Dermatology Flashcards
Inheritance of Marfan syndrome
Autosomal Dominant
What is defective in Marfan syndrome?
Protein called fibrillin
*Located in ECM
Autoantibodies to adhesion proteins (ie: desmosomal or hemidesmosomal) can lead to what 2 diseases, respectively?
Desmosomal autoantibody:
Pemphigus vulgaris
Hemidesmosomal autoantibody:
Bullous pemphigoid
Another name for atopic dermatitis
Eczema
Eczema is considered which hypersensitive reaction/
Type 1
*Treat with topical steroids
Microscopy analysis of atopic dermatitis would show what?
Spongiosis (increasing spacing of epidermal cells due to intercellular edema)
What is the difference in location of the erythematous lesions in infants vs children/adults who are affected by eczema?
Infants: Extensors
Adults: Flexors
What can be in the family history of a patient who has eczema?
Family has hay fever, allergic rhinitis, or asthma
What type of sensitivity reaction is contact dermatitis?
Type 4
Cause of lichen simplex chronicus?
Itching & scratching
*Results in leathery thick skin
Differentiating factor between eczema & seborrheic dermatitis?
Seborrheic dermatitis is confined to head & scalp with red patchy infiltrate
Explain how restoration naturally of the epidermis can occur?
Stem cells located in the hair follicles and sweat glands within the dermis
**Because items above are derived from ectoderm too, which includes hair follicles, sebaceous glands, & sweat glands
***Dermis is derived from mesoderm
Where are melanocytes derived from embryonically?
Neural crest cells
Inheritance of xeroderma pigmentosum
Autosomal recessive
Characteristic of xeroderma pigmentosum
Extreme sensitivity to sunlight
*predisposition to malignancy –> malignant melanoma, BCC, & SCC
Reason for xeroderma pigmentosum
Defective nucleotide excision repair (NER) mechanism of UV damaged DNA, causing pyridimidine dimers
*Thymidine nucleotides form covalent bonds with each other rather than complementary bases on opposite DNA strand
**These mutations accumulate and can cause cancer
Explain the ABCDE rule and what is it used to identify?
Melanoma
- Asymmetry of growth
- Borders are irregular
- Color variations
- Diameter great than 6 mm
- Evolution over time
What is the name of the ulcerative form of impetigo?
Ecthyma
2 main forms of impetigo
1) Non-bullous [either S. aureus or S. pyogenes]
- Lesions begin as papules & progress to vesicles
- Later become pustules
2) Bullous [only S. aureus]
- Caused by exfoliative toxin A
What is + Nikolsky sign suggestive of? What is it?
Rubbing results in skin exfoliation
Dx: Pemphigus Vulgaris (autoimmune disease)
*Caused by dysfunctional desmosomes in epidermis
What disease is associated with a defect in actin?
NONE! trick question….not a single one exists
Inflammation of the nasolacrimal sac & cause?
Dacryocystitis
*Due to obstruction of nasolacrimal duct, causing reduced tear flow & infection
Infant who develops generalized severe erythema and flaccid bull (+ Nikolsky sign) is indicative of what infection?
Scalded skin syndrome
*S. aureus
**Treat baby like burn victim
In scalded skin syndrome of the infant, where are symptoms especially located?
Around the umbilicus
What do exotoxins in staph infections typically target in scalded skin syndrome?
Desmosomes between keratinocytes in the stratum granulosum
What diseases demonstrate a + Nikolsky sign? (3)
- Scalded skin syndrome
- Epidermal necrolysis
- Pemphigus vulgaris
Maculopapular rash that begins in the face and neck, traveling to the extremities, and sparing the palms & soles is what disease?
Scarlet fever
*S. Pyogenes
What is the severe cause of Steven-Johnson syndrome?
Toxic epidermal necrolysis
*Presents similar to scalded skin syndrome but patients are older, involve mucous membranes, and triggered by new medicines
Vitamin C is an essential cofactor for what in scurvy?
Necessary for hydroxylation of proline & lysine residues on pre-pro-collagen chains
Cause of albinism
Deficiency in enzyme tyrosinase (AR inheritance)
*Inability to synthesize melanin from tyrosine (aromatic amino acid)
Function of hemidesmosomes
Connect basal surface of epithelial cells to underlying basement membrane
In which layer are blisters formed in bullous pemphigoid?
Subepidermal
What are antibodies being made against in the disease involving hemidesmosomes?
Dystonin
*Bullous pemphigoid