midterm3 Flashcards
most common benign “tumor” of oral cavity. Reactive hyperplasia (not neoplasia) of fibrous CT in response to local irritation or trauma. Most commonly seen along bite line. Grows as long as you keep trauma on it. Normal colored, sessile (non-mobile), bump. TX: cut it out, get it biopsied
Fibroma
Fibrous CT tumor, not associated with chronic irritation. Predilection for gingiva. (retrocuspid papilla: is bilateral and behind mandibular canines). Have a papillary surface. Occurs at younger age
Giant cell fibroma
tumor-like hyperplasia of fibrous CT. Epulis Fissuratum is name for IFH associated with flange of ill-fitting denture. Usually develops on facial aspect of alveolar ridge. TX: surgical excision and remake denture
Inflammatory Fibrous Hyperplasia
reactive tissue growth that develops under a denture (from bacteria: wearing the denture 24/7). Typically occurs on hard palate. Asymptomatic erythemous tissue with a pebbly or papillary surface. TX: remove denture, antifungal, excision
Inflammatory Papillary Hyperplasia
not a true granuloma. Reactive lesion to local irritation or trauma (poor oral hygiene). Most common on gingiva (75%), then lips, tongue, buccal mucosa. Children and young adults. Typically pedunculated, lobular mass, that is red, ulcerated, and bleeds easily. Frequently in pregnant women (pregnancy tumor).
Pyogenic Granuloma
reactive lesion cause by local irritation or trauma. Occurs exclusively on gingiva or edentulous alveolar ridge. Blue, purple, red. May produce “cupping” resorption of underlying bone. 10% recur after excision. Average age is 35
Peripheral Giant Cell Granuloma
exclusively on gingiva. Pink (normal color), or pale and hard. Average age is 15. 2/3 in females.50% in incisor/cuspids area. 15% recur
Peripheral Ossifying Fibroma
benign tumor of fat. Most common mesenchymal (non-epithelial) neoplasm. Soft smooth nodular mass. Yellow or mucosal colored. 50% in buccal mucosa. Older than 40. Floats in formalin.
Lipoma
Traumatic neuroma (proliferation of neural tissue after injury [not neoplasm], common in mental foramen area, 1/3 is painful. TX surgical excision) Palisading encapsulated neuroma (benign neural tumor)
Neuroma
benign neural tumor of Schwann cell origin. Slow growing encapsulated nerve tumor. Tongue is the most common origin. Bilateral Schwannoma is characteristic of neurofibromatosis type 2(chromosome 22, AD) Antoni A: spindle shaped Schwann cells organized (palisading) around verocay bodies- eosinophils. Antoni B: disorganized
Schwannoma
most common type of peripheral nerve neoplasm. Can arise as solitary tumor or be a component in neurofibromatosis. Slow growing, soft, painless lesion. Tongue and buccal mucosa. Evaluate for neurofibromatosis.
Neurofibroma
- Type 1: hereditary condition, termed von Recklinghausen’s disease of skin. Plexiform variant of NF is pathognomonic (feels like a bag of worms), most common NF. DX (2 or more) 1) six or more café au lait macules (big milk colored coffee macules. “c
Neurofibromatosis (Types 1 & 2):
narrow face with thick lips, marfinoid limb structure. Bilateral neuromas of the commissural mucosa. 50% develop pheochromocytoma (adrenal gland tumor), 90% get medullary carcinoma of the thyroid gland. Also tumors of parathyroid, pituitary, pancreas.
Multiple Endocrine Neoplasia, Type 2B
rare pigmented lesion, usually occurs in first year of life. More common in anterior maxilla. Black/blue. High urinary levels of vannillylmandelic acid. Benign.
Melanotic Neuroectodermal Tumor of Infancy
most common tumor of infancy. More common in white females. 60% on head or neck. Capillary (red and does not blanch) or cavernous (red to purple, blanches)
Hemangioma
not genetic developmental condition. Born with a dermal capillary vascular malformation known as “port wine stain” or “nevus flammeus”. Unilateral distribution along segments of the trigimenal nerve (not all that have this have disease). Involvement of opthalamic segment more likely to have the disease. Pts have leptomeningeal angiomas. Typically associated with a convulsive disorder (think phenytoin-drug induced gingival hyperplasia). May result in retardation and contralateral hemiplegia.
