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
ventral pterygium
scleroderma
most common cause of death in scleroderma? How do you screen for it?
interstital lung disease. Screen with CT and PFTs
pulmonary problem in diffuse scleroderma
ILD
pulmonary problem in limited scleroderma? What does limited scleroderma look like clinically?
pulmonary hypertension. Also with sclerosis of distal fingers and face.
most common site of visceral disease in scleroderma?
esophageal dysphagia and dysmotility causing aspiration and esophagitis
diffuse systemic sclerosis patients should be given what to prevent scleroderma renal disease?
ACE-inhibitors
what can you give for digital ulcers in scleroderma?
IV iloprost (prostacycline analogue), Bosetan (endothelin receptor antagonist, prevent new ulcers)
what is scleroderma’s earliest presenting features?
raynauds and hand edema
what’s a new biomarker for skin and lung fibrosis and PAH?
CXCL4
does eosinophilic fasciitis have raynauds phenomenon?
no it does not
where is the eosinophilia in eosinophilic fasciitis?
pheripheral eosinophilia»_space; tissue eosinophilia
what is a new serologic marker of eosiniphilic fasciitis activity?
metalloproteinase inhibitor-1 (TIMP-1)
cause of nephrogenic systemic fibrosis?
gadolinium-containing contrast exposure in the setting of acute kideny injury or CKD
onset of NSF fibrosis?
about 2-4 weeks after gadolinium exposure
What do you see in the eyes of nephrogenic systemic fibrosis patients?
scleral plaque (white yellow plaques) with dilated capillaries in patients <45yo
How does histopath of NSF fibrosis different from scleromyx?
the fibrosis of NSF goes down to the fat and fascia
NSF fibrosis is mediated by what cells?
CD34+ and Procollagen I+ cells
Tx for NSF?
skin is refractory to treatment. The treatment of kideny disease is most important
What types of scleredema can have facial involvement?
Types 1 (post-infectious) and Type 2 (monoclonal gammopathy)
Name the 3 types of scleredema?
Type 1 Strep pharyngitis. Type 2 IgGk monoclonal gammopathy. Type 3 Diabetes.
What is scleromyxedema associated with? Gammopathy. Extracutaneous involvement.
IgG lamda. GI:dysphagia, MSK: arthritis, proximal muscle weakness, carpal tunnel, Neuro: peripheral neuropathy
Glomeruloid hemangiomas associated with?
POEMs (polyneuropathy, organomegaly, endocrinopathy, M -protein, skin changes)
Diabetic cheiroarthropathy
symmetric painless loss of joint mobility , stiffness of joints with scleroderma-like thickening of the dorsal aspect of the hands and feet (prayer sign)
What exogenous substances cause scleroderma?
aniline contaminated rapeseed cooking oil, L-tryptophan (eosinophlia-myalgia syndrome), Polyvinyl Chloride, Bleomycin, taxanes (diffuse leg edema followed by sclerosis)
What agents can cause sclerosis at injection sites?
Vitamin K, bleomycin, opiods (methadone, pentazocine)
Familial and acquired perforating dermatosis difference? What is perforating?
Collagen. Familial reactive perforating is rare childhood onset at sites of trauma vs acquired perforating dermatosis is in adults and d/t diabetes and/or renal failure
Wher do you find EPS commonly on the body?
serpiginous plaques with keratotic papules along the rim on the lateral neck
MAD PORES is for what disease? What does it stand for
EPS (Marfans, Acrogeria, Downs, Penicillamine/PXE, Osteogenesis Imperfecta, Rothmund-Thomson, Ehers-Danlos, Scleroderma)
What is perforatin calcific elastosis?
Transepidermal elimination of clacified elastic fibers periumbilical multiparous black women
Anetoderma on histopathology
Normal H&E. elastic tissue stain shows near complete loss of elastic fibers in papillary and reticular dermis;fragmented elastic fiber remnants visible
What is follicular atrophoderma?
Ice pick depresseions on the doral hands/feet and cheek since childhood
What syndrome is follicular atrophoderma associated with?
Bazex Dupre Christol syndrome (follicular atrophoderma, BCC of the face, milia, hypohidrosis anove neck, hypotrichosis). Think of a priveliged french person with acne scars, cosmetic treatements that made him hairless, sweatless)
What is atrophoderma vermiculatum?
A variant of follicular atrophoderma on the face/cheeks exclusively
What syndrome is atrophoderma vermiculatum associated with?
Rombo (atrophoderma vermiculatum, BCC, milia, acral erythema, peripheral vasodilation with cyanosis) and Naicolau Balus (eruptive syringomas, atrophoderma vermiculata and milia)
Perforating GA most commonly on? Perforating with what?
Dorsal hands and fingers. Perforating with degenerated collagen
Interstitial granulomaous drug eruption culprit?
CCBs, ACE, TNFalpha
NXG clinical presentation?
Yellow-xanthomatous plaques in the periorbital region
NXG associated with what gammopathy? What other organ system can it also affect?
