Oral Path Exam IV

ankyloglossia

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- Results from the complete or partial fusion of the
lingual frenum to the floor of the mouth (sometimes it is to the lingual alveolar mucosa)
- Complete ankyloglossia is rare
- Partial ankyloglossia is more common
- Patients may have speech problems
- Patients may have gingival recession and bone loss if the
frenum is attached high on the alveolar ridge

Treatment
Surgical removal of a portion of the lingual frenum
(frenectomy)

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Terms in this set (57)

ankyloglossia

developmental cysts

- Cyst: an abnormal sac or cavity lined by epithelium and surrounded by connective tissue
- Dental lamina, dental organ — > Not inflamed cyst.

- Three types
*Odontogenic cysts
Nonodontogenic cysts
Pseudocysts (False cyst- Not lined)*

Dentigerous cyst

- Forms around the crown of an unerupted or developing tooth
- Second most common odontogenic cyst after the radicular cyst (non vital tooth- most common)
- Fluid forms between the reduced enamel epithelium and the crown
- Most common location: mandibular third molar area
- second most common maxillary canine
- Most common in young adults
- Asymptomatic or it may cause tooth displacement

Radiographic features
- Well-defined, unilocular radiolucency around the crown of an impacted or unerupted tooth

Microscopic feature
- Lined by cuboidal or stratified squamous epithelium
- Fibrous connective tissue wall

Treatment
- Surgical excision of the cyst and the involved tooth

Odontogenic Keratocyst (OKC)

- Also known as keratocystic odontogenic tumor (Cyst not a tumor)
- Unique cyst that is most often found in the mandibular third molar area
- But can be anywhere in the mouth
- Can move teeth and resorb tooth structure; usually do not cause bone expansion

Radiographic features
- Well-defined, unilocular or multilocular radiolucent lesion
- Thins out bone, but does not cause bone expansion

Microscopic features
- Lined by stratified squamous epithelium that is 8-10 cell layers thick
- (uniformed)
- Luminal epithelium is composed of parakeratin
- Basal cell layer is palisaded and prominent
- Interface between epithelium and connective tissue is flat
(No rete pegs)

Treatment
- Surgical excision and osseous curettage (get the entire thing out or it will reoccur)

Prognosis
- High recurrence rate (25%) (Highest of any odontogenic cyst- most aggressive)
- Follow-up examinations are important

Lateral periodontal cyst

- Arises in the mandibular cuspid and premolar area
- Usually asymptomatic; adults between 50-70;
- M > F

Radiographic features
- Unilocular radiolucent lesion
- Lateral aspect of a vital tooth root

Treatment
- Conservative surgical excision (curette it out)

Prognosis
- Recurrence is rare but may occur

Gingival cyst

(VARIANT OF LATERAL PERIODONTAL CYST)
- Small bulge or swelling of the attached gingiva or
interdental papillae in the mandibular cuspid and premolar area
- Soft tissue counterpart of the lateral periodontal cyst
- Radiograph of underlying bone is normal
- F = M

Treatment
- Surgical excision

Prognosis
- Excellent

Nonodontogenic cysts

• Nasopalatine canal cyst
• Dermoid cyst
• Thyroglossal tract cyst

nasopalatine canal cyst

- Also known as an incisive canal cyst (canal dilates)
- Located in the nasopalatine canal or incisive papilla (Between 8 and 9)
- In soft tissue and the bone
- Arises from epithelial remnants of the embryonic nasopalatine ducts
- Adults between 40-60; M > F
- Asymptomatic
- Adjacent teeth are vital
- Pressure resorption on the root

Radiographic features
- Well-defined, radiolucent lesion
- Heart shaped

Treatment
- Surgical enucleation

Prognosis
- Recurrence is rare

dermoid cyst

- Often present at birth or noted in young children
- Oral examples occur in the anterior floor of the mouth
- Midline
- May cause posterior displacement of the tongue
- Dough-like consistency when palpated
- Depresses and rises back up
- Asymptomatic

Microscopic features
- Lined by keratinized stratified squamous epithelium
- Lumen is filled with keratin
- Connective tissue wall contains hair follicles, sebaceous glands sweat glands

Treatment
- Surgical excision

Prognosis
- Good

Thyroglossal duct cyst

- Forms anywhere along the thyroglossal tract from the foramen cecum to the thyroid gland (Remnants from the tract is what creates this cyst)
- Most cases occur below the hyoid bone (neck)
- Young individuals usually under 20; M = F