Sturge-Weber-Angiomatosis
benign tumor of lymphatic vessels. Capillary, cavernous (mouth), or cystic (neck). Younger patients get diagnosed (under 2) common on anterior 2/3 of tongue (macroglossia) resembles frog eggs or tapioca pudding.
Lymphangioma
benign smooth muscle tumor. Common in uterus, GI, skin. 75% of oral cases are vascular variant. Asymptomatic, firm mucosal nodule. TX: Excision
Leiomyoma
(malignant counterpart): 60% of soft tissue sarcomas of childhood. Skeletal muscle. Face and orbit and males common. Painless, infiltrative mass that grows rapidly. Tx. Surgery and chemo
Rhabdomyoma
may spread by lymphatics, from lower part of body is probably blood borne. Bratson’s plexus: valve-less vertebral venous plexus that allows retrograde spread bypassing lungs. Gingiva is most common site for soft tissue (50%), then tongue (25%). Nodular hyperplastic growth. Carcinomas rather than sarcomas.
Metastases to oral soft tissues
decreased in volume of RBCs. Often a sign of underlying disease. Symptoms: tired, headache, fainting/lightheadedness, pallor. Oral symptoms: pallor, bald tongue, burning/pain on tongue.
Anemia
decreased platelet count. Decreased production, increased destruction of sequestration in spleen. No clinical symptoms until under 100k, small capillaries leak (small=petechiae, ecchymosis, hematoma)- Thrombotic Thrombocytopenia pupura (ttp): endothelial damage causes a serious coagulation disorder. Bad news- refer to physician- Idiopathic thrombocytopenia pupura (itp): classically after a viral infection, resolves in 6 months
Thrombocytopenia
enlargement of tissue due to infection. Lymph nodes, weldeyer’s ring, or any lymphatic tissue. Acute: enlarged, tender, soft, freely movable nodules. Chronic: enlarged, non-tender, firm, freely movable nodules. Look for symmetry (might be normal). Posterior lateral tongue is commonly bilateral.
Lymphoid Hyperplasia
hematopoietic stem cell derivation: proliferated in bone marrow. Acute: aggressive and lead to death in a few months if untreated. Chronic is more indolent. Myeloid or lymphoid. CML associated with 9/22 translocation (Philadelphia). Environmental factors like: pesticides, benzene, ionizing radiation, HTLV-1 virus. ALL more common in children. CLL most common. Pts have decreased 02 carrying capacity, bruise easily. Boggy, ton-tender pus filled swelling in mouth.
Leukemia
antigen presenting cells- dendritic mononuclear cells found in epidermis, mucosa, lymph nodes, and bone marrow. Eosinophilic granuloma of bone: one or multiple lesions, no visceral involvement. Acute disseminate histiocytosis: visceral involvement, mostly in infants, (letterer-siwe disease). Chronic disseminate histiocytosis: bone skin and viscera (hand-schuller-christian disease). Pain and tender bone lesions in skull, ribs, vertebrae, and mandible (15%). XRAY: sharply demarcated punched out radiolucency, mandibular lesions appear scooped out, teeth look like they are “floating in air”.
Langerhan’s Cell Histiocytosis
malignant lymphoproliferative disorder. Neoplastic cells are reed-sternberg cells (typically binucleated). Linked to EBV. 75% occur in cervical or supraclavicular nodes. Bimodal age (15-35 and 50+). Persistently enlarging, nontender, discrete mass, in lymph node region, early they are movable but late are more secure. Nodular lymphocyte is more dominant and Classical has 5 histio subtypes. 15 years post tx mortality likely due to complications of tx.
Hodgkin’s Lymhoma
malignancy of plasma cell origin. If metastatic disease is excluded this accounts for 50% of malignancies. Symptoms result from uncontrolled proliferation of cells and protein products- bone pain, most common is spine) Average age is 65. Most common hematologic malignancy in African Americans. XRAYS: multiple well defined punched out RL, may be ragged, may be evident on skull film. Renal failure from excess proteins: termed bence jones proteins. Bisphosphonates given to reduce fractures
Multiple Myeloma
“marble bone disease” increased density of bone, defect in remodeling caused by a failure of normal osteoclast function. Marrow failure (infantile), frequent fractures (nutrients go away). Really white radiograph. Adult is mild and have long term survival. Infantile does not
Osteopetrosis
clavicle hypoplasia. Short stature, frontal bossing, ocular hypertelorism, broad nose, supernumerary teeth, prolonged retention of primary teeth.
