Epidermis Diseases

Question 1

Epidermolytic ichthyosis

Answer 1

• Generalized erythema, blisters, and inflamed skin starting at birth and lasting until early childhood. In late childhood/adulthood, there is extensive scaling of the skin.
• Disorder of keratins K1 or K10

Question 2

Pemphigus

Answer 2

• epidermal blistering that heals without scarring

- Autoimmune disease caused by antibodies that affect the desmosomes.

Question 3

Autosomal recessive congenital ichythosis (ARCI)

Answer 3

• thick, plate-like scales and red skin are found in a general distribution
• mutations in keratinocyte transglutaminase result in a defective cornified envelope

Question 4

X linked recessive ichthyosis (XLRI)

Answer 4

• large, dark scales that are found on the trunk, extremities, and sides of the neck.
• defect in steroid sulfatase prevents desquamation of the cornified epithelium

Question 5

Xerosis

Answer 5

• dry skin that is scaly, rough, and itchy skin especially in seasons of low ambient humidity.
• itching damages the barrier, leading to further inflammation
• tx: emollients, steroids, antihistamines

Question 6

Atopic dermatitis

Answer 6

• aka eczema
• often associated with asthma or allergic rhinitis
• often associated with a loss of filligrin function.

Question 7

Psoriasis

Answer 7

• scaly, hyperkeratotic red plaques
• Th17 immunologic dysfunction, keratinocyte hyperproliferation, keratinocyte nuclei may be maintained in the strateum corneum
• tx targets immune system factors leading to hyperproliferation