Ch 10 Connective Tissue Disorders Flashcards
SLE Ab
dsDNA, smith (u1,u2)
phospholipid
Drug induced lupus
histones
subacute lupus
Ro
La
-placenta and heart block
Dermatomyositis
Jo-1
Mi-2
Systemic Sclerosis
SCL-70 topoisomerase
CREST
Centromere
Mixed CT
U1-RNP
Lichen Sclerosis
extracellular matrix protein 1
Localized CT
Discoid lupus
Morphea
Intermediate
Subactue Lupus
- Juvenile Dermatomyositis
- Limited scleroderma (CREST)
Systemic
SLE
Adult dermatomyositis
Systemic Sclerosis
Overiding path/histo of CT
Dermal atrophy, arthritis, fibrosis, epidermal thinning
SLE
-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
Subacute
- Less
- Ro/La
- Plaquenil is better
Discoid
- 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
Tumid
Dermal component only, no epidermal change
Dermatomyositis
-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
Lupus diagnostic citeria
4/9 -Malar Rash -Photosensitivity -Mouth ulcers -Arthritis Serositis -Renal -Discoid Plaques -Neuro -Heme -Immuno -ANA
Lupus Tests
Skin Biopsy: granular Ig deposition, epidermal thinning and degeneration of basal cells
- low complement
- Always test kidney
ANA in Lupus
DNA is SLE
- SSA in SCLE
- not in discoid
Sun Sensitivity in lupus
Yes in SLE and SCLE
Much less in discoid
Systemic Sclerosis
- 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
CREST
-Centromere
Morphea
Pale indurated plaque
- Can cause bone dysgrowth and hemifacial atrophy
- Halo
- Plaque is morphea, macular is sclerosis
Eosinohilic Fascitits
- Indurated skin following URI
- There will be eosinophils in the blood but often not in the tissue
- Tx is steroids and usually resolves
Lichen Sclerosis
- Pale macule
- Can cause SCC
- Often vaginal
- Extracellular Matrix Protein 1
Mixed CT
- U1 RNP
- Overlap with signs of others
RA
Rheumatoid Nodules
-Reiters
- Arthritis, Urethritis
- May cause erythroderma
- Keratoderma Blennohragicum (pustular eruption on feet)
Paniculutis
- Think of EN
- Also could be dialysis related with Ca
- Get and inscisional bx as punch is often not enough