IgG kappa. Enodcardial involvement. Hepatosplenomegaly common.
Cutaneous Crohns disease can manifest as what skin diseases?
Erythema nodosum, PG, pyostomatitis vegetans, EB aquisita (IBD is the most common cause of EBA), acrodermatitis enteropathical-like syndrome
Genital crohns can look like what?
labial or scrotal edema with erythema +/- ulcerations and fissures
What does oral crohns look like?
cobblestoning of buccal mucosa, pyostomatitis vegetands, cheilitis granulomatosa
Sarcoidosis is a multisystem grnaulomatos disease cause by upregulation of what type of T cells?
Th1 cells
What are three drugs that can cause sarcoidosis?
Hepatitis C pts on IFN-alpha, and ribavirin, HIV patients on HAART, TNF alpha inhibitors
What percentage of sarcoid patient have skin lesions?
35%
why do sarcoid patients have hypercalcemia?
due to calcitriol synthesis by arcoidal granulomas (convert 25-hydroxyvitamin D) to more active 1,25-dihydroxyvitamin D
What laboratory or imaging tests do you need for sarcoidosis? What would it show?
CXR/CT scan to detect hilar lymphadenopathy, PFTs to detect restrictive lung disease (decreased TLC, DC, Vital Capacity), increased ACE levels (useful for monitoring response to treatment)
Is measuring ACE levels in Sarcoidosis useful?
Yes, useful for monitoring response to treatment
What is darier-roussy? Whats the prognosis?
subcutaneous sarcoid, painless, firm, often multiple lesions. A/w good prognosis
whats lofgren syndrome? Whats the prognosis?
acute form of sarcoidosis with erythema nodosum and hilar adenopathy and fever and acute iritis and polyarthritis. a/w good prognosis
What’s heerfordt’s syndrome?
Uveitis + parotid gland enlargmeent + fever + cranial nerve palsy (facial nerve)
Blau syndrome?
Early onset sarcoid-like disease caused by NOD2 mutation. Triad of skin, eye, and joint disease
what is the prognosis for lupus pernio?
strongly associated with chronic sarcoid lung (75%) and upper respiratory tract disease (50%) along with cystic degeneration of the bones. Poor prognosis
what color is the most comon cause of delayed reaction in tattoo?
Red (mercuric sulfide, cinnabar)
what colors are the most comon causes of photoallergic reaction in tattoo?
Yellow (cadmium sulfide), red ink (cadmium selenide), yellow-red (azo dye)
what type of reaction does red tattoo have on histopath?
Lichenoid dermatitis sor pseudolymphoma
what type of reaction does aluminum tattoo have on histopath?
granulomatous
Silica foreign body reactions are from penetrating injuries from what 4 things over 25 years?
Sand, soil, rocks, glass
what type of reaction does silica foreign body reaction have on histopath?
sarcoidal granulomas containing colorless, birefrigent crystals
Where do you find zirconium? What can it cause clinically?
in antiperspirants. Causes soft brown papules in axilla.
what does a zirconium foreign body reaction look like on histopath?
no polarizable particles seen
what 3 foreign body materials are too small to be seen by polarized light microscopy?
zirconium, beryllium, aluminum
Where do you find beryllium What can it cause clinically?
found in the manufacturing of fluorescent lights. Inhalation can cause granulomatous lung disease. Localized puncture wounds by fluorescent bulbs can cause slowly healing nodules/ulers
how do you diagnosis systemic berylliosis?
bronchioalveolar lavage
how do you get alumunium in the skin?
vaccine injection sites, hemostasis
how do you get zinc in the skin?
zinc-containing insulin shots
what does a zinc foreign body reaction look like on histopath?
birefringent rhomboid crystals
how do you get starch in the skin?
contamination of rounds from surgical gloves with starch lubricant
what does a starch foreign body reaction look like on histopath?
oivoid basophilic starch granules that stain postive with PAS
what does a ILK foreign body reaction look like on histopath?
Granuloma with central pale bluish material that resembles mucin on H&E
Is Silica PAS positive or negative?
negative
Is talc PAS positive or negative?
negative
Is zinc PAS positive or negative?
negative
Is keratin PAS positive or negative?
negative
Is sea urchin spine PAS positive or negative?
negative
Is sutures PAS positive or negative?
negative
Is arthropod parts PAS positive or negative?
negative
Is starch PAS positive or negative?
positive
Is cactus spines PAS positive or negative?
positive
Is wood splinters PAS positive or negative?
positive
Macrophage histiocytes stain positive for what 2 markers?
CD 68 and HAM 56
Dermal dendritic cells stain positive but singly for what 2 markers?
Factor 13a for papillary dermis dendritic cells and CD 34+ for reicular dermis dendritic cells
Langerhan cell histiocytosis have mutations in what?
BRAF V600E mutation (60%) and MAP2K1 mutations
Langerhan cell histiocytosis stain negatively for what?
Factor 13a, CD 68, HAM 56