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Flashcards in Dermatologic Diseases Deck (57)
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1

hereditary condition of defective ectodermal development; over 170 subtypes, with the best known being hypohidrotic ectodermal dysplasia (X-linked); autosomal dominant and recessive forms are also present

ectodermal dysplasia

2

M>F; heat intolerance; sparse hair, dystrophic nails; wrinkled, hyperpigmented periocular skin; midface hypoplasia, protuberant lips; oral: xerostomia, hypodontia with malformed crown shapes

ectodermal dysplasia

3

autosomal dominant genodermatosis caused by mutations in keratin genes 4 and 13; the defect results in abnormal keratinization of the mucosa

white sponge nevus

4

bilateral, thickened, white corrugated plaques primarily affecting the buccal mucosa; most mucosal sites are also susceptible

white sponge nevus

5

autosomal dominant genodermatosis characterized by a lack of cohesion among the surface epithelial cells; mutation affects the integrity of the desmosomal complex; oral involvement is much more subtle or absent (15-50%)

darier's disease (keratosis follicularis)

6

erythematous, pruritic papules of the skin; pits and keratoses of the palms and soles; nails that feature longitudinal lines, ridges, or splits; oral lesions: multiple normal to white flat-topped papules; frequently seen on the palate or alveolar mucosa

darier's disease

7

suprabasilar acantholysis; "test tube" rete ridges; central keratin plug; corps ronds and grains

darier's disease

8

solitary lesion of unknown etiology; histologically similar to the lesions of Darier's disease; M>F, older patients; solitary umbilicated papule of the head and neck; oral lesions are pink or white and primarily involve keratinized mucosa

warty dyskeratoma

9

autosomal dominant condition caused by a defect in the LKB1 gene, which codes for a serine/threonine kinase; significance lies in the increased incidence of GI adenocarcinoma and other malignancies

Peutz-Jeghers syndrome

10

periorificial and intraoral freckling; intestinal polyps with intussusception; similar freckling affects the skin of the extremities in about 50% of patients; lesions do not wax and wane with UV exposure; GI polyps- benign overgrowths of intestinal glandular epithelium

Peutz-Jeghers syndrome

11

9% of patients will develop GI adenocarcinoma by 40 years of age; 33% by 60; overall 18-fold increased frequency of malignancy (breast, ovary, pancreas)

peutz-jeghers

12

autosomal dominant condition caused by a mutation in the endoglin (HHT1) or ALK-1 (HHT2) genes; characterized by numerous vascular hamartomas of skin and mucosa

hereditary hemorrhagic telangiectasia

13

numerous red papules which blanch upon diascopy; epistaxis; predisposition for arteriovenous fistulas of the lung, liver, and brain; superficial collections of thin-walled vascular channels

hereditary hemorrhagic telangiectasia

14

any 3 of the following criteria leads to diagnosis of _: recurrent spontaneous epistaxis; telangiectasias of the mucosa and skin; arteriovenous malformation involving the lungs, liver, or CNS; family history of HHT

hereditary hemorrhagic telangiectasia

15

heterogeneous group of connective tissue disorders; probably 7 categories of this condition currently recognized; autosomal dominant, recessive, and X-linked patterns recognized

ehlers-danlos syndromes

16

_ type of Ehlers-Danlos: (abnormal type V collagen) hyperelasticity of skin; cutaneous fragility; papyraceous scarring

classical

17

_ type of Ehlers-Danlos: no evidence of scarring; joint hyperextensibility

hypermobility

18

_ type of Ehlers-Danlos: (abnormal type III collagen) bruising; arterial rupture

vascular

19

oral signs of _: gorlin sign; bruising and bleeding during minor manipulation; pulp stones

Ehlers-Danlos

20

uncommon autosomal dominant syndrome caused by a mutation of the TSC1 or TSC2 (most common) genes; these genes probably have a tumor suppressor function; TSC2 mutation tend to have more severe expressions than TSC1

Tuberous Sclerosis

21

seizure disorders and mental retardation; facial angiofibromas; ungual fibromas; CNS tumors (tubers); shagreen patches; ash-leaf spots; cardiac rhabdomyomas and angiomyolipomas

tuberous sclerosis

22

oral manifestations: enamel pitting; fibrous papules, primarily of the anterior gingiva; radiolucencies of the jaws

tuberous sclerosis

23

Rare autosomal dominant syndrome caused by a mutation of the PTEN gene; the gene acts as a tumor suppressor; trichilemmomas; acral keratosis; palmoplantar keratosis; hemangiomas, xanthomas, and lipomas

cowden syndrome

24

females- fibrocystic disease and breast cancer, GU tumors; GI polyps; multiple oral papules; high arched palate

cowden syndrome

25

two of the following for diagnosis of _: facial trichilemmomas, multiple oral papules; acral keratosis

cowden syndrome

26

heterogeneous group of inherited blistering mucocutaneous disorders; autosomal dominant or recessive patterns, as well as an acquired form

epidermolysis bullosa

27

_ epidermolysis bullosa: keratin gene defect

simple

28

_ epidermolysis bullosa: hemidesmosome defect

junctional

29

_ epidermolysis bullosa: collagen VII defect

dystrophic

30

_ _ form of epidermolysis bullosa: vesicles/bullae that become ulcers and erosions that heal with scarring; gingival erythema, recession and reduction in vestibular depth

dominant dystrophic