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pathology ii > Skin and Mucosal Diseases I > Flashcards

Skin and Mucosal Diseases I Flashcards

1
Q

what is ectodermal dysplasia and its inheritance pattern

A
  • two or more ectodermally derived structures fail to develop
  • hair, skin, nails, teeth, sweat glands, salivary glands are affected
  • autosomal dominant, autosomal recessive, X- linked inheritance
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2
Q

what are the clinical features of ectodermal dysplasia

A
  • hyphohidrotic ectodermal dysplasia - most common type
  • male predominance
  • reduced number of sweat glands -> cannot regulate heat
  • sparse hair, reduced eyebrows, eyelashes
  • salivary glands may be hypoplastic or absent
  • teeth- hypodontia, abnormal shape
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3
Q

what is the tx for exctodermal dysplasia

A
  • genetic counseling
  • prosthetic appliances
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4
Q

what is white sponge nevus, the inheritance pattern and the mutation

A
  • defect in the normal keratinization of oral mucosa
  • Autosomal dominant inheritance
  • keratin 4, keratin 13 mutations
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5
Q

what are the clinical features of white sponge nevus

A
  • usually appear at birth, early childhood
  • symmetrical, thickened plaques
  • white, corrugated appearance
  • most common buccal mucosa bilaterally
  • ventral tongue, labial mucosa, soft palate, alveolar mucosa, FOM
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6
Q

what is the tx for white sponge nevus

A

none

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7
Q

describe ehlers danlos syndrom

A
  • CT disorder, production of abnormal collagen
  • many genes involved
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8
Q

what are the clinical features of ehlers danlos syndrome

A
  • hyperelasticity of the skin, cutaneous fragility
  • hypermobility of joints- remarkable flexibility
  • patients may bruise easily
  • gorlin sign: touch the tip of the nose with tongue in 50% of patients
  • type VIII- rare type, periodontal disease
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9
Q

what is the treatment for ehlers danlos syndrom

A
  • depends on the subtype
  • mild type: compatible with normal life span
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10
Q

what is the inheritance of peutz jeghers syndrome and the mutation

A
  • autosomal dominant inheritance
  • mutations in tumor suppressor gene STK11
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11
Q

what are the clinical features of peutz jeghers

A
  • pigmented lesions on periorificial areas- mouth, nose, anus, genital region and extremities
  • intestinal polys- may develop into adenocarcinoma
  • increased frequency of other malignancies
  • intraoral lesions: buccal mucosa, labial mucosa and tongue
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12
Q

what is the tx for peutz jeghers

A

patients should be monitored for tumor development

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13
Q

describe epidermolysis bullosa

A
  • mucocutaneous disease, several types
  • genetic mutation
  • defect in attachment mechanisms of epithelial cells
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14
Q

what are the clinical features of epidermolysis bullosa

A
  • vesicles and bullae develop from low grade trauma
  • result in erosions and ulcerations that cause scarring
  • minor forms and severe forms
  • oral: gingival erythema, recession, loss of vestibule depth
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15
Q

what is the tx for epidermolysis bullosa

A
  • would care, antibiotics, surgery
  • recommended noncariogenic soft food diet, atraumatic oral hygiene procedures
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16
Q

what is the histological appearance of epidermolysis bullosa

A

separation of epithelium and CT

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17
Q

describe Behcet syndrome and what is the genetic predisposition gene

A
  • combination of chronic ocular inflammation, oro-genital ulcerations and systemic vasculitis
  • abnormal immune process triggered by an infectious or environmental antigen
  • genetic predisposition- HLAB51
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18
Q

what are the clinical features of behcet syndrom

A
  • MC in 3rd or 4th decade
  • increased prevalance in males
  • most patients have oral ulcerations
  • ulcerations may appear similar to aphthous stomatitis as they are usually surrounded by a larger area of erythema
  • genital lesions: irregular ulcerations
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19
Q

what is the vascular disease component in behcets

A

veins affected more frequently - inflammation and thrombi

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20
Q

what is the cutaneous lesion involvement in behcets

A

erythematous papules, vesicles, and pustules

21
Q

what is the ocular involvement in behcets disease

A

uveitis, conjunctivitis, corneal ulceration, arteritis
- blindness in 25% of patients

