Raynauds
14% with Raynauds progressed to CTD - predictors are F, old age at onset, initial positive ANA, thickening of fingers
Pallor - cyanosis - rubor
High homocysteine in 1’ and 2’ raynauds
Pts with SS and RP have ⬆️endothelin which correlates with nailfold changes and advanced dz
Raynauds
Cold, CTD, nervous system (CRPS), pianist/typists, endocrine, nipple variant with pain during lactation
Meds- beta blockers (incl eye drops), IFN, bleomycin, ergot, CSA, vinyl chloride, cocaine
Raynauds dz
Usually BL, gangrene in <1%
Tx CCB - sildenafil, SSRIs, losartan (angiotensin II R antagonist), bosentin (endothelin R antagonist), iloprost, Botox
Erythromelalgia
Primarily feet
Min to days, triggered by exercise or heat
Romiplostin may case (induced thrombopoeisis), also isopropyl alcohol and mushroom (clitocybe)
Tx - cold, ASA, amytryptaline 1% in ketamine 0.5% gel
Neurological sequale like neuropathy, small fiber neuropathy
AD form, SCN9A encodes a na channel
Red ear syndrome
Relapse of redness/burning, affects both ears but usually one at a time
More common in winter, precipitatited by touching and exposure to warmth
Associations- trigeminal or glossopharyngeal do, lupus, migraines
Tx - tcas
Lobe involved vs Relapsing polychomdritis
Med induced livedo
Amantadine, quinidine, quinine, mino, gemcitabine, heparin, IFN, bismuth
Parkinson’s, moya moya, factor 5 leiden, oxalosis, graves (with anticardiolipin ab), TB, atrial myxoma, syphilis
Pt with SLE are apt to have more severe dz if livedo
Vasculopathy
Etiology is thrombosis (vs inflammation of vessels)
Livedoid vasculopathy evaluation
Anticardiolipin ab, lupus anticoagulant, factor v Leiden, protein c and s, antithrombin III, prothrombin, cryoproteins, homocysteine
Mononeuropathy multiplex associated
Mixed cryoglobulinemia
Hcv in >90%
Waldenstroms hyperglobulinemic purpura
Episodic showers of petechiae of the legs
Peak incr of IgG on spep
RF pos usually, 80% Ro/La pos
Sjogrens, RA, adverse fetal outcome, hep c in men
LCV in 1/3 - higher risk of aurticular inv
Benign chronic course
WM - proliferation of B cells, elevated igM, gum bleeding, epistaxis, purpura, old men, urticaria, disseminated xanthoma, amyloid, deck chair sign
2 types of skin lesions - Igm aggregates (translucent papules, subepi blister, pas pos but neg Congo red fissured pink deposits) and infiltrate of neoplasticism lymphocytes
Drug induced purpura
NSAIDS, allopurinol, cephalosporins, thiazides, gold, sulfa, hydralazine, phenytoin, quinidine, PCN, Emla in 30 min (toxic effect on cap endothelium), Tylenol use in mono, pseudoephedrine (ppd like rash)
Benadryl + pyrithyldione cause purpuric mottling with necrosis
Purpuric contact derm
Rubber and textile dyes
Superficial migratory thrombophlebitis
Linear painful red induration
Anything that causes hypercoag
Mondors = breast
Postcardiotomy syndrome
2-3 weeks after
Fever, pleuritis, pericarditis, arthritis, petechiae
Eculizumab
C5 ab
Used in PNH
Achenbach syndrome
Rapid onset and resolution paroxysmal hand hematoma
Painful bruising sundrome (Gardner diamond syndrome, psychogenic purpura)
Localized purpura in young women with personality do
Painful bruises on ext > trunk or face resolves in 5-8 d
Pigmented purpuric contact dermatitis
Nickel, fragrance, disperse blue dye
Purpuric agave derm from agave tropical plant
LCV recurrence rate
10%
LCV
Immune complex mediated
Vasculitis work up
CBC, Ua, aso, hep b and c, ana, rf screening
Consider spep, cryoglobulins, ancas
LCV
Rest, elevation, NSAIDS - steroids or colchicine - dapsone
Concentrated along wallaces line
UV MC systemic sx
Arthritis 50%
May also have GI, obstructive pulmonary dz, glomerulonephritis
Rare hypocomlementemic form with Jacquets arthropy and valvular heart dz
Golfers/exercise related vasculitis
Not a true LCV
1/2 are on antithrombotic agents
PAN
Necrotizing vasculitis of small and medium a. AT branching pt
2 forms - benign cutaneous and systemic
10 dx criteria - livedo racemosa, polymorphonuclear arteritis, leg pain/ myopathy/weakness, neuropathy (foot drop), hep b pos, wt loss > 4kg, testified pain, DBP > 90, elevated bun/cr, arteriographic abnormality
Men age 50
Cutaneous vasculitis identical to PAN seen in IVDU
5-7% associated with hep b
Skin involved in 50% of pt with systemic form, MC subcutaneous nodules along bld v.
