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OPT Derm
| Term | Definition |
|---|---|
| ectodermal dysplasia | inherited disorders of missing skin, hair, sweat glands, salivary glands, nails and teeth; has 200 subtypes |
| hypohidrotic ectodermal dysplasia | X-linked subtype of ectodermal dysplasia; heat intolerance, fine blonde hair, hypodontia and pointed crowns |
| white sponge nevus | inherited keratin defect of buccal mucosa bilaterally; diffuse white thickened plaques |
| hereditary benign intraepithelial dyskeratosis (HBID) | inherited skin disorder with lesions similar to white sponge nevi in addition to ocular lesions |
| Darier disease (keratosis follicularis) | AD disorder due to abnormal desmosomes; prussic patches on trunk and scalp with bad odor and cobblestone appearance of white lesions |
| warty dyskeratoma | isolated lesion like Darier disease but is unrelated; small papule on head and neck |
| Peutz-Jeghers syndrome | freckle-like lesions of hands, lips, and oral mucosa with intestinal polyposis; {intestinal polyps}- hamartomatous polyps concentrated in the small intestine, telescoping of intestines |
| hereditary hemorrhagic telangiectasia (HHT) | vascular hamartomas of skin and mucosa, spontaneous nosebleeds; pts develop aneurysms of lungs, liver, brain and brain abscesses occur due to right to left shunt -consider antibiotic prophylaxis for dental treatments |
| Ehlers-Danlos syndrome | inherited CT disorders due to abnormal collagen; skin elasticity, bruising, flexibility -Gorlin sign- touch tongue to nose |
| tuberous sclerosis | hamartomatous growths in brain (tubers); cause mental retardation, seizures and angiofibromas on face -can cause enamel pitting on facial of anterior teeth, fibrous papules and gingival hyperplasia |
| Cowden syndrome | multiple hamartomatous growths (trichilemmoma) on skin of head and neck, acral keratosis, palmar/plantar keratosis, high risk for malignancies |
| epidermolysis bullosa | group of disorders of mucocutaneous blistering due to an defect in attachment; 4 types: simple, junctional, dystrophic and hemidesmosomal (Kindler syndrome) |
| direct immunofluorescence | detects tissue-bound antibodies |
| indirect immunofluorescence | detects circulating antibodies |
| pemphigus vulgaris | blisters of skin and oral mucosa due to autoantibodies against {desmoglein 3} and {desmoglein 1}, components of {desmosomes} that bind epithelial cells to each other -oral lesions usually precede skin lesions, most often in posterior palate |
| positive Nikolsky sign | creation of new vesicles in pemphigus vulgaris by applying lateral pressure on normal skin -{intraepithelial} separation occurs above basal layer |
| Tzanck cells | free-floating rounded spinous cells seen in pemphigus vulgaris |
| Rituximab | monoclonal antibody that targets B-cells, first line therapy for pemphigus vulgaris |
| paraneoplastic pemphigus | vere severe form of pemphigus seen in patients with malignancies (especially leukemias and lymphomas) that develops more quickly |
| mucous membrane pemphigoid | chronic blistering disease due to autoantibodies against basement membrane; more common than pemphigus -denuded mucosa and ulceration can be diffuse or limited -ocular involvement is most serious complication, can lead to blindness |
| desquamative gingivitis | gingival involvement of MMP; seen also in lichen plans, pemphigus and other ulcerative diseases |
| subepithelial | type of separation of epithelium from CT that occurs at the level of the basement membrane |
| anti-laminin 332 mucous membrane pemphigoid | form of pemphigoid associated with widespread involvement and risk of malignancy |
| bullous pemphigoid | most common autoimmune blistering condition; autoantibodies attack hemidesmosomes; bullae of skin with blisters containing eosinophils |
| erythema multiforme | acute, immunologic disease that occurs after recent infection; vesiculobullous process of skin that lasts 2-6 weeks and goes away |
| erythema multiforme minor | skin lesions with or without oral ulcers; characteristic target lesions |