Oral examples
- Posterior aspect of the dorsal tongue (thats where the tract starts)
- Smooth mass
- Dysphagia or difficulty extending tongue

Neck examples
- Smooth bulge or swelling in the midline of the neck
- Mass rises when the patient swallows or the tongue is extruded

Treatment
- Complete excision of the cyst and tract, including a portion of the hyoid bone and adjacent muscle

Prognosis
- Good
- Malignant transformation has been reported (thyroid tissue in the wall is becoming malignant)

PseudocystsStatic bone cyst/Stafne Defect Simple bone cystStafne DefectStatic bone cyst (know this name) Radiographic features - Well-defined cyst-like radiolucency in the *posterior mandible inferior to the mandibular canal* - Caused by a lingual depression in the mandible which surrounds normal salivary gland tissue - Asymptomatic; M > F - Salivary gland tissue pushing on lingual surface of the Mandible - Usually unilateral Treatment - NoneSimple Bone Cyst- Also known as a traumatic bone cyst - Pathologic cavity in bone; asymptomatic - *Young individuals;* M = F - No epithelial lining- surgeons just fall into an empty hole. Fluid may be present - Curette the wall and start bleeding so osteoblasts get excited and start to create bone Radiographic features - Well-defined unilocular or multilocular radiolucency that *scallops between the roots of teeth (teeth should be vital)* - Mandibular posterior region Treatment - Curettage to establish bleeding Prognosis - Excellent - Recurrence is unusualAbnormalities in the number of teethHypodontia Supernumerary teethOdontogenic cystsDentigerous cyst Odontogenic keratocyst Lateral periodontal cyst Gingival cystAbnormalities in the size of teethMicrodontia MacrodontiaHypodontia*- Lack of one or more teeth* - Permanent dentition > deciduous dentition Permanent dentition - Maxillary and mandibular third molars - Maxillary lateral incisors - Mandibular second premolars Deciduous dentition -Mandibular incisors Treatment - Prosthodontic replacement - Orthodontic evaluation - May be a component of a syndromeSupernumerary teeth- Extra teeth - Deciduous or permanent dentitions - *Maxilla* > mandible - Unilateral or bilateral - Usually *smaller* than a normal-sized tooth Several specific types - Mesiodens - DistomolarMesiodens- Most common supernumerary tooth - Located *between the maxillary central incisors (embedded or impacted)* - Small tooth with a conical crown and short roots - May erupt or may be embedded or impactedDistomolar- Second most common supernumerary tooth *- Maxillary fourth molar* - Located *distal* to the third molar - Rarely erupts into the oral cavity - Supernumerary teeth may cause crowding malpositioning of adjacent teeth, or non eruption of normal teeth Other supernumerary teeth - Mandibular and maxillary premolars - Maxillary lateral incisors - Maxillary paramolar (Means around- Lingual side) Treatment - Surgical excision - May be a component of a syndromeMicrodontia- One or more teeth are *smaller* than normal hypodontia = microdontia Three classifications - True generalized microdontia (all teeth) - Generalized relative microdontia - Microdontia involving a single tooth *True generalized microdontia* - Extremely rare; *due to lack of growth hormone production* - Pituitary dwarf, Down syndrome - *All teeth are smaller* than normal *Generalized relative microdontia* - *Normal-sized teeth appear small in large jaws* - May be due to heredity (from mom and dad) *Microdontia involving a single tooth* - *Most common type of microdontia* - Usually involves the *maxillary lateral incisor ("peg lateral")* and maxillary third molar (second most common) Treatment - Impacted microdonts should be extracted - Erupted microdonts can be treated with cosmetic dentistryMacrodontia- One or more teeth are *larger than normal* Three classifications - True generalized macrodontia - Generalized relative macrodontia - Macrodontia involving a single tooth *True generalized macrodontia* - Rare; *pituitary gigantism* *Generalized relative macrodontia* - Normal-sized teeth in small jaws - May be due to heredity *Macrodontia involving a single tooth* Uncommon Treatment - NoneAbnormalities in the shape of teethGemination Fusion Concrescence Dilaceration Enamel pearl Taurodontism Dens invaginatusGemination- Occurs when a single tooth germ attempts to divide resulting in the *incomplete formation of two teeth* - Cause