Ch 10 Connective Tissue Disorders

Question 1

SLE Ab

Answer 1

dsDNA, smith (u1,u2)

phospholipid

Question 2

Drug induced lupus

Answer 2

histones

Question 3

subacute lupus

Answer 3

Ro
La
-placenta and heart block

Question 4

Dermatomyositis

Answer 4

Jo-1

Mi-2

Question 5

Systemic Sclerosis

Answer 5

SCL-70 topoisomerase

Question 6

CREST

Answer 6

Centromere

Question 7

Mixed CT

Answer 7

U1-RNP

Question 8

Lichen Sclerosis

Answer 8

extracellular matrix protein 1

Question 9

Localized CT

Answer 9

Discoid lupus

Morphea

Question 10

Intermediate

Answer 10

Subactue Lupus

• Juvenile Dermatomyositis
• Limited scleroderma (CREST)

Question 11

Systemic

Answer 11

SLE
Adult dermatomyositis
Systemic Sclerosis

Question 12

Overiding path/histo of CT

Answer 12

Dermal atrophy, arthritis, fibrosis, epidermal thinning

Question 13

SLE

Answer 13

-Histology shows loss of basal layer, epidermal thinning and increase in the corneum, C3 and Ig granular deposits at BM zone
-periungal telangectasia, marginal hair loss, palatal ulcers
-Bullous form
Scaring alopecia

Question 14

Subacute

Answer 14

• Less
• Ro/La
• Plaquenil is better

Question 15

Discoid

Answer 15

• Confined to skin
• Scarring alopecia and follicular plugging
• Diff from psoriasis by less scale and location (Sun exposure)
• Topical steroids are treatment of choice, OK on face

Question 16

Tumid

Answer 16

Dermal component only, no epidermal change

Question 17

Dermatomyositis

Answer 17

-Heliotrope rash, shawl sign, muscle weakness, gottrons papules on knuckles, malar edema
-May be caused by Toxo
-May have CREST like symptoms
-Jo-1/Mi-1
Steroids

Question 18

Lupus diagnostic citeria

Answer 18

4/9
-Malar Rash
-Photosensitivity
-Mouth ulcers
-Arthritis
Serositis
-Renal
-Discoid Plaques
-Neuro
-Heme
-Immuno
-ANA

Question 19

Lupus Tests

Answer 19

Skin Biopsy: granular Ig deposition, epidermal thinning and degeneration of basal cells

• low complement
• Always test kidney

Question 20

ANA in Lupus

Answer 20

DNA is SLE

• SSA in SCLE
• not in discoid

Question 21

Sun Sensitivity in lupus

Answer 21

Yes in SLE and SCLE

Much less in discoid

Question 22

Systemic Sclerosis

Answer 22

• SCL-70, topoisomerase
• Th cells lead to release of IL-4 and activation of TGF-a,b which cause increase collagen production and decrease destruction
• sclerosis and periungal telangectasis
• Risk is systemic involvment
• Skin involvment can be ulcers on skin from raynauds

Question 23

CREST

Answer 23

-Centromere

Question 24

Morphea

Answer 24

Pale indurated plaque

• Can cause bone dysgrowth and hemifacial atrophy
• Halo
• Plaque is morphea, macular is sclerosis

Question 25

Eosinohilic Fascitits

Answer 25

• Indurated skin following URI
• There will be eosinophils in the blood but often not in the tissue
• Tx is steroids and usually resolves

Question 26

Lichen Sclerosis

Answer 26

• Pale macule
• Can cause SCC
• Often vaginal
• Extracellular Matrix Protein 1

Question 27

Mixed CT

Answer 27

• U1 RNP

- Overlap with signs of others

Question 28

RA

Answer 28

Rheumatoid Nodules

Question 29

-Reiters

Answer 29

• Arthritis, Urethritis
• May cause erythroderma
• Keratoderma Blennohragicum (pustular eruption on feet)

Question 30

Paniculutis

Answer 30

• Think of EN
• Also could be dialysis related with Ca
• Get and inscisional bx as punch is often not enough