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Pathology 2 > Skin and Mucosal Diseases part 1 > Flashcards

Skin and Mucosal Diseases part 1 Flashcards

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1
Q

What is ectodermal dysplasia?

A
  • Two or more ectodermally derived structures fail to develop
    — Hair, skin, nails, teeth, sweat glands, salivary glands
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2
Q

What is the inheritance of ectodermal dysplasia?

A
  • AD, AR, X-linked inheritance
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3
Q

What are the clinical features of ectoderml dysplasia?

A

Hypohidrotic ectodermal dysplasia:
- 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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4
Q

What is the treatment for ectodermal dysplasia?

A

genetic counseling, prosthetic appliances

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5
Q
A

ectodermal dysplasia

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6
Q

What is white sponge nevus?

A
  • Defect in the normal keratinization of oral mucosa
  • Keratin 4, Keratin 13 mutations
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7
Q

What is the inheritance of white sponge nevus?

A

AD inheritance

autosomal dominant

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8
Q

What are the clinical features of white sponge nevus?

A
  • Usually appear at birth, early childhood
  • Symmetrical, thickened plaques
  • White, corrugated appearance
  • MC buccal mucosa bilaterally
  • Ventral tongue, labial mucosa, soft palate, alveolar mucosa, FOM
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9
Q

What is the treatment for white sponge nevus?

A

none

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10
Q
A

white sponge nevus

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11
Q

What are the differential diagnoses for white sponge nevus?

A
  • cheek chewing
  • leukoedema
  • lichen planus
  • candidiasis
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12
Q
A

white sponge nevus

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13
Q

What is this unique histology show and what disease is it for?

A

perinuclear condensation of keratin tonofilaments
- white sponge nevus

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14
Q

What is ehlers-danlos syndrome?

A
  • Connective tissue disorder, production of abnormal collagen
  • Many genes involved
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15
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! (50% of patients)
  • Type VIII – rare type, periodontal disease
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16
Q

What type of Ehlers-danlos syndrome has periodontal disease (especially at a young age)?

A

type VIII

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17
Q

What is the treatment for Ehlers-danlos syndrome?

A

Depends on subtype
- Mild type: compatible with normal life span

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18
Q
A

Ehlers-Danlos syndrome

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19
Q

What is this called and what disease is associated with it?

A

papyraceous scarring
- Ehlers-danlos syndrome

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20
Q

What is Peutz-Jeghers syndrome?

A

Mutations in tumor-suppressor gene – STK11

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21
Q

What is the inheritance of Peutz-Jeghers syndrome?

A

AD inheritance

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22
Q

What are the clinical features of Peutz-Jeghers syndrome?

A
  • Pigmented lesions on periorificial areas (mouth, nose, anus, genital region) and extremities
  • Intestinal polyps – may develop into adenocarcinoma
  • Increased frequency of other malignancies
  • Intraoral lesions: buccal mucosa, labial mucosa, tongue
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23
Q

What is the treatment for Peutz-Jeghers syndrome?