Cleidocranial Dysplasia
area of hematopoietic marrow that produces a radiolucency. NOT pathology but looks like one. Typically asymptomatic, ill-defined borders with central trabeculations. Most common in adult females
Focal Osteoporotic Bone Marrow Defect
Hardening of bone with no known cause (not by a massive restoration). Arise in teenage years and stays static. XRAY: well definied, elliptical/round RO, usually associated with root apex, NO RL rim, 2-3mm. Biopsey only if continued growth , symptoms, expansion.
Idiopathic Osteosclerosis
abnormal resorption and deposition of bone, bone pain common, weakened bones. Older people, more males. Forms near joints and promotes osteoarthritic changes (joint immobility). Increases circumference of skull (hat/denture doesn’t fit anymore). More common in max (enlargement of middle 1/3) than mand. XRAY: patchy sclerotic areas, cotton roll appearance, hypercementosis. Pts have elevated serum alkaline phosphatase with normal calcium and phosphorus levels. Tx: NSAIDs and bisphosphonates. Can cause an osteosarcoma.
Paget’s Disease of bone
considered non-neoplastic lesion (plateaus). Most common in anterior jaw and crosses midline. Most are asymptomatic. XRAY: not diagnostic, RL multi or unilocular, well delineated, non-corticated margins. Tx: curettage with 20% chance of regrowth. Good long term prognosis, no chance of metastasis
Central Giant Cell Granuloma
AD. Bilateral involvement of posterior mandible (chubby cheeks), eyes turned up to heaven (wide rim and stretched skin). Ages 2-5. After puberty it slowly recedes. XRAY: multilocular, expansile, RL, usually bilateral. Unpredictable prognosis: may go away, may have complications.
Cherubism
scalloped radiograph with clinical picture of empty cavity. Benign empty or fluid filled space. Not a true cyst due to no epithelial lining. Trauma and hemorrhage theory. Frequently in mandible of teens. Curettage to fill up with blood
Traumatic Bone Cyst
full of spongey material (CT) and blood. Not a true cyst. In mandible of 20 y/o. XRAY: RL with marked cortical expansion and thinning. Usually unilocular, variable borders, described as blow out or ballooning distension (of posterior inferior mandible) . “Blood soaked sponge”
Aneurysmal Bone Cyst
tumor like condition, bone gets replaced with fibrous CT interspersed with bone. Sporadic condition resulting from a postzygotic mutation. Tends to stabilize at skeletal maturation. Orange peel on xray1) Monostotic: one bone. 80% of cases. Teenage jaws. “ground glass” RO, results from superimposition of poorly calcified bone in disorganized fashion. Margins blend into rest of bone. Maxilla leads to obliteration of maxillary sinus.2) Polyostotic: more than 1 bone. • Jaffe-Lichtenstein: Café au lait (coast of maine-ragged)• McCune Albright syndrome: café au lait (coast of maine), multiple endocrinopathies, sexual precocity, pituitary adenoma, and or hyperthyroidism.
Fibrous Dysplasia
occurs in tooth bearing areas of jaws. 1) Focal cemento-osseous dysplasia: single site of involvement. 90% in females. About 40 years old. Posterior mandible lesion smaller than 1.5 cm. XRAY: RL to RO (usually mixed), thin RL rim, well defined. 2) Periapical cemento-osseous dysplasia: involves periapical area of anterior mandible. Multi focal. PDL stays intact (lesion doesn’t fuse with teeth). Mainly women, blacks. XRAY: circumscribed areas of RL involving apex of tooth, progress to mixed RL and RO, end RO with RL rim. 3) Florid cemento-osseous dysplasia: multiple focal involvement, not limited to anterior mandible. Females and blacks. Bilateral and symmetrical. Same maturation as before
Cemento-osseous Dysplasia
true neoplasm (unlimited growth potential) composed of fibrous tissue that contains a variable of bone and cementum. XRAY: well defined, typically unilocular, mixed RL-RO, affects the roots (resorption), characteristic downward bowing of the inferior cortex of the mandible. Easily enucleated.
