Scleroderma/morphea/lichen sclerosus Flashcards
What is the biggest cause of mortality in systemic sclerosis?
Lung involvement (used to be kidney)
Do you see eosinophils in the tissue bx in eosinophilic fasciitis?
Not necessarily, you have to go off the peripheral blood test.
What is a key component of nephrogenic systemic sclerosis diagnosis?
It retains the CD34 cells (there are increased numbers of CD34 cells) this is lost in the morphea and systemic sclerosis
Does morphea turn to systemic sclerosis?
No
Why is it important to see if morphea crosses joint lines?
If this occurs you have to treat much more aggressively as this can affect the joint function
Why do you have to treat the morphea?
The effects of the scarring is irreversible once it occurs. You can treat the disease but the scarring can continue.
What is the most common variant in adults of morphea?
Morphea en plaque
What happens to the adnexal structures in morphea?
You get loss of the adnexal structures, so look for that clinically.
What specialty must be consulted for morphea on the head/face?
Ophthalmology, you can get the involvement of the eye.
How should you dose the topical CS in lichen sclerosis et atrophicus?
You should treat aggressively, you don’t need to take the week break and such. You already have the atrophy.
What are the 3 major clinical varients of systemic sclerosis?
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- Systemic sclerosis sine scleroderma
What is the first presenting sx in all 3 of the tyeps of systemic sclerosis?
Raynouds phenomenon, all have capillary nailbed changes.
Clinical of limited cutaneous systemic sclerosis?
Formally known as CREST -Early-onset of raynouds that predates the other sx’s. -Then the progression of distal skin findings, sclerosis of hands, face -Internal sx’s occurs 10-15 years later after onset of skin findings. Usually pulmonary artery hypertension
What antibody is a/w limited cutaneous systemic sclerosis?
Anti-centromere
What are the 2 “other” types of systemic sclerosis?
- Pre-systemic sclerosis (full extent of skin dz not reached)
- Overlap systemic sclerosis (polymyositis, SLE, CREST (calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia)
Clinical of diffuse systemic sclerosis?
Sudden onset of Raynauds, rapid onset of progressive and widespread sclerosis (lasts 12-18 months), 90% have systemic sx’s within 5 years (ILD in lungs mc, kidney disease also common)
Clinical of systemic sclerosis sine scleroderma?
Basically limited cutaneous systemic sclerosis w/o the skin findings.
Physical exam findings in systemic sclerosis?
RP - mat telangiectasias - dilated capilary loops - cutaneous ulcers - calcinosis cutis - dyspigmentation (diffuse hyperpigmentation in sun-exposed areas or pressure related areas, leukoderma of SSc ‘salt and pepper sign’)
Tx for cutaneous sclerosis?
UVA1 tx -maybe tyrosine kinase inhibitors like imatinib (experimental)
Tx for Renaud’s phenomenon?
1st line: cold avoidance, hand/feet warming products, discontinue all tobacco products 2nd line: CCB: nifedipine (SR 30mg BID) or amlodipine (2.5-10 mg /day) 3rd line: Sildenafil, prazosin, arb’s, endothelin receptor antagonists (bosartan)
Tx for calcinosis cutis in systemic sclerosis?
- Low dose warfarin
- CCB
- Physical (surgery, laser, etc)
Does the degree of skin sclerosis correlate with systemic disease in systemic sclerosis?
No
Most common cause of death in systemic sclerosis?
Pulmonary dz (ILD >> PAH) is mcc of mortality
What are the 3 cutaneous clinical phases of systemic sclerosis?
(i) early edematous - puffy hands and pitting edema of digits
(ii) indurated - hardening of skin with taut and shiny appearance
(iii) atrophic - potential / gradual softening of skin