Other vascular disorders Flashcards
What are the two main types of telangiectasia?
Primary and Secondary
What are the 6 main primary types of telangiectasia?
Spider, Hereditary benign, angioma serpiginosum, unilateral nevoid telangiectasia, generalized essential telangiectasia, cutaneous collagenous vasculopathy
What are the main types of secondary telangiectasia?
-Photodamage post-radiation, telangiectatic rosacea, involuted hemangioma, estrogen-related (liver dz, pregnancy, hormone replacement tx, OCP’s), corticosteroid use, AICTD ( CREST), HIV (chest), TMEP (mastocytosis), carcinoid, and drugs (CCB’s –> telangiectasias on sun-exposed areas)
Genoderms associated with telangiectasia?
CMTC, HHT, A-T, K-T syndrome, Rombo, Bloom, Rothmund-Thomson, dyskeratosis congenita, XP, Goltz (within Blaschko’s lines), prolidase deficiency, and hypotrichosis-lymphedema-telangiectasia syndrome
What is angioma serpiginosum?
Usually occurs in women <20 y/o
Pinpoint punctate blanching red-purple petechiae in clusters/patches in a serpiginous pattern typically on one extremity
What is unilateral nevoid telangiectasia?
Telangiectasias in the trigeminal/upper cervical dermatomes and can be in Blashko’s lines
Can be related to increased estrogen
What is generalized essential telangiectasia?
Typically in adult women
Starts on the lower extremities and spreads to other larger areas
What is cutaneous collagenous vasculopathy?
Large anatomic areas and does not have a tendency to affect women
- Look for diffuse, asymptomatic, blanchable, dusky red macular telangiectasias symmetrically spread on the legs
- Can progressively spread to trunk and other extremities
Histology of cutaneous collagenous vasculopathy?
Ectatic dermal vessels w/ thick hyalinized BMZ surrounding vessels (these stain + for PAS and are made up of Type IV collagen)
What is erythromelalgia?
Red, painful/burning edematous, condition w/ a sensation of heat that usually occurs on the distal extremities (often the lower extremities/feet)
What are most cases of erythromelalgia associated with?
Small fiber neuropathy
What things usually make erythromelalgia worse?
Activity, heat
What things make erythromelalgia feel better?
Cooling, classically pt’s describe putting their feet in cold water
What is type I erythromelalgia?
Occurs in the setting of thrombocythemia and can lead to ischemic necrosis
What is type III erythromelalgia?
Occurs w/ an underlying condition (that is NOT thrombocythemia)
What is the difference between livedo retiularis and livido racemosa?
Reticularis: can be bad if secondary, or benign if primary
Racemosa: Always bad (always secondary)
Morphologically: livedo reticularis has complete rings, whereas racemosa has broken and asymmetric rings
What testing must ALL patients with livedo racemosa get?
Always work up for anti-phospholipid syndrome
What is the physiology underlying livedoid vasculopathy?
The rings are created by congested venous Plexi
Leds to decrease blood flow and decrease blood draining out of skin –> de-oxygenated blood in venous plexus
What are the major causes of livedo reticularis?
Physiologic/idiopathic: arteriole vasospasm –> persistent LR of the lower extremities
Secondary to vasospasm: can be seen w/ Raynaud’s or autoimmune CTD
Secondary to vessel wall damage: medium vessel vasculitis (especially PAN, but also cryoglobulinemic vasculitis, and vasculitis from auto-immune CTD’s or calciphylaxis)
Secondary to intraluminal issues: slow blood flow within vessels from cyroglobulenemia, hypofibrinogenemia, polycythemia vera, APLS, protein C/S/anti-thrombin III deficiencies
- Also obstruction: cholesterol emboli, APLS, heparin warfarin necrosis, hyperoxaluria, and livedoid vasculopathy
- Other: amantadine, pheochromocytoma, reflex sympathetic dystrophy
Causes of livedo racemosa?
APLS and Sneddon syndrome
Where should a livedoid vasculopathy be biopsied?
Large double punch or excisional bx from the CENTER/normal-appearing skin in the center of the net-like pattern
What procedure is the most common cause of auriculotemporal nerve syndrome (Frey Syndrome)
Parotidectomy
What is auriculotemporal nerve syndrome?
Aberrant regeneration of parasympathetic fibers of auriculotemporal nerve after an injury –> unilateral flushing and or seating in the distribution of the auriculotemporal nerve w/ ingestion of certain foods (especially sour or spice foods)
What is the presentation of cholesterol emboli?
Levido reticularis> retiform purpura or gangrene in the distal extremities
Occurs post-catheterization (hours or days), thrombolytics (hrs-days) or anticoagulation (1-2 months)
Systemic manifestations of cholesterol emboli?
febrile, hypertensive, or may have altered mental status
Lab abnormalities that can be seen: eosinophilia, elevated ESR, elevated BUN/Cr
What is the shared pathophysiology of the pigmented purpuras
Inflammation of the capillaries int he skin –> hemorrage
Clinical presentation of Shamberg’s purpura?
Cayenne-pepper purpura on the lower extremities that can extend beyond that in older adults
Clinical presentation of Purpura annularis telangiectodes of Majocchi?
Annular patches with punctate petechiae on trunk and lower extremity in adolescents/young-adult women
Clinical presentation of Eczematid-like purpura of Doucas and Kapetanakis?
Scaly and eczematous petechiae and purpura in middle-aged to older men
What is the presentation of linear pigmented purpura?
A unilateral, linear eruption of yellow-brown macules, patches, and red-brown purpura; adolescents and children mostly
What is the histology of the pigmented purpura conditions?
Hemosiderin containing macrophages w/ RBC extravasation, endothelial swelling, and a perivascular lymphocytic infiltrate
Guogerot: lichenoid infiltrate
Doucas and Kapetanakis: spongiosis and parakeratosis
What is the clinical appearance of hypergammaglobulinemic purpura of Waldenstrom?
Crops of burning/stinging petechiae and /or puprua on the lower extremities, often seen in women
A/w a polyclonal gammopathy (IgG/IgA RF) and CTD (Sjogren’s)
What is levamisole in and why?
It is found in some cocaine as it is an antihelminthic agen and increases the stimulatory effects of cocaine (also increases bulk)
What is the classic presentation of levamisole-induced vasculitis?
Purpura and necrosis of the earlobes (also the nose, cheek, and extremities)
Can also have LCV-like lesions, ecchymoses, and systemic vasculitis, especially of the kidney/lung/testes
What is Sneddon syndrome?
Livedo racemosa and livedoid vasculopathy w/ labile blood pressure and CNS dz (TIA, stroke, dementia) and extracerebral thrombosis
Pathophysiology of Sneddon syndrome?
Associated with anti-phospholipid syndrome, vasculopathy or vasculocoagulopathy
Histology of Sneddon syndrome?
Endothelial inflammation; subendothelial intimal smooth muscle proliferation; partial or complete occlusion of arterioles
