Gen Derm 3 Flashcards
What is the most common type of juvenile idiopathic arthritis? What joints does it affect?
Oligo/pauciarticular arthritis (60%). Usually favors knees.
There are two types of oligo arthritis type JIA. What are they?
Type 1 is the most common type. In 1-8yo, have uveitis in 50%. ANA positive, RF negative. Type 2 is in 9-16yo, ANA -, RF-
Is JIA an autoimme disease?
No, should be classifed as an autoinflammatory sndrome as it is due to activation of innage immune system’s iL1
How does JIA present?
High episodic fevers with salmon pink rash in the late afternoon/evening
What does the two JIA rashes look like?
There’s the pink evanesent rash that favors the waist and axilla, and also koebnerizes. And there’s another one with persistent papules and plaques
What does the two JIA rash look like on histopath?
The evanescent one looks like urticaria but with more neutrophils. The persistent papules one look like urticaria + parakeratosis and superfically scattered necrotic keratinocytes
What are two types of good treatments for juvenile stills?
IL1 receptor inhibitor: anakinra, rilonacept, canakinumab and IL6 receptor inhibitor tocilizumab
What is a classic arthritic finding for adult onset stills?
Carpal ankylosis: limited range of motion with minimal pain
What does ANA, RF, ESR, CRP and ferritin look like for adult stills?
ANA RF negative for adults. ESR/CRP high and Ferritin >4000
If the JIA kid has polyarthritis instead of oligoarthritis, and the ANA and RF are positive, what does that mean?
That he has erosive joint disease and like will get rhueumatoid nodules
Most common type of morphea in adults and kids
plaque type in adults, linear in kids
What Borrelia spp is associated w morphea in europe and japan?
Borrelia afzelii and Borrelia garinii
most common site for linear morphea
legs
antibody for linear morphea?
ssDNA
What does atrophoderm of Pasini and Pierini look like clinically and histologically?
Hyperpigmented oval atrophic plaques with cliff drop borders. On histopath, you have decreased dermal thickness
How is linear atrophoderma of moulin different from other types of morphea?
less induration and less pigmentary changes
NSF, Scleromyxedema, morphea, scleroderma. Which ones lose CD34?
Morphea and scleroderma lose CD34+ dendritic cells
What antibody do all forms of morphea have?
All forms of morphea are positive for anti-topoisomerase 2 antibodies. They are all negative for topoisomerase 1 (scl70) and centromere. Those are positive in systemic sclerosis
Linear morphea and generalized morphea are most likes to be + for what antibodies?
ANA and antihistone and ssDNA
What Xray finding do you see in linear morphea?
Melorheostosis. Which is roughening long bone surfaces underlying area of linear morphea. Resembles wax dripping. Also seen in buschke Ollendorff
how do you treat superficial generalized morphea?
nbUVB
how do you treat deep generalized morphea?
MTX, pulsed intravenous corticosteroids + MTX
What is the profibrotic cytokine seen in scleroderm, morphea and eosinophilic fascitiis?
TGF BETAAAA
Nail dermoscopy for scleroderma?
dilated capillary loops alternating with capillary drop out