Ossifying Fibroma
rapidly growing, well-circumscribed, RL with central RO. More in males, and maxillary. 2 types: trabecular and psammatoid
Juvenile (Active) Ossifying Fibroma
benign tumors of mature bone (look like tori). Surface of bone or within medullary bone. Pain, swelling, sinusitis, or nasal discharge. In compact bone RO, in cancellous it’s mixed. XRAY: circumscribed masses, exhibit continued growth
Osteoma
bowel polyps (develop during teenage years, will become adenocarcinoma- get removed as they come up), skeletal abnormalities (osteomas common), dental abnormalities (supernumerary teeth, impacted, odontomas), epidermoid cysts
Gardner Syndrome
closely related benign bone tumors. Arise from osteoblasts. Non-tooth bearing areas. • Osteoblastoma (2-4 cm, pain, tenderness, and swelling, aspirin does not relieve pain (too big). XRAY: RL with varying degrees of central RO• Osteoid osteoma: nocturnal pain, relieved by aspirin. Well circumscribed, RL defect, less than 2 cm (usually about 1 cm), small RO nidus resulting in a target like appearance.
Osteoid Osteoma/ Osteoblastoma
75% arise in premolar area of mandible. Typically before age 30 with pain and swelling. XRAY: RO mass fused to roots, outline of root/roots is obscured, surrounded by thin RL rim.
Cementoblastoma
mesenchymal malignancy. Excluding hematopoietic neoplasms (leukemia, Langerhans ect) it is the most common type of malignancy to originate within bone. Intramedullary, juxtacortical, or extraskeletal (soft tissue). Bimodal: between 10-20 and above 50. Most common in distal femur or proximal tibia. Mandible is more common in posterior and maxillary is more common inferior (palate alveolar ridge, (not eyes)). XRAY: ill defined border, spiking resorption (root resorption causes narrowing), classic sunburst appearance, triangle elevated periosteum (codman’s triangle), early finding is symmetrical PDL widening.
Osteosarcoma
metastatic cancer is most common form of cancer involving bone. Most common origin for gnathic mets: Females: breasts and lungs, males: lung and prostate. Numb chin syndrome: metastasis involves inferior alveolar nerve. XRAY: ill-defined borders (“moth eaten”), widening of PDL, Some may stimulate new bone formation (mixed RL-RO)
Metastatic Tumors to Jaws
cyst that originates from the separation of the follicle from the crown of an uninterrupted cyst. Fluid accumulates between reduced enamel epithelium and tooth crown. Most common developmental cyst. Most often in 3rd molars. Can grow to considerable size and expand bone. XRAY: unilocular RL associated with crown of uninterrupted cyst. TX: enucleate along with tooth
Dentigerous Cyst
soft tissue analog of dentigerous cyst. Soft translucent swelling in the gingiva mucosa overlying the crown of an erupting tooth. Surface trauma results in blood (blue/black).
Eruption Cyst
30% recurrence rate. Associated with a syndrome. Arise from cell rests of the dental lamina. Posterior mandible, tend to grow in anterior-posterior direction. Does not cause bone expansion. XRAY: smaller lesions: well-defined, unilocular RL with corticated margins. Larger: multilocular. Cheese like filling- pop like a pimple. Friable margins, test for Gorlin’s syndrome. Palisading basal cell layer on histo, made from parakeratin. (orthokeratinizeed odontogenic cyst is irtho and no palisading layer)
Odontogenic Keratocyst
AKA Gorlin’s syndrome. Chromosome 9 PTCH gene. Multiple basal cell carcinomas (around puberty on skin not associated with sunlight), odontogenic keratocycts, calcification of the falx cerebri, rib anomalies,
Nevoid Basal Cell Carcinoma Syndrome
soft tissue counterpart of lateral periodontal cyst. Derived from rests of serres. Predilection for mandibular canine and premolar region. Facial gingiva or alveolar mucosa, painless domelike swellings which are less than 5mm.
