chpt 16- dermatology ppt Flashcards
inherited defects of ectoderm ally derived anatomic structures
ectodermal dysplasia
affects the skin, hair, nails, teeth, sweat glands
ectodermal dysplasia
characterized by fine, sparse hair, hypodontia, heat intolerance, periocular hyper pigmentation, xerostomia
ectodermal dysplasia
tx for ectodermal dysplasia
- genetic counseling
- prosthetic replacement of teeth
Why would an ectodermal dysplasia patient have xerostomia
decreased salivary gland development
Tooth problems with ectodermal dysplasia
hypodontia
Cone shaped teeth
Benign, autosomal dominant inherited skin disorder manifesting as thick white buccal mucosa bilaterally, may be corrugated or velvety
White sponge nevus (Canon’s Disease)
Tx for white sponge nevus
none
Autosomal dominant condition seen in descendants of North Carolina Indians, with thick white plaques on the buccal mucosa(like white sponge nevus) and the conjunctiva
Hereditary Benign Intraepithelial Dyskeratosis
Histology Dr Siversky said to know for Hereditary Benign Intraepithelial Dyskeratosis
Epithelial clefting
What 3 diseases show intra-epithelial clefting
1) HBID
2) Darier’s Disease
3) Pemphigus Vulgaris
Autosomal dominant skin problem with erythematous puritic papules on the trunk and scalp with FOUL ODOR, dystrophic nails (ridged and split)
Keratosis follicularis (Darier’s disease)
What is the oral manifestations of Keratosis Follicularis/ Darier’s Disease
multiple white papules
How treat Keratosis Follicularis/ Darier’s Disease
Keratolytic agents or emollients
Histopathologically identical to Darier’s Disease , but manifests orally as a single white papule
Warty dyskeratosis
Where does the single white papule of Warty dsykeratoma appear
hard palate or alveolar ridge
Tx for warty dyskeratosis
Excision
Autosomal dominant with freckle like lesions in and around oral cavity, on the hands, and having intestinal polyps with a predisposition to go to adenocarcinoma
Peutz Jeghers
2 disorders that have intestinal polyposis
1) Peutz Jegers Syndrome
2) Gardner Syndrome