A

patients should be monitored for tumor development

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24
Q
A

Peutz-Jeghers syndrome

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25
What are these and what disease can lead to them?
intestinal polps - Peutz-Jeghers syndrome | these could lead to adenocarcinoma
26
What is Epidermolysis Bullosa?
- Mucocutaneous disease, several types - Genetic mutation - Defect in attachment mechanisms of epithelial cells
27
What are the clinical features of epidermolysis bullosa?
- 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
28
What is the treatment for epidermolysis bullosa?
wound care, antibiotics, surgery - Recommend noncariogenic diet (soft foods), atraumatic oral hygiene procedures
29
epidermolysis bullosa
30
What does this show and what disease is associated with it?
separation of epidermis and dermis (blister formation) - epidermolysis bullosa
31
What disease is very similar to epidermolysis bullosa in terms of histology?
Pemphigoid
32
epidermolysis bullosa
33
What is Behcet syndrome?
- Combination of chronic ocular inflammation, oro-genital ulcerations, and systemic vasculitis - Abnormal immune process triggered by an infectious or environmental antigen - Genetic predisposition – HLA-B51
34
Behcet syndrome is associated with HLA...
HLA-B51
35
What are the clinical features of Behcet syndrome?
- MC 3rd and 4th decade - Increased prevalence in males - Most patients have oral ulcerations - Ulcerations may appear similar to aphthous stomatitis --- Usually surrounded by a larger area of erythema - Genital lesions: irregular ulcerations - Vascular disease: veins affected more frequently – inflammation, thrombi - Cutaneous lesions: erythematous papules, vesicles, pustules - Ocular involvement: uveitis, conjunctivitis, corneal ulceration, arteritis - Blindness occurs in 25% patients - CNS involvement: includes paralysis and dementia
36
What is the treatment for Behcet syndrome?
may require systemic meds (corticosteroids, immunosuppressants)
37
Can you identify if a patient has Behcet syndrome just from looking at the ulceration?
NO - look at patient's history - refer
38
What is reactive arthritis (reiter syndrome)?
- Likely an immunologically mediated disease, triggered by an infectious agent - Genetic predisposition – HLA-B27
39
Reactive arthritis (reiter syndrome) is associated with HLA...
HLA-B27
40
What are the clinical features of Reactive arthritis (reiter syndrome)?
- Prevalent in young adult men - Triad: --- Urethritis (often first sign) --- Conjunctivitis --- Arthritis (usually affects joints of lower extremities) - Oral: erythematous papules, shallow ulcers - Tongue, buccal mucosa, palate, gingiva - Skin lesions on penis – balanitis circinata: --- Similar appearance to geographic tongue
41
What is the triad associated with reactive arthritis (reiter syndrome)?
- Urethritis - Conjunctivitis - Arthritis –usually affects joints of lower extremities | can't pee, can't see, can't climb a tree
42
What skin lesion on the penis has a similar appearance to geographic tongue? What disease is this linked to?
balanitis circinata - Reactive Arthritis (reiter syndrome)
43
What is treatment for Reactive Arthritis (reiter syndrome)?
NSAIDS for managing arthritis, corticosteroids, immunosuppressants
44
Reactive Arthritis (reiter syndrome)
45
What is psoriasis?
- 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
46
What are the clinical features of psoriasis?
- Onset during 2nd-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 on surface - Lesions improve during summer and worsen during the winter - geographic tongue is more often associated than the average population
47
What is the treatment for psoriasis?
topical corticosteroids for moderate involvement
48
psoriasis
49
What is lupus erythematosus?
- Immune mediated condition - Common connective tissue disease in the US
50
What are the types of lupus erthematosus?
- Systemic lupus erythematosus (SLE): multisystem disease: solid organs, cutaneous and oral manifestation - Chronic cutaneous lupus erythematosus (CCLE): primarily affects skin and oral mucosa
51
What are the clinical features of lupus erthematosus (SLE)?
- Females: 8-10x more common - MC diagnosis in 4th decade - Fever, weight loss, arthritis, fatigue - Butterfly rash (erythematous rash) over malar area and nose (40-50% patients) ---Sunlight may exacerbate lesion - Kidneys affected (40-50% patients) – may lead to kidney failure - Cardiac involvement - endocarditis - Oral lesions: may appear lichenoid, erythema and ulcerations may be present --- often nonspecific --- palate, buccal mucosa, lips, gingiva
52
What are the clinical features of lupus erthematosus (CCLE)?
- Few or no systemic signs or symptoms - Lesions limited to skin or mucosal surfaces - Skin lesion: scaly, erythematous patches (sun-exposed skin) - Oral lesions: lichenoid appearance - Rarely occur in absence of skin lesions
53
How is lupus diagnosed?
Antibodies directed against double-stranded DNA (70% SLE patients)
54
What is the treatment for lupus?
- Avoid excessive sunlight exposure - Mild cases may be managed with NSAIDS - Severe cases: systemic corticosteroids, immunosuppressive medications - Prognosis depends on organs affected
55
lupus erythematosus
56
lupus erythematosus
57
What is angioedema?
- Diffuse, edematous swelling of soft tissue - involves subcutaneous and submucosal connective tissue caused by... - MC cause: mast cell degranulation → histamine release - IgE-mediated hypersensitivity (drugs, foods, plants, dusts) - Alternative mechanism: ACE inhibitors (0.1 – 0.2%) --- Excess bradykinin
58
What are the clinical features of angioedema?
- Rapid onset of soft, nontender tissue swelling - MC in extremities, also face, neck, trunk, genitals - ACE-inhibitor associated angioedema: frequently affects H&N --- Face, lips, tongue, FOM, pharynx, larynx --- 3-4x Black patients
59
How is angioedema diagnosed?
Clinical presentation and determination of antigenic stimulus
60
What is the treatment for angioedema?
- Allergic: oral antihistamine therapy - ACE-inhibitor: avoid all medications in drug class
61
angioedema

Pathology 2 (39 decks)

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