Gingival Cyst of the Adult
intrabony counterpart of gingival cyst of the adult. Predilection for mandibular canine-premolar-lateral central area. XRAY: well circumcised lesion on lateral root surface, multilocular/grape like. Teeth are vital
Lateral Periodontal Cyst
AKA Gorlin’s cyst. 65% are in incisor/canines. Mx=md. Average age 35. XRAY: unilocular, well defined RL with calcified flecks. 1/3 in unerrupted tooth
Calcifying Odontogenic Cyst
buccal aspect of mandibular first permanent molar. 1/3 is bilateral. XRAY: well circumscribed, unilocular radiolucency involving buccal bifurcation. 1.5 cm. root apices are tipped toward the lingual mandibular cortex. Perio probing reveal pocket formation on buccal aspect (enamel extension)
Buccal Bifurcation Cyst
most common clinically significant odontogenic tumor. Conventional solid or multicystic (85%), unicystic (15%), peripheral (1%). Equal prevalence from 3-7 decade. 85% in mandible (molar ascending ramus area). Can grow to grotesque sizes. XRAY: “soap bubble” when RL loculations are large. “Honeycombed” when they are small. Buccal and lingual cortical expansion is present. Resorption of roots common. 6 different patterns. (desmoplastic ameloblastoma does not share features with the other forms: predilection for anterior maxilla, mixed RO-RL, due to osseous dysplasia) palisading and reverse polarity on Histo. Tx. Simple enucleation and curettage to en bloc resection.
Ameloblastoma
young patients (10-20) Anterior jaws, mx>md, Asymptomatic and discovered during xray to see why tooth hasn’t erupted. 75% of time it’s a circumscribed unilocular RL involving the crown of an unerrupted tooth (most often canine). XRAY: fine snowflake calcifications (helps ddx from dentigerous). Enucleation easy.
Adenomatoid Odontogenic Tumor
AKA pindborg cyst. M=F, md>mx, posterior>anterior. Older aged. Painless slow growing swelling. XRAY: uni or multilocular, margins are scalloped and well defined, corticated or ill-defined, frequently associated with impacted tooth, calcifications around crown in “driven snow” pattern. Amyloid like ECM, congo red, green under polarized light. Calcifications with concentric rings in the amyloid like areas (liesgangs ring).
Calcifying Epithelial Odontogenic Tumor
Most common odontogenic tumor. (most common clinically significant is ameloblastoma). Developmental abnormality (hamartomas) rather than true neoplasm. Compound (multiple, small tooth like structures) and complex (conglomerate mass of enamel and dentin, bears no anatomic resemblance to a tooth.) Average age 15. Completely asymptomatic. Discovered when XRAY taken to see why tooth hasn’t erupted. Compound more common in anterior maxilla. Complex more common in molar region. Tx. excision
Odontoma
2 or more ectodermal anatomic structures fail to form. Skin, hair, nails, teeth, sweat glands. Best known is hipohidrotic ectodermal dysplasia. X linked, male prevalence, fine sparse hair. Small pointy teeth.
Ectodermal Dysplasia
genetically determined skin lesion (genodermatosis). AD. Defect in keratins 4,13. Lesions appear at young age/birth. Symmetrical thickened white corrugated diffuse papules on the bilateral buccal mucosa. Benign asymptomatic with no treatment. Biopsy to check
White Sponge Nevus
rare. Freckle like lesions of hands, perioral skin, and oral mucosa, and anus. Intestinal polyps (not malignant). Predisposition for affected people to get cancer (18x). Intestinal blocking due to intussusception (telescoping of the proximal segment of the bowel into the distal portion). Monitor for intussusception and cancer.
Peutz-Jeghers Syndrome
Facial angiofibromas (multiple smooth surface papules occurring primarily on nasolabial fold area). Ungual fibromas (same as angiofibromas but around the nails). Shagreen patches (CT harmartomas- is a benign (noncancerous) tumorlike malformation made up of an abnormal mixture of cells and tissues found in areas of the body where growth occurs). Ash-leaf spots (ovoid areas of hypopigmentation), tubers (75%), seizure (75%- think phenytoin and gingival hyperplasia), mental retardation. Rarely see rhambomyoma (heart tumor), angiomyolipoma (kidney). Developmental enamel pitting on anterior permanent teeth, fibrous oral papules.
