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OPT Derm

TermDefinition
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