74(5) Cutaneous Manifestations of Hypercoagulable States Flashcards
Petechiae are a sign of what underlying issues?
- Trauma (marked relative to degree of trauma if low plts or aberrant hemostasis)
- Thrombocytopenia
- Vasculitis
- Anticoagulant therapy
Petechiae are pinpoint (
Workup for petechiae?
- No diagnostic tests necessary.
- In certain scenarios obtaining skin bx may help r/o occult vasculitis.
- W/u for thrombocytopenia may be warrented.
What do purpura signify?
Both hypo- and hypercoagulable states may present with purpura can distinguish w/ hx, px and labwork (PTT, INR)
- Hypocoagulable states often manifest as nonpalpable purpura/ecchymoses at sites of trauma.
- Hypercoagulable states often exhibit retiform purpura (stellate-appearing purpura w/ incomplete net-like vascular background).
- Retiform purpura implies systemic disease causing microvascular occlusion requiring thorough workup/bx. Ddx: Vasculitis, vasculopathy, or occlusion.
*Palpable purpura often indicate a vasculitic disorder.
What is Livedo Reticularis?
An erythematous to violaceous, lacy, net-like, exaggerated venous pattern visible in states of slow venous flow. Classically presents on LE, exacerbated by cold temps, reverses w/ warming but once established is permanent.
Ddx: physiologic response to cold, upstream occlusive process of arteries/arterioles (eg. vasospasm or luminal osbstruction), or neurologic disorders affecting vascular tone.
What is Livedo Racemosa?
When livedo reticularis is fixed, the lacy pattern includes broken circles, and the patterns dont reverse w/ warming.
Ominous sign of systemic disease.
Take bx from pale perilesional skin, may reveal vasculopathy or vasculitis, sludging of RBCs, intraluminal thrombi, or arterial obliteration.
What is Livedoid Vasculopathy (LV)?
A syndrome associated with hypercoagulability comprised of painful, punched out lower leg and foot ulcers on a background of LR or retiform purpura that health w/ Atrophie Blanche.
Must workup for hypercoagulable state and connective tissue dis.
AB = atrophic, stellate white scars bordered by telangiectasia and hemosiderin deposition)
Describe venous ulcers?
Typically appear in the “gaiter are” (medial ankle to mid-calf), are shallow with fibrinous material overlying granulation tissue at the base, and have varying degrees of pain. There is often a background of pitting edema, varicosities, lipodermatosclerosis, and hemosiderin deposition.
Chronic venous insufficiency can result from longstanding, recurrent superficial and deep venous thromboses associated w/ primary or secondary HS.
Describe arterial ulcers?
Classically appear over the lateral malleoli or distal phalanges as painful, punched out ulcers w/ purpuric borders and prominent eschar. The affected limb may feature pallor and diminished arterial pulses.
Upstream arterial occlusion can results from: atherosclerosis, vasospasm, underlying syndrome, or combination of above.
Workup for atypical nonhealing wounds, esp LE ulcers?
If no signs of venous or arterial insufficiency, do workup for underlying hypercoagulable state including: cryoglobulinemia, cryofibrinogenemia, and antiphospholipid syndrome. Calciphylaxis may cause chronic painful ulcers in pts w/ an underlying HS.
What does atrophie blanche suggest?
Common presentation of livedoid vasculopathy.
Purpura fulminans?
Purpuric lesions that enlarge and become vesiculated - produce hemorrhagic bullae with subsequent necrosis and black eschar formation.
Hypercoagulation workup?
- PT and PTT (warfarin, liver dis prolong PT; antiphospholipid syn can prolong PTT)
- Protein C and S activity (Prot C dec on Warfarin; Prot S exclude acquired causes first, dont test in acute setting)
- Homocysteine level (if inc - exclude folate/B12 def;
- Factor V Leiden (Genetic test - mut present or absent)
- Antiphospholipid antibody panel (ie anticardiolipin IgG and IgM, lupus anticoagulant, and B2-glycoprotein-1) (if any pos repeat in 12wks)
- Cryoglobulin (if neg but suspicion high, ensure lab stored at correct temp)
- Cryofibrinogen (“ “)
- Antithrombin III (Genetic test - pos or neg)
- Prothrombin G202120A
Principles of proper wound care?