Tuberous Sclerosis
oral lesions are first to show and last to go. Autoantibodies directed at desmosomes. (cell to cell), result in a split in epithelium. Ulcers in oral tissue and other epthelium. Skin lesions are flaccid bullae that rupture quickly (rupture when speaking or eating in mouth). Get sepsis without tx. Positive Nikolsky sign (firm lateral pressure causes bullous, spraying air separates tissue. Direct immunofluorescence is positive for c3 and IgG between epitheliual layers. Indirect immunofluorescence correlates with disease activity. Systemic corticosteroids and immunosuppressive drugs. 75% have long term resolution (hayley-hayley disease: familial localized pemphigus)
Pemphigus Vulgaris
oral vesicles that rupture, but able to see before rupture. Intraoral blood blister is pathognomonic. Oral lesions dont scar. Up to 25% ocular involvement, conjunctiva becomes inflamed and attempt of healing lead to scarring. Adhesions called symplepharons develop. Scarring turns eyelids in (entropion), closes lacrimal glands, keratin deposits.
Benign Mucous Membrane Pemphigoid
blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis (likely immune related). 50% of cases clinician can identify a preceding infection (herpes simplex or mycoplasma pneumoniae) or exposure to a medication (abx or analgesics). EM minor, EM major (stevens-johnson syndrome) and toxic epidermal necrolysis (lyell’s disease). Acute onset with wide spectrum. Acute=oral lesions (erythemous patches that undergo necrosis and ucerate)[ entire oral cavity except gingiva and hard palate), chronic = all lesions (flat round red w necrotis center (bulls eye)). 20-30. Prodromal: fever, headache, cough, sore throat 1 week before onset. TX. Take off causing drug, antivirals for herpes, IV fluids. (steroids for minor and major but NOT TEN), Ten in burn unit.
Erythema Multiforme
erythema multiforme major. Triggered by drug, skin, oral mucosa, and either ocular or genital mucosa involvement.
Steven-Johnsons
most severe form. Almost always triggered by drug. Diffuse sloughing, older people, more females, if survive 2-4 weeks heal time.
Toxic epidermal necrolysis
common chronic dermatologic disease from medications, amalgam, ect. Female middle aged adults. 1% of population. Skin lesions (purple, pruritis (itchy), polygonal, papule (raised)) too painful to itch, have thin lacelike network of lines called wickham’s straie. Drug induced on lower lip as non-healing ulcers. • Reticular: much more common, asymptomatic, involves posterior buccal mucosa bilaterally, (wickhams straie), if on tongue It’s plaque like, lesions wax and wane. No need to biopsy• Erosive: symptomatic, atrophic, erythematous area with central ulceration. If confined to gingiva it’s called desquamative gingivitis. Biopsy. 28% have vaginal ulcer. Ulcers induced by stress, trauma, Advil (nsaid), yeast (STAY)
Lichen Planus
- F. immune mediated, collagen vascular or CT disease. Weight loss, arthritis, fatigue, maylar rash (butterfly, sunlight worsens it). Kidneys affected in 50%. Cardiac issues (pericarditis, warty vegetations on valves called libman-sacksendocarditis). 40% get lichenoid like oral lesions. • Chronic cutaneous lupus erythemous: lesions on skin and mucosal surface. Begin as scaly erythematous patches on sun exposed skin, especially head and neck. Heal and recur. Leads to atrophy, scarring, and altered pigmentation. Oral lesions resemble lichen planus• DIF of clinically normal skin will show IgM, IgG, or C3 at the Basal membrane zone (positive lupus band test). 90% have antinuclear antibodies. Give antimalarial drugs (may give blue palate)
Lupus Erythematosis
dense collagen is deposited in the tissues of the body in extraordinary amounts. F. disease of adults. Raynauds phenomena (vasoconstrict to emotional distress or cold- tips of fingers are pale and tingle like funny bone). Resorption of terminal phalanges and flexion to make claw-like fingers. Vascular events and collagen depositon contribute to fingernail lesions. Skin develops hard diffuse smooth surface. Subcutaneous collagen deposition make face taught and masklike. Fibrosis of lung, heart, kidney, GI tract. Microstomia. Xerostomia. Localized scleroderma-en coup de sabre. Waxes and wanes.