| erythema multiforme major | widespread skin lesions with 2 or more oral mucosal sites |
| Stevens-Johnson Syndrome | oral, ocular, and genital lesions triggered by a drug reaction; seen mostly in young adults |
| toxic epidermal necrosis (TEN) | oral, ocular, and genital lesions triggered by a drug reaction; seen in older people, more severe than SJS |
| erythema migrans | benign inflammatory condition usually on dorsal tongue, taking off the fungiform papillae |
| Munro abscesses | collections of neutrophils concentrated in upper spinous layer of epithelium in erythema migrans |
| lichen planus | dermatologic disease of unknown etiology on oral mucosa; lace-like white lines {Wickham's striae} -lesions seen bilaterally and change patterns over time |
| lichenoid drug reaction | medication-induced lesions that look like lichen planus |
| lichenoid foreign body gingivitis | lesions that look like lichen planus but isolated to gingiva and non-migratory; often due to dental materials |
| reticular lichen planus | asymptomatic, involving posterior buccal mucosa bilaterally with interlacing white striae; most common type of LP |
| erosive lichen planus | presents with tissue-damage and pain, ulcers with white periphery and can be confined to gingiva {desquamative gingivitis} |
| aqueous tacrolimus rinse | rinse that can manage erosive LP by inhibiting T-cell activation |
| chronic ulcerative stomatitis | immune-mediated mucosal disease that mimics erosive lichen planus, antibodies against delta-Np63alpha -presents with desquamative gingivitis, ulcers on tongue and buccal mucosa; tongue can look like {cracked river bed} appearance |
| hydroxychloroquine | most effective drug for chronic ulcerative stomatits; anti-malarial |
| graft-versus-host disease | occurs in marrow transplant patients when transplant tissue tries to destroy host tissue; lichenoid white striae, ulcers, salivary gland destruction -{acute GVHD} occurs in first few weeks -{chronic GVHD} occurs after 100 days |
| psoriasis | activated T-cells create white scaly plaques on scalp, elbows, and knees |
| Auspitz sign | when plaque is peeled off, leaves small pinpoint bleeding point; seen in psoriasis |
| lupus erythematosus | immune-mediated systemic disease that affects collagen vasculature and CT; oral lesions like lichen planus and deep infiltration of inflammatory cells, kidney failure is most common cause of death |
| systemic lupus erythematosus | multi system disease affecting skin, oral cavity, kidney and heart; fatigue, weight loss, arthritis, butterfly rash |
| chronic cutaneous lupus erythematosis | mostly affects skin and oral mucosa with fewer systemic issues; exhibits discoid lupus erythematosus |
| discoid lupus erythematosus | skin changes in lupus, disc-shaped scaly patches on head and neck; exacerbated by the sun |
| subacute cutaneous lupus erythematosus | condition between systemic and chronic cutaneous lupus with non scarring lesions |
| systemic sclerosis / scleroderma | immunologic disease of deposition of dense collagen in skin and organs; primary cause of death: pulmonary hypertension and heart failure from pulmonary fibrosis |
| Raynaud's phenomenon | first sign of scleroderma, vasoconstriction triggered by stress or cold |
| morphea | localized scleroderma, sometimes called linear scleroderma |
| Crest syndrome (acrosclerosis) | mild form of systemic sclerosis, confirmed by presence of anticentromere antibodies; calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasis |
| anticentromere antibodies | presence confirms diagnosis of Crest syndrome |
| acanthosis nigricans | velvety brown pigmentation, especially in axilla; indicative of internal malignancy |
| benign acanthosis nigricans | brown pigmentation seen in association with diabetes, Addison's disease, medications and obesity |
| pseudoacanthosis nigricans | acanthosis nigricans associated with obesity |
| malignant acanthosis nigricans | brown pigmentation seen with internal malignancy, especially GI adenocarcinoma |
Created by:
aharnold
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