Other vascular disorders

Question 1

What are the two main types of telangiectasia?

Answer 1

Primary and Secondary

Question 2

What are the 6 main primary types of telangiectasia?

Answer 2

Spider, Hereditary benign, angioma serpiginosum, unilateral nevoid telangiectasia, generalized essential telangiectasia, cutaneous collagenous vasculopathy

Question 3

What are the main types of secondary telangiectasia?

Answer 3

-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)

Question 4

Genoderms associated with telangiectasia?

Answer 4

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

Question 5

What is angioma serpiginosum?

Answer 5

Usually occurs in women <20 y/o

Pinpoint punctate blanching red-purple petechiae in clusters/patches in a serpiginous pattern typically on one extremity

Question 6

What is unilateral nevoid telangiectasia?

Answer 6

Telangiectasias in the trigeminal/upper cervical dermatomes and can be in Blashko’s lines

Can be related to increased estrogen

Question 7

What is generalized essential telangiectasia?

Answer 7

Typically in adult women

Starts on the lower extremities and spreads to other larger areas

Question 8

What is cutaneous collagenous vasculopathy?

Answer 8

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

Question 9

Histology of cutaneous collagenous vasculopathy?

Answer 9

Ectatic dermal vessels w/ thick hyalinized BMZ surrounding vessels (these stain + for PAS and are made up of Type IV collagen)

Question 10

What is erythromelalgia?

Answer 10

Red, painful/burning edematous, condition w/ a sensation of heat that usually occurs on the distal extremities (often the lower extremities/feet)

Question 11

What are most cases of erythromelalgia associated with?

Answer 11

Small fiber neuropathy

Question 12

What things usually make erythromelalgia worse?

Answer 12

Activity, heat

Question 13

What things make erythromelalgia feel better?

Answer 13

Cooling, classically pt’s describe putting their feet in cold water

Question 14

What is type I erythromelalgia?

Answer 14

Occurs in the setting of thrombocythemia and can lead to ischemic necrosis

Question 15

What is type III erythromelalgia?

Answer 15

Occurs w/ an underlying condition (that is NOT thrombocythemia)

Question 16

What is the difference between livedo retiularis and livido racemosa?

Answer 16

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

Question 17

What testing must ALL patients with livedo racemosa get?

Answer 17

Always work up for anti-phospholipid syndrome

Question 18

What is the physiology underlying livedoid vasculopathy?

Answer 18

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

Question 19

What are the major causes of livedo reticularis?

Answer 19

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

Question 20

Causes of livedo racemosa?

Answer 20

APLS and Sneddon syndrome

Question 21

Where should a livedoid vasculopathy be biopsied?

Answer 21

Large double punch or excisional bx from the CENTER/normal-appearing skin in the center of the net-like pattern

Question 22

What procedure is the most common cause of auriculotemporal nerve syndrome (Frey Syndrome)

Answer 22

Parotidectomy

Question 23

What is auriculotemporal nerve syndrome?

Answer 23

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)

Question 24

What is the presentation of cholesterol emboli?

Answer 24

Levido reticularis> retiform purpura or gangrene in the distal extremities

Occurs post-catheterization (hours or days), thrombolytics (hrs-days) or anticoagulation (1-2 months)

Question 25

Systemic manifestations of cholesterol emboli?

Answer 25

febrile, hypertensive, or may have altered mental status

Lab abnormalities that can be seen: eosinophilia, elevated ESR, elevated BUN/Cr

Question 26

What is the shared pathophysiology of the pigmented purpuras

Answer 26

Inflammation of the capillaries int he skin –> hemorrage

Question 27

Clinical presentation of Shamberg’s purpura?

Answer 27

Cayenne-pepper purpura on the lower extremities that can extend beyond that in older adults

Question 28

Clinical presentation of Purpura annularis telangiectodes of Majocchi?

Answer 28

Annular patches with punctate petechiae on trunk and lower extremity in adolescents/young-adult women

Question 29

Clinical presentation of Eczematid-like purpura of Doucas and Kapetanakis?

Answer 29

Scaly and eczematous petechiae and purpura in middle-aged to older men

Question 30

What is the presentation of linear pigmented purpura?

Answer 30

A unilateral, linear eruption of yellow-brown macules, patches, and red-brown purpura; adolescents and children mostly

Question 31

What is the histology of the pigmented purpura conditions?

Answer 31

Hemosiderin containing macrophages w/ RBC extravasation, endothelial swelling, and a perivascular lymphocytic infiltrate

Guogerot: lichenoid infiltrate

Doucas and Kapetanakis: spongiosis and parakeratosis

Question 32

What is the clinical appearance of hypergammaglobulinemic purpura of Waldenstrom?

Answer 32

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)

Question 33

What is levamisole in and why?

Answer 33

It is found in some cocaine as it is an antihelminthic agen and increases the stimulatory effects of cocaine (also increases bulk)

Question 34

What is the classic presentation of levamisole-induced vasculitis?

Answer 34

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

Question 35

What is Sneddon syndrome?

Answer 35

Livedo racemosa and livedoid vasculopathy w/ labile blood pressure and CNS dz (TIA, stroke, dementia) and extracerebral thrombosis

Question 36

Pathophysiology of Sneddon syndrome?

Answer 36

Associated with anti-phospholipid syndrome, vasculopathy or vasculocoagulopathy

Question 37

Histology of Sneddon syndrome?

Answer 37

Endothelial inflammation; subendothelial intimal smooth muscle proliferation; partial or complete occlusion of arterioles