is unknown - Deciduous > permanent; anterior > posterior - Most common teeth: *deciduous mandibular incisors and permanent maxillary incisors* - Appears as two crowns joined by a notched incisor area *- Tooth count is normal* Radiographic features -One single root and one common pulp canal Treatment Prosthodontic therapyFusion- Results from the union of two normally separated adjacent tooth germs - Cause is unknown - Always involves confluence of dentin (Dentins are attached) - Deciduous > permanent; anterior > posterior *- Most often seen in the maxillary anterior region* - Most common teeth: incisors - Appears as a single large crown that occurs in place of two normal teeth *- Tooth count is one less than normal* Radiographic features Separate or fused roots and root canalsConcrescence*- Two adjacent teeth united by cementum only* - Form of fusion that occurs after tooth formation is complete - Due to crowding or trauma that results in close approximation of adjacent tooth roots - Most common teeth: *maxillary molars* Treatment - None - May cause problems if a tooth needs to be extractedDilaceration*- Abnormal curve or angle in the root of a tooth* - Due to trauma to the tooth germ during root development - Deciduous or permanent dentitions - May appear anywhere along the root of a tooth Treatment - None - May cause problems if extraction or endodontic therapy is necessaryenamel pearl- Small, spherical enamel projection located on a root surface *- Due to abnormal displacement of ameloblasts during tooth formation* - Usually found on *permanent maxillary molars* - Can cause periodontal defects. Have to keep the area really clean - Attached to cementum near the root bifurcation or trifurcation - May contain enamel only or enamel, dentin, and pulp Treatment - None - Removal is necessary if periodontal problems occur (enamel pearl will be removed only, not the entire tooth)Taurodontism- Bull-like" teeth - Teeth exhibit *elongated, large pulp chambers and short roots* - Cause is uncertain - Deciduous and permanent dentitions *- Unilateral or bilateral* - Usually affects a single molar tooth or several molars in the same quadrant Radiographic findings - Enlarged and elongated pulp chamber - Roots appear short - Furcation is located near the apices Treatment - NoneDens invaginatusAlso known as *dens in dente* (tooth in a tooth) - Results when the enamel organ invaginates into the crown of a tooth *before* mineralization - Tooth may be normal in shape, or a malformed crown may be present that exhibits a deep pit or crevice in the area of the cingulum - Invaginated tooth maintains a communication with the outside of the tooth - Usually affects a single tooth - Anterior teeth > posterior teeth - Often the *maxillary and mandibular incisors* *- Most common tooth: maxillary lateral incisor* - Vulnerable to caries, pulpal infection, and necrosis - Tooth is often *nonvital* Radiographic features - Tooth-like structure appears within the involved tooth - Elongated bulb or pear-shaped mass of enamel is seen in dentin surrounding a radiolucent area - Usually involves the coronal third of the tooth but may extend to involve the entire root length Treatment - Prophylactic restoration to prevent caries and pulpal necrosis - Non Vital tooth - Endodontic therapyenamel hypoplasia- Incomplete or defective formation of enamel resulting in the alteration of tooth form or color - Due to a *disturbance of or damage to ameloblasts during enamel matrix formation* - Deciduous or permanent dentitions Numerous reasons - Amelogénesis imperfecta (Genetic disease) - Febrile illness (measles, chickenpox, scarlet fever) (HIGH temp) - Do not want a child or a pregnant woman getting these diseases. - Vitamin deficiency (A, C and D) - Local infection (Single tooth) *- Ingestion of fluoride* - Congenital syphilis Clinical appearance - Depends on the extent of the injury - Enamel may appear yellow to brown *- Severe pitting and deformity (contour change)* - Mottled discoloration (more than one color) *Local infection or trauma* *- Turner tooth* - Hypoplasia of a single permanent tooth due to *infection or trauma in a deciduous tooth* - Most commonly affected teeth: - Permanent maxillary incisors *(trauma)* - Permanent mandibular premolars *(infection)* *Fluoride ingestion* - Ingestion of more than 0.7 parts fluoride/million gallons of water - Affects all permanent teeth - Teeth are decay resistant *Congenital syphilis* - Affects the permanent incisors and first molars - Hutchinson incisors - Look like screwdrivers - broad cervically and narrow incisally; notched incisal edge - Mulberry molars - Multiple tiny globules of enamel instead of cusps Treatment - Composites, porcelain veneers, full coverage crowns, bleaching, bondingEndogenous staining of teeth- Due to the deposition of substances circulating systemically during tooth development - Commonly occurs as a result of: - Tetracycline ingestion (classic) - Erythroblastosis fetalis - Neonatal liver disease - Congenital porphyria (metabolic disease)Gross Chromosomal Abnormalities- Caused by alterations in chromosome number or alterations in structure (chromosomal breaks or abnormal rearrangements) Trisomy 21 Turner syndrome Klinefelter syndromeTrisomy 21- Also known as *Down syndrome (3-21's) (EXTRA)* - Only suppose to have 2 (one from mom one from dad) - 95% of cases are due to late maternal age at the time of conception - (over the age of 35) - Patients demonstrate slanted eyes, short stature, heart abnormalities (30%) - Near normal to low intelligence (big range) - Spacing between great toe and second toe - Horizontal palmar crease Oral manifestations - Fissured tongue, *macroglossia* - Premature loss of teeth - Gingival and periodontal disease (90%) - Hypodontia, abnormally shaped teeth, malpositioned and crowding of teethTurner Syndrome- *44* autosomal chromosomes and one x chromosome - *only seen in females* - 45 X (missing x chromosomes) - Clinical features - Short stature - Webbing of the neck (Lymphangioma during birth) - Edema of the hands and feet - Low hairline on the nape of the neck - Broad chest with widely spaced nipples Clinical findings - Abnormal aorta - Sparse body hair - External genitals are *infantile* - Ovaries are not developed - *primary amenorrhea (no menstrual cycle)* - Will not reproduceKlinefelter syndrome- 44 autosomal chromosomes and 3 sex chromosomes (XXY) - *only seen in males (47)* - Inherit extra x chromosomes. - Detected *after puberty* Clinical features - Taller than normal - *Reduced facial, body, and pubic hair* - Gynecomastia (50%) (Breast development) - *Low intelligence* (10%) (important- remember this) - Penis is normal, testes are smaller than normal - With additional X chromosomes, the clinical manifestations are more pronounced, and the intelligence is lower - More x's they get clinical appearance more pronounced and lower intelligence Oral manifestations - *Hypoplastic maxilla*Inherited disorders affecting the jaw bones and faciesGardner syndrome Nevoid basal cell carcinoma syndromeInherited disorders affecting the oral mucosaNeurofibromatosis of von Recklinghausen Peutz-Jeghers syndromeInherited disorders affecting the teethAmelogenesis imperfecta Dentinogenesis imperfecta Dentin dysplasiaGardner syndrome- Also known as familial adenomatous polyposis - Inherited, benign tumor of gland, polyp. - Autosomal dominant - "Do your parents have it?" Oral manifestations - *Numerous osteomas: #1 frontal bones,* mandible, maxilla - *Multiple odontomas, supernumerary teeth, impacted teeth* - Hypercementosis Clinical features - Multiple *colorectal polyps* - Become *malignant* at age 30 or later Polyps affect the *colon and rectum* - Colon removal - Intestinal resection before cancer developsNevoid Basal Cell Carcinoma Syndrome- Also known as Gorlin syndrome - Autosomal dominant inherited. Known family history Clinical features - *Hypertelorism (widely spaced eyes), broad nasal root,* frontal and parietal enlargement - Mild prognathism (mandible is further out than it should be) - Cutaneous manifestations, called nevi, are *basal cell carcinomas* - *Appear early in life* - *May occur anywhere* (even in areas not exposed to sun) Palmar and plantar pitting Oral manifestations - *Multiple odontogenic keratocysts* - Develop as early as *5-6 years of age* - Interfere with development of the jawbones and teeth - Frequently recur after surgical removal Skeletal abnormalities - Bifurcation or splaying of one or more ribs - Spina bifida occulta - Kyphoscoliosis - *Scoliosis* - abnormal lateral curvature of spine - *Kyphosis* - abnormal vertical curvature of spine (hunchback) - Medulloblastoma - brain tumorNeurofibromatosis type 1- Autosomal dominant (Inherited) Clinical features - Multiples neurofibromas - May occur anywhere - Present at birth or develop early in life - *Malignant transformation* occurs in 3%-15% of patients Oral manifestations - 10% of patients - Single or multiple neurofibromas - Often on *lateral tongue or gingiva* - May occur as a radiolucency in the mandible Clinical features - *Café au lait pigmentation* (coffee with milk) (Lots of them) - 90% of patients - Usually precedes development of neurofibromas - In the axilla: *Crowe sign* - ArmpitPeutz-Jeghers syndrome- Also called hereditary intestinal polyposis - Small bowel and does not come malignant - Autosomal dominant Clinical features - *Multiple melanotic macular pigmentations* of the skin and mucosa associated with gastrointestinal polyposis Clinical features - Diminish with age - *Around the eyes, nose, and mouth* - 98% of patients have intraoral pigmentation - Often on the lips and gingiva Clinical features - Pigmentation of hands, nasal mucosa, and eyes - *Intestinal polyps in small intestines* - Hamartomas (proliferation of normal tissue in a normal location) - Rarely undergo malignant transformation - Causes obstruction and painamelogenesis imperfecta- Group of inherited conditions affecting enamel - Not associated with any systemic defects - Affected primary and permanent teeth (ALL) Four types Type I: hypoplastic Type II: hypocalcified Type III: hypomaturation Type IV: hypoplastic-hypomaturation *Type I: hypoplastic* - Enamel is not of normal thickness - Ameloblasts *do not* lay down *enamel matrix* properly - Most frequent variety: *pitted* *Type II: hypocalcified* - Enamel is of normal thickness but is poorly calcified - Teeth are *yellow to orange* in color and enamel is very soft - Enamel is lost rapidly leaving exposed dentin *Type III: hypomaturation* - Large amounts of enamel matrix so the enamel is softer than normal - Enamel chips easily from the tooth *Type IV: hypoplastic-hypomaturation* - Enamel is thin, pitted, and yellow to brown in color - Associated with *taurodontism* Radiographic features - Enamel is missing or has a similar radiodensity as dentindentinogenesis imperfecta- May involve teeth only or be associated with a systemic disorder (osteogenesis imperfecta: Imperfect formation of bone) - Also known as hereditary opalescent dentin - Teeth have *bulbous crowns with an opalescent brown to brownish-blue* - Deciduous teeth are affected more severely than permanent teeth - Dentin is very soft which produces chipping of the enamel resulting in tooth attrition - Junction between dentin and enamel is abnormal so enamel can easily chip away. Marked tooth attrition. Radiographic features - Cervical constriction - No pulp chambers or root canals - Roots are short and thin - Periapical radiolucenciesDentin DysplasiaTwo types - Type I: radicular dentin dysplasia - Type II: coronal dentin dysplasia *Radicular dentin dysplasia (type 1)* - Normal crowns, abnormal roots - Autosomal dominant (Inherited) - Deciduous teeth = permanent teeth Radiographic features *- Total or partial lack of pulp chambers and root canals* Radiographic features - Short, blunted roots - Teeth exfoliate early - Half-moon appearance in pulps of permanent teeth - Multiple periapical radiolucencies *Coronal dentin dysplasia (Type 2)* - Autosomal dominant - Deciduous dentition has features similar to dentinogenesis imperfecta - Deciduous dentition Translucent teeth with an *amber color (gray blue)* - Permanent dentition Normal crowns with *normal color* Radiographic features - Deciduous dentition - Lack of pulp chambers and small root canals (just like DI) - Type 2 DD is the same as what we see in DI (clinically and radiographically) - Permanent dentition - Thistle-shaped or bow-tie shaped pulp chambers - Pulp stonesEndocrine disorders- Group of integrated glands and cells that secrete hormones - Secretion of hormones is controlled by feedback mechanisms - Disease occurs when *too much or too little hormone* is produced or there is *dysfunction* of the glands themselves Hyperpituitarism Hyperthyroidism Hypothyroidism Diabetes Addison diseasehyperpituitarism- Excess hormone production by the anterior pituitary gland - Usually caused by a *benign tumor that produces too much growth* hormone - Two forms: gigantism and acromegaly *Gigantism* - Occurs *before* epiphyseal plate closure - Excessive growth of the overall skeleton - More than 7 feet tall and weigh several hundred pounds *Acromegaly* - Occurs