Livedo + subq nodules = PAN
Hep b associated PAN has skin inv in 30%
P anca is predominant (also MPA) - hep b rarely panca pos
Death due to renal, cardiac or GI
Cyclophosphamide +- steroids, IFN if hep
Cutaneous PAN
10% of PAN pts with limited sys and only skin
Neuropathy in 20%
MC childhood pattern
Hep b, c, crohns, takayasu arteritis, RP, strep, tb, minocycline
Asa and NSAIDS first line
Antiproteinase 3 ab relatively specific for
Wegeners and MPA
Both p anca and c anca
Think drug induced
Thrombophlebitis in 8% of anca vasculitis
Microscopic polyangitis
Most with constitutional sx
80-90% with segmental necrotizing and and crescenteric glomerulonephritis
Pulmonary capillaritis complicated by hemorrhage
Vasculitis neuropathy and eye dz
Anca pos in 70%, MC p anca
Separated from PAN by glomerulo, pulmonary sx, and abscense of HTN and microanyeurisms
MC initial presentation of wegeners
Rhinnorhea, sinusitis, nasal mucosal ulceration, nodules in nose, larynx, trachea or bronchi
Granulomas may occur in ear or mouth
45% with skin
Nodules on ext ext, may ulcerated - livedo rare
Focal necrotizing glomerulonephritis in 85%
C anca pos (anti pr-3, cytoplasmic)
Bactrim decreases relapse rate due to decreased staph carriage, a known trigger
Cocaine associated vasculitis
Cutaneous lesions resemble LCV but ecchymosis and necrosis is MC
*earlobe predilection
Agranulocytosis is seen
cANCA, reacts with human neutrophil elastase (vs wegeners and microscopic polyangitis which are hne-anca negative)
Eradication of staph carriage if prominent nasal findings
Giant cell arteritis/temporal arteritis
Whites
UL HA, ear and parotid pain, mastication induced pain
Fever, anemia, high esr, polymyalgia rheumatica (all day stiffness/pain)
Blindness is most severe complication
Actinic granuloma may develop - actinically induced?
Affected a. May be pulsating or necrosis may occur
2 cm a. Biopsy
TNF polymorphisms may show genetic predisposition
Steroid responsive
Takayasu arteritis
F, 2nd or 3rd decade
EN and PG like lesions (UE)
Thrombophlebitis obliterans
Instep and foot claudication
Diffuse dermal angiomatosis
Breast, next to scars from breast reduction
May be telangectasias, purpura or ulceration
Nipple spared
RF for hypercoag
Chronic ischemia leads to vascular proliferation
Early finding in KD (first week)
Desquamating perianal eruption
In KD may also see pincer nails, pseudo intestinal obstruction, facial n. Paralysis, PSO 10-20 d later (superantigens trigger PSO)
CAA in 20-25% of untreated kids, 3-5% of treated (after day 10 with elevated plt)
Increased superantigens in stool
Kids of parents who had KD have twofold risk of developing
Linked to CAMK2D
MC sign in HHT
Epistaxis, GI bleed is the presenting sign in 25%
Worsening epistaxis may herald high output heart failure
Pregnancy may exacerbate
Dental prophylaxis due to risk of cerebral abscess
Telangectasias first appear on the undersurface of the tongue/mouth floor at puberty
HHT with juvenile polyposis - MADH4
Increased VEGF levels in HHT
OCPs, topical tranexamic acid for epistaxis
Thalidomide blocks VEGF and reduces GI bleeding and transfusion dependence
MCC of lynphedema in US
Postsurgical
Lipedema
BL symmetric LE enlargement due to subcutaneous fat deposition
Feet are spared, buttocks to ankles affected
No response to compression
Skin fold at the base of 2nd toe is too thick to pinch in lymphedema but nl in lipedema (stemmers sign)
Nonne milroy
Painless edema, pitting (vs lymphedema that is usually non)
Not associated with other d/o
MC UL
Emberger syndrome
Primary lymphedema associated with myelodysplasia
Skeletal, deafness, multiple warts
WILD
Warts, immunodeficiency, lymphedema, anogenital dysplasia
Th reduced
Hypotrichosis-telangectasia-lymphedema syndrome
Vascular dilations on palms/soles
Sox18
Secondary lymphedema
HL and KS (need chemo bc lymphatics inv)
Postinflammatory lymphedema from strep cellulitis in post op CABG patients
Secretan syndrome - facticial lymphedema from from blunt tx of dorsum hand
Podoconiosis- mossy foot, noninfectious lymphedema, tropical regions of Africa, S. America and India from walking barefoot in soil of volcanic origin
Hand edema persistent in divers from suit constriction
Marshall white syndrome and bier spots
Mottling from BP cuff