Systemic Sclerosis
women in their 60s. monitor for pulmonary hypertension• Calcinosis cutis: multiple, movable, nontender, subcutaneous, nodular calcifications• Raynauds phenomena: severe blanching of fingertips when in cold. Turn blue. Red.• Esophageal dysfunction: abnormal collagen deposition in esophageal submucosa• Sclerodactyly: fingers stiff, skin shiny, claw deformation, abnormal collagen deposition in dermis• Telengectaisis: scattered red papules. Blanch. Vermillion zone of lips and facial skin. Bleeding
CREST Syndrome
deposition of extracellular, proteinaceous substance called amyloid. Cause of macroglossia. Organ-limited (rarely seen in oral cavity, amyloid nodule [solitary, asympt, submucosal deposit], no association with any systemic condition), systemic- primary and myeloma associated (older adults, M, 20% due to multiple myeloma, carpals tunnel, mucocutaneous, hepatomegaly, smooth firm waxy nodules, eyelid, neck, lips.), systemic-secondary (result of chronic inflammatory process such as TB/sarcoidosis/osteomyelitis, spares heart but affects liver, kidney, spleen), systemic-hemodialysis associated (secondary, protein not removed by dialysis so it accumulates, then deposits into bone and joint, systemic-heredofamilial (uncommon AD, polyneuroopathies, heart issues and kidneys) most pts die from cardiac failure/ arrhythmia/ renal disease in a few years.
Amyloidosis
(retinol): essential for vision. May lead to blindness.
• Vitamin A
thiamin): maintain proper functioning of neurons. Beriberi
• Vitamin B1
riboflavin): necessary for cellular oxidation-reduction reactions. Oral alterations like angular cheilitis, glossitis.
• Vitamin B2
niacin): acts as a coenzyme for oxidation-reduction reactions. Pellagra= dermatitis, dementia, diarrhea.
• Vitamin B3
pyridoxine): cofactor associated with enzymes that participate in amino acid synthesis.
• Vitamin B6
ascorbic acid): necessary for the proper synthesis of collagen. Scurvy
• Vitamin C
considered a hormone, necessary for calcium absorption from the gut. Rickets, Osteomalacia.
• Vitamin D
α-tocopherol): antioxidant.
• Vitamin E
necessary for proper clotting (needed in clotting factors II,VII, IX, X). Coagulopathy because of inadequate synthesis or prothrombin.
• Vitamin K
• Most common cause of anemia in the U.S and the World• Develops from excessive blood loss, increased demand for RBC’s, decreased uptake of iron, decreased absorption of iron.• 20% women of childbearing years (menorrhagia) from chronic blood loss.• Oral manifestations: angular cheilitis, atrophic glossitis (bald tongue)• Laboratory finding: hypochromic microcytic RBC’s
Iron-deficiency Anemia
Iron deficiency anemia, glossitis, dysphagia. Associated with a high frequency of oral and esophageal squamous cell carcinoma. Premalignant process. Burning in mucosa, angular cheitis, smooth, red tongue. Esophagus shows abnormal bands of tissues termed esophageal webs. Brittle, spoon shaped nails. Tx: iron pills, track for cancer (50%).
Plummer-Vinson Syndrome
• Megaloblastic anemia• Caused by poor absorption of cobalamin (Vitamin B12, extrinsic factor)• Most patients lack intrinsic factor because of autoimmune destruction of the parietal cells of the stomach, which results in decreased absorption of cobalamin.• Oral manifestations: atrophic glossitis- beefy red tongue
Pernicious Anemia
• Increased production of growth hormone, usually related to a functioning pituitary adenoma.o Takes place before the closure of the epiphyseal plates- teenagero Oral finding: generalized macrodontia (big teeth)
Gigantism
• Excessive production of growth hormone after the closure of the epiphyseal plates• Increased growth of mandible (prognathism) with diastema formation, macroglossia
Acromegaly