after epiphyseal plate closure - Onset is slow; M = F - Headaches, fatigue, muscle and joint pain, poor vision - Enlargement of the hands and feet - Enlargement of the maxilla and mandible - *Separation of teeth and malocclusion* - Mandibular prognathism - Anterior open bite - Thickened lips - *Macroglossia* Diagnosis - Measurement of growth hormone levels (draws blood and measures) Treatment - Surgical removal or radiation of pituitary adenoma Prognosis - GoodHyperthyroidism- Excessive production of thyroid hormone ( too much T3 an T4) - F > M; diagnosed in 30s and 40s - Most common cause is *Graves disease* - *Autoimmune disorder* in which thyroid stimulating immunoglobulins (antibodies) stimulate thyroid cells resulting in enlargement of the thyroid and production of too much thyroid hormone (T3 AND T4) - Thyroid enlargement (goiter) - *Excessive sweating, fine hair, softened nails* - Exophthalmos (protrusion of the eyeballs) - Anxiety, weakness, restlessness, tachycardia - Premature exfoliation of deciduous teeth - Premature eruption of permanent teeth - Dental caries and periodontal disease progress faster - Burning tongue Diagnosis - Evaluation of thyroid hormones (T3 and T4) and thyroid-stimulating hormone (TSH) in blood (TSH coming from pituitary) - *T4 (thyroxine) is elevated* - TSH is depressed Treatment - Surgery - Medications to suppress thyroid activity - *Radioactive iodine (most common treatment in adults)* - Treatment may lead to hypothyroidismHypothyroidism- Decreased production of thyroid hormone - Classified as primary or secondary - Primary: thyroid gland is abnormal (t3 and t4 low) - Secondary: pituitary gland *does not* make sufficient TSH - Due to developmental disturbances, autoimmune disease, iodine deficiency, drugs, surgery, radiation therapy - Two forms: cretinism and myxedema *Cretinism* - Occurs during *infancy and childhood* - Thickened lips, enlarged tongue - Macroglossia - Delayed eruption of teeth *Myxedema* - Occurs in *older children and adults* - Enlarged tongue, thickened lips - Macroglossia - Slow heart rate and lower body temperature - Laboratory tests: - Primary: T4 are normal or low, TSH is *high* *(Problem is the thyroid)* - Secondary: T4 and TSH are *low* *(Problem is Pituitary gland)* Treatment - Thyroid replacement therapy *(levothyroxine)*diabetes- Disorder of carbohydrate (glucose) metabolism characterized by abnormally high blood glucose levels (hyperglycemia) - Due to lack of insulin OR increased peripheral resistance to insulin and an inadequate secretory response by the pancreatic beta cells - Two major types Type 1: insulin-dependent Type 2: non-insulin dependent *Type 1 diabetes* - Autoimmune destruction of *islets of Langerhans* - Located in pancreas - 3% of cases (minority) - Peak age = 20 - Individuals are thin *Type 2 diabetes* - Increased insulin resistance - Onset occurs between 35-40 - 97% of cases (Majority) - Individuals are obese Clinical features - *Polydipsia, polyuria, polyphagia* - Accelerated atherosclerosis (lead to stroke) - Gangrene, high blood pressure, kidney failure, stroke, blindness - Slow wound healing - Increased susceptibility to infection Oral manifestations - Fungal infections: candidiasis, mucormycosis - Bilateral parotid gland enlargement (deposition of fat or hypertrophy of salivary gland tissue) - Xerostomia: dental caries - Burning mouth syndrome - Hyperplastic and erythematous gingiva - Periodontal disease Treatment - Eliminate infections - Antibiotic medication - Calculus and plaque removal - Effective oral hygiene care - Adequate nutritionAddison Disease- *Insufficient production of glucocorticoids (cortisol) and mineralocorticoids (aldosterone)* that are normally produced by the adrenal cortex - Predominant cause: destruction of the adrenal gland by an autoimmune disease - Other causes: malignant tumor, tuberculosis, deep fungal infections, HIV infection - *90%* of adrenal gland must be destroyed *before* clinical features occur - Onset is slow and includes depression, weakness, and low blood pressure - Weight loss, nausea and vomiting, craving for salt - *Pituitary gland increases production of adrenocorticotropic (ACTH)* hormone in response to decreased production of adrenal steroids - *ACTH stimulates melanocytes to make melanin* - Bronze pigmentation of skin - Melanotic macules occur on skin and oral mucosa - Similar to physiologic