- Optimizing granulation tissue formation
- Decreasing bacterial burden
- Decreasing the inflammatory response
- Maintain proper moisture balance
How does FVLM present?
May be associated w/ nonspecific cutaneous findings (eg petechiae, purpura, livedo reticularis, livedoid vasculopathy (reticulated purpura w/ atrophie blanche), and LE ulcers).
Usually occurs later in life.
How does Prothrombin G202120A mut present?
Tender erythematous and ulcerated papules of the LEs, ankle edema, LV and AB. Can mimic chronic LE venous insuf, athersclerotic peripheral arterial disease, inflam vasculitis or PG.
How does ATIIID present?
Cutaneous manifestations are uncommon.
Recurrent venous or arterial thrombosis at an early age.
Neonatal purpura fulminans has been described.
How does Protein C present (homo, hetero, acquired)?
Homozygous: rare, life-threatening bleeding d/o that can present in the immediate neonatal period w/ purpura fulminans that is usually fatal.
Heterozygous: AD condition assoc w/ DVT and superficial phlebitis later in life.
Acquired: may present as calciphylaxis w/ skin findings mimicking warfarin induced skin necrosis.
How does Protein S deficiency present?
Homo: Purpura fulminans in neonates.
Hetero: Associated with Sneddon Syndrome and WISN.
Acquired: chronic kidney and liver disease, Vit K def, DIC, varicella inf (Postinfectious purpura fulminans). May present w/ LV.
- Sneddon’s syndrome is a non-inflammatory arteriopathy in which livedo reticularis is associated with cerebrovascular disease. It is slowly progressive and may go undiagnosed for some time. It may be seen in patients with an autoimmune disorder - eg, antiphospholipid syndrome or systemic lupus erythematosus (SLE).
How does Hyperhomocysteinemia present?
Can present w/ LV in addition to pale or pink skin, a malar rash and fine hair.
How does sickle cell disease present?
Complications include: acute pain episodes, increased rates of vasoocclusive crises (stroke), and pregnancy complications.
VTE is a common presentation. Typically present to dermatologists w/ painful leg ulcers a/ an AB-like appearance w/ pain out of proportion compared to clinical presentation.
Skin ulcers occur in areas w/ little subcut fat, thin skin, and dec blood flow (eg. ant tibia, dorsal feet, achilles tendon, or ankles). Usually starts around 20yo. Men more likely to develop ulcers than women. The ulcers resemble venous and arterial ulcers but involves deeper tissue.
How does warfarin induced skin necrosis present?
Usually occurs 3-5d after initiating warfarin w/ large loading dose or w/out heparin bridge. Skin necrosis affects areas of the body w/ high fat content and decreased blood supply (eg. breasts, buttocks, abdo, thighs, calves).
First signs are pain and purpura that then progress to full-thickness skin necrosis.
Can also precipitate calciphylaxis.
Prevent by initiating Heparin for 4-5 days prior to starting warfarin.
Tx: stop warfarin, start heparin, give vit K or FFP to reverse warfarin effect.
How does HIT present?
Thrombocytopenia and increased risk of venous or arterial thrombosis. Generally presents w/ skin necrosis (there are reports of HIT causing skin necrosis w/out a drop in plts)
Tx: stop heparin, start another anticoag (Lepirudin, Agartroban, Rivaroxiban). Good local wound care.
How does antiphospholipid syndrome present?
Can present w/ LV, skin ulcers or PG.
Tx: After single episode of arterial or venous thromboses, tx w/ warfarin (INR 2-3), tie controversial.
How does TTP present?
Pentad of:
- Fever
- Hemolytic anemia (microcytic anemia, high bili)
- Thrombocytopenia
- Renal failure
- Neurologic symptoms.
Cutaneous: petechial and purpuric lesions.
Confirm w/: anti-ADAMTS13 Abs, low plts, inc bili, inc LDH, neg Coombs test.
Tx: plasmapheresis and rituximab.
