Chapter 16: Dermatologic Diseases I Flashcards
___ is a group of conditions in which two or more ectodermal derived anatomic structures fail to develop; what are the structures?
- ectodermal dysplasia
- skin, hair, nails, teeth, sweat glands
there are over ___ types of ectodermal dysplasia
170
what is the best known type of ectodermal dysplasia?
hypohidrotic ectodermal dysplasia
describe hypohidrotic ectodermal dysplasia
- X-linked; male predominance
- heat intolerance
- fine, sparse hair
- periocular wrinkling with hyperpigmentation
- dystrophic or brittle nails
- hypoplastic or absent salivary glands
- teeth are markedly reduced in number and have abnormal crown shapes
what is the treatment for hypohidrotic ectodermal dysplasia?
treatment includes genetic counseling and various dental prosthetics
hypohidrotic ectodermal dysplasia
___ is a rare genodermatosis/genokeratosis (a genetically determined skin disorder) that is inherited as an autosomal dominant
white sponge nevus
white sponge nevus is caused by a defect in ___
keratins 4 and 13
when do white sponge nevus lesions appear?
at birth or early childhood
describe the clinical presentation of white sponge nevus lesions
- symmetrical, thickened, white, corrugated, diffuse plaques on the bilateral buccal mucosa
- may also occur in other parts of the oral cavity
is white sponge nevus symptomatic or asymptomatic?
asymptomatic
what is the treatment for white sponge nevus?
it is a benign condition that does not require treatment
white sponge nevus
___ is a rare but well-recognized autosomal dominant condition characterized by freckle-like pesions of the hands, perioral skin, and oral mucosa, and intestinal polyposis?
peutz-jeghers syndrome
patients with peutz-jeghers syndrome have a predisposition to develop ___
cancer
describe the skin lesions associated with peutz-jeghers syndrome
- develop in childhood and involve periorificial areas
- resemble freckles, but do not wax and wane with sun exposure
describe the intestinal polyps associated with peutz-jeghers syndrome
- hamartomatous growths
- not premalignant
- scattered throughout the mucous-producing areas of the GI tract
patients with peutz-jeghers syndrome also have a problem with intestinal obstruction due to ___
intussusception
“telescoping” of a proximal segment of the bowel into the distal portion
patients with peutz-jeghers syndrome are ___ times more likely to develop a malignancy in their lifetime; describe the malignancies
- 18x
- GI adenocarcinoma also develops (but not from the polyps)
- other tumors such as breast cancer
describe the peutz-jeghers syndrome oral lesions
- an extension of the perioral freckling
- 1-4mm brown macules
- primarily affect the labial mucosa, buccal mucosa, and tongue
- seen in 90%
what is the treatment of peutz-jeghers syndrome?
monitoring for development of intussusception or tumors
peutz-jeghers syndrome
tuberous sclerosis is a system complex characterized by what 7 things?
- CNS manifestations
- mental retardation
- seizure disorders
- potato-like growths (“tubers”)
- angiofibromas of the skin
- ungual (or periungual) fibromas
- characterstic skin lesions
- shagreen patches
- ash-leaf spots
describe the facial angiofibromas associated with tuberous sclerosis
multiple, smooth-surfaced papules occurring primarily on the nasolabial fold area