pigmentation but of recent onset - Similar to PEUTZ-JEGHERS SYNDROME Treatment - Corticosteroid replacementiron deficiency anemia- Occurs when an insufficient amount of iron is supplied to the bone marrow for red blood cell development - Most common cause of anemia in the United States Causes - Deficient intake of iron - Chronic blood loss (heavy menstruation or chronic gastrointestinal bleeding) - Poor iron absorption - Increased requirement for iron (pregnancy, infancy) - *Plummer-Vinson syndrome* - Long-standing iron-deficiency anemia - Dysphagia (swallowing), angular cheilitis, atrophic glossitis (burning tongue) - Atrophy of the upper alimentary tract (Esophagus get thin) - "Spoon-shaped" nails - *Predisposition to oral and esophageal cancer* - Esophageal web - Often asymptomatic - Patients may demonstrate weakness and fatigue, low energy, shortness of breath, cardiac palpitations - Angular cheilitis, mucosal pallor - Erythematous, painful tongue Diagnosis - Low hemoglobin, hematocrit (volume of RBCs in blood), and iron - Microcytic, hypochromic anemia - Small and lack of color Treatment - Increase intake of iron - Oral lesions resolve when deficiency is correctedpernicious anemia- Deficiency of intrinsic factor due to *autoimmune destruction of parietal cells in the stomach* - Parietal cells secrete intrinsic factor - *Necessary for absorption of vitamin B12* (b12 is excreted if not absorbed) - Vitamin B12 is needed for DNA synthesis, especially in rapidly dividing cells (bone marrow cells and epithelial cells) - Cells in the mouth are rapidly dividing - Weakness, pallor, fatigue - Nausea, dizziness, diarrhea - *Severe paresthesia and tingling (B12 helps to maintain the myelin sheath)* - Depression, dementia, schizophrenia-like symptoms Oral manifestations - Angular cheilitis, mucosal pallor - Painful, erythematous mucosa with ulcerations - Burning, atrophic tongue Diagnosis - Low serum vitamin B12 - Megaloblastic anemia Immature, abnormally large red blood cells with nuclei *(megaloblasts)* Treatment - Monthly injections of *cyanocobalamin* (synthesized form of vitamin B12 - Inject it in the hip or the butt, bypass the GI tract - Increased risk of gastric cancerLeukemias- Malignant neoplasm of hematopoietic (blood-forming) stem cells - Originates in the *bone marrow but spreads to the circulating blood* - Cause is unknown but some cases are due to oncogenic viruses - Classified by the *cell type involved and maturity* of the neoplastic cellAcute leukemias- Characterized by the presence of very *immature cells (blasts)* - Acute lymphoblastic leukemia (ALL) and acute myelogenous/myeloid leukemia (AML) - Onset is *abrupt and dramatic* - Rapidly fatal if not treated ALL - Affects children and young adults - Good prognosis AML - Adolescents and young adults - Poor prognosis Clinical features - Weakness (anemia), fever (infection), lymphadenopathy (early in the course of the disease), bleeding and spontaneous bruising (thrombocytopenia) - Late findings - Enlargement of the spleen *(splenomegaly)* - Enlargement of the liver *(hepatomegaly)* Oral manifestations _ *Gingival enlargement* (infiltration of leukemic cells) - Necrotizing ulcerative gingivitis - Bleeding gingiva, petechiae, ecchymoses Diagnosis - *Elevated white blood cell count* - Many blasts (immature cells) - Low red blood cell (anemia) and platelet count (thrombocytopenia) Treatment - Chemotherapy - Bone marrow transplantation - Antibiotics to prevent infection - Nutritional supplementationChronic leukemias(mature white blood cells) - Chronic lymphocytic leukemia (CLL) and chronic myelogenous leukemia (CML) - CLL accounts for 1/4 of all cases of leukemia - CML is associated with an identified *abnormal karyotype* - *Philadelphia chromosome (CML)* - Translocation between chromosomes 9 and 22 - CLL may be associated with an abnormal karyotype but not the same as seen in CML Clinical features - Adults; slow onset - Easy fatigability, weakness, weight loss, anorexia Oral manifestations - Pallor of lips and gingiva - Gingival enlargement, petechiae and ecchymoses - Gingival bleeding, atypical periodontal disease - Cervical *lymphadenopathy* (early sign) Diagnosis - White blood cell count can increase to > 500,000/mm3 (normal: 4,000 to 11,000/mm3) - Bone marrow pushing out mature white blood cells Treatment - Chemotherapy - Bone marrow transplantation - Long-term prognosis is poor