with a sub basement membrane split
