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
What is the difference, as far as the intestinal polyps, between Peutz-Jeghers and Gardners Syndrome
Peutz-Jeghers has benign intestinal polyps while Garderns are going to go malignant
Treatment for Peutz-Jeghers
Monitor for intussusceptions or malignant transformation
characterized by brown/black freckle like lesions in and around the oral cavity –> perioral and intramural and circumoral pigmentation
Peutz Jegher
what percent of Peutz Jegher vs Gardner intestinal polyposis become malignant
PG: 2-3%
Gardner: 100%
Autosomal dominant conditions of multiple vascular hamartomas due to decreased blood vessel wall intregrity.
Hereditary Hemorrhagic telangiectasia
Another name for HHT
Osler-Weber-Rendu Syndrome
Associated with frequent epistaxis, telangiectasias that are intraoral, hands, feet, GI tract, GU tract and eye, includes iron deficiency anemia if severe
Hereditary Hemorrhagic telangiectasia
Genetic abnormalities that lead to abnormal collagen manifesting as hypermobile joints, elasticity of skin
Ehler’s Danlos
Oral manifestations of Ehlers-Danlos
- subluxation of TMJ
- Don’t respond well to surgery due to defective collagen Bruise and bleed easily
Chronic inflammatory disease causing white striations, papules or plaques on buccal mucosa, tongue and gingival, thought to be autoimmune related by CD8 T cells causing oral epithelial cells apoptosis
Lichen Planus
Demographics for Lichen planus
45-60 y.o (rare in children) Women 2x more than men
Is Lichen planus only caused by autoimmune response
No, there can be drugs whose side effects cause a Lichenoid drug reaction, however, that goes away when drugs are stopped. True Lichen planus is chronic
Oral manifestation of Lichen Planus
Striae of Wickham
skin manifestations of Lichen planus
purple puritic polygonal papules
When biopsying Lichen planus, do you biopsy the red or the white area
White area, red will just show ulceration (basement membrane with no epithelial covering)
Histology for Lichen Planus
saw tooth rete pegs and loose basal cell layer
Which phase of Lichen planus is treated: Reticular or erosive, and with what
Erosive. Corticosteroids because it is autoimmune mediated
What can superimpose on top of Lichen planus especially during corticosteroid treatment of the Erosive form
candidiasis
Dislodgement of skin by lateral pressure, can be a symptom in multiple diseases
Nikolsky sign
2 disease we have been taught that have a positive Nikolsky sign
Pemphigus
Pemphigoid
Histologically, which has a change in the basement membrane: Pemphigoid or pemphigous
Pemphigoid (goid goes down to the basement)
Histologically, which has intraepithelial separation: Pemphigoid or pemphigous
Pemphigus
Which has larger lesions Pemphigoid or pemphigous and why
pemphigoid, because they are thicker (all the way to the basement)
Pemphigoid type that affects women over the age of 60 manifesting orally as vesicles or bullae
benign mucous membrane pemphigoid
What are the clinical sizes of the benign mucous membrane pemphigoid vesicles and bullae
vesicles are less than 5mm
Bullae are greater than 5 mm
What is the histology for benign mucous membrane pemphigoid (Siversky says to be able to ID this for the fina
subepithelial clefting Immunofluorescence at basement membrane
Most commonly used antibiotics for treating benign mucous membrane pemphigoid
Tetracycline Niacinamide Nicotinamide
If going to treat someone with benign mucous membrane pemphigoid with Dapsone, what must you check first
that they are not Glucose-6-Phosphate dehydrogenase deficient. Can cause aplastic anemia
Most common of the autoimmune blistering conditions
bullous pemphigoid
