03a: Systemic Disease

Question 1

Painless, red-brown papules and plaques primarily found on shins that may signify underlying metabolic abnormality.

Answer 1

Necrobiosis lipoidica (20-35% have diabetes)

Question 2

Necrobiosis lipoidica: as plaques progress, how do their features change?

Answer 2

Start as red-brown papules/plaques; progress to yellow-brown atrophic plaques with irregular violaceous borders and telangiectasias

Question 3

Rx for necrobiosis lipoidica:

Answer 3

Compression stockings, topical steroids

Question 4

Pretibial myxedema is associated with which underlying disease?

Answer 4

Autoimmine thyroid disease (Graves’)

Question 5

Spontaneous bruising around eyes in elderly patient is sign of which underlying disease?

Answer 5

Amyloidosis

Question 6

Amyloidosis causes spontaneous bruising, especially in (X) location, via which mechanism

Answer 6

X = periorbital (large blood vessels close to skin)

Amyloid binds Factor X, causing bleeding diathesis (due to factor deficiency)

Question 7

(Primary/secondary) amyloidosis, aka AL, occurs as result of:

Answer 7

Primary

Untreated multiple myeloma

Question 8

(Primary/secondary) amyloidosis, aka AA, occurs as result of:

Answer 8

Secondary
Chronic illness (RA, Hodgkin's, RCC)

Question 9

T/F: Cutaneous involvement is more common in secondary amyloidosis.

Answer 9

False - rare cutaneous involvement in AA; deposits in kidney, liver, and spleen

Question 10

Dermatitis herpetiformis presents as (X) and is associated with which disease?

Answer 10

X = very pruritic lesions on extensor surfaces of elbows, shins, buttocks (bilaterally)

Celiac’s

Question 11

Dermatitis herpetiformis: auto-(X) attacks which self antigen?

Answer 11

X = IgA
Epidermal transglutaminase (very similar structure to tissue transglutaminase)

Question 12

Pyoderma grangenosum associated with which underlying disease?

Answer 12

Mainly IBD (Ulcerative colitis);

Also RA and malignancy

Question 13

First-line Rx for Pyoderma grangenosum:

Answer 13

Systemic steroids

Question 14

(X) syndrome is an AD-inherited mutation in (Y) gene. Abnormal findings include oral and digital lentigines as well as multiple (Z) growths in GI tract.

Answer 14

X = Peutz-Jeghers
Y = tumor suppressor
Z = hamartomatous polyps

Question 15

Pt with Peutz-Jeghers syndrome is at increased risk for which malignancies?

Answer 15

Visceral (colon, rectum, pancreas, stomach, testicles, ovaries, lung, cervix)

Question 16

Most common cause of porphyria cutanea tarda:

Answer 16

Liver dysfunction

Others: EtOH, exogenous estrogens (OCPs), Fe overload

Question 17

Renal dialysis patient presents with severe pruritis on bilateral arms. You notice hyperkeratotic papules with occasional white tip. What process is occurring?

Answer 17

Perforating disorder (degenerated collagen and elastin is being extruded through stratum corneum)

Question 18

Renal dialysis patient has angulated plaques with an adherent black eschar/crust. This is likely (X) and is treated with (Y).

Answer 18

X = Calciphylaxis
Y = IV Na thiosulfate (Ca chelator)

Question 19

Renal failure patient that gets an MRI is at risk for which skin complication?

Answer 19

Nephrogenic systemic fibrosis (due to exposure to gadolinium) - progressive skin fibrosis causing joint contractures; rarely seen anymore (renal function checked before MRI)

Question 20

Tuberous sclerosis: initial skin findings include

Answer 20

1. Ash leaf-shaped hypopigmented macule

2. Collagenoma (Shagreen patch)

Question 21

Tuberous sclerosis: typical skin finding on presentation to doctor

Answer 21

Facial angiofibroma (red/brown papules)

Question 22

Tuberous sclerosis: complications include (X) growths in multiple areas such as..

Answer 22

X = fibromas (periungal), angiomyolipoma (kidney), nodules (brain)

Question 23

Sign of Leser-Trélat:

Answer 23

Sudden onset of many seborrheic keratoses associated with GI adenocarcinoma

Question 24

Most common site of basal cell carcinoma is (X), squamous cell carcinoma is (Y), and melanoma is (Z).

Answer 24

X = head and neck
Y = head and neck and dorsal hands
Z = back for men; legs for women

Question 25

Which two autoimmune diseases target (X) desmosome proteins?

Answer 25

Pemphigus foliaceus (desmoglein 1) and Pemphigus vulgaris (desmoglein 3 and maybe 1)

Question 26

Skin: How does Desmoglein 1 differ from 3?

Answer 26

Distribution (1 along entire epidermis; 3 only in deeper layers)

Question 27

Pemphigus vulgaris with only desmoglein 3 affected will present with which findings?

Answer 27

Only mucous membrane involvement (where desmoglein 3 extends along entire epidermis)

Question 28

T/F: Pemphigus vulgaris patient with cutaneous involvement must have Ab attacking both Desmoglein 1 and 3.

Answer 28

True