22
Q

what is the CNS involvement in Behcets

A

paralysis and dementia

23
Q

what is the tx for behcets

A

systemic meds such as corticosteroids and immunosuppressants

24
Q

what is reactive arthritis, the genetic predisposition and another name for it

A
  • likely an immunologically mediated disease triggered by an infectious agent
  • genetic predisposition: HLA- B27
  • Reiter syndrome
25
Q

what are the clinical features of reactive arthritis

A
  • prevalent in young adult men
  • triad: urethritis, arthritis, conjunctivitis
26
Q

what is often the first sign of reactive arthritis

A

urethritis

27
Q

what joints are affected in reactive arthritis

A

joints of lower extremities

28
Q

what are the clinical features of reactive arthritis

A
  • oral: erythematous papules, shallow ulcers
  • tongue, buccal mucosa, palate, gingiva
  • skin lesions on penis- balantitis circinata- similar appearance to geographic tongue
29
Q

what is the tx for reactive arthritis

A

NSAIDS for managing arthritis, corticosteroids, immunosuppressants

30
Q

describe psoriasis

A
  • chronic skin disease, affects 2% of US population
  • increased proliferative activity of cutaneous keratinocytes
  • genetic and environmental factors may play a role
  • prevalence of erythema migrans appears to be higher than general population
31
Q

what are the clinical features of psoriasis

A
  • onset during 2-3rd decade
  • persists for years with periods of exacerbation and inactivity
  • MC on scalp, elbows, knees, often symmetrically distributed
  • well demarcated, erythematous plaque with silvery scale of surface
  • lesions improve during summer and worsen during the winter
32
Q

what is the tx for psoriasis

A

topical corticosteroids for moderate involvement

33
Q

what is lupus erythematous

A
  • immune mediated condition
  • common CT disease in the US
34
Q

what are the two types of lupus erythematous and describe each

A
  • systemic lupus erythematous (SLE): multisystem disease affected solid organs, cutaneous and oral manifestation
  • chronic cutaneous lupus erythematosus (CCLE): primarily affects skin and oral mucosa
35
Q

what are the clinical features of lupus

A
  • females: 8-10x more common
  • most common dx in 4th decade
  • fever, weight loss, arthritis, fatigue
  • butterfly rash over malar area and nose in 40-50% of patients
  • sunlight may exacerbate lesion
36
Q

what are the clinical features of SLE

A
  • kidneys affected in 40-50% of patients- may lead to kidney failure
  • cardiac involvement- endocarditis
  • oral lesions: may appear lichenoid, erythema, ulcerations, often nonspecific on palate, buccal mucosa, lips, gingiva
37
Q

what are the clinical features of CCLE

A
  • few or no systemic signs or symptoms
  • lesions limited to skin or mucosal surfaces
  • skin lesion: scaly, erythemaous patches- sun exposed skin
  • oral lesions: lichenoid appearance. rarely occur in absence of skin lesions
38
Q

what is the dx for lupus

A

antibodies directed against double stranded DNA in 70% SLE patients

39
Q

what is the tx for lupus

A
  • avoid excessive sunlight exposure
  • mild cases may be managed with NSAIDS
  • severe cases: systemic corticosteroids, immunosuppressive medications
  • prognosis depends on organs affected
40
Q

describe angioedema

A
  • diffuse, edematous swelling of soft tissue
  • involves subcutaneous and submucosal CT
  • most common cause: mast cell degeneration -> histamine release
  • IgE- mediated hypersensitivity to drugs, foods, plants, dusts
  • alternative mechanism: ACE inhibitors in 0.1-0.2% of users - excess bradykinin
41
Q

what are the clinial features of angioedema

A
  • rapid onset of soft, nontender, tissue swelling
  • most common in extremities, also face, neck, trunk and genitals
  • ACE- inhibitor associated angioedema frequently affects head and neck such as face, lips, tongue, FOM, pharnyx, and larynx , 3-4x in black patients
42
Q

what is the dx and tx for angioedema

A
  • dx: clinical presentation and determination of antigenic stimulus
  • tx: allergic: oral antihistamine therapy or ACE inhibitor: avoid all medications in drug class
43
Q

what is the diff dx for white sponge nevus

A
  • cheek chewing
  • leukoedema
  • lichen planus
  • candidiasis
44
Q

does white sponge nevus dissappear when stretched

A

no

45
Q

is white sponge nevus more localized or diffuse

A

diffuse never localized

46
Q

epidermolysis bullosa histology looks similar to:

A

pemphigoid

47
Q

what is the #1 differential for behcets disease

A

reactive arthritis

48
Q

what is the most common form of angioedema that dentists will see

A

ACE inhibitor associated angioedema

pathology ii (19 decks)

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