Chapter 23 - Purpura: Mechanisms and Differential Diagnosis

Question 1

How does livedo reticularis differ from retiform purpura?

Answer 1

By the presence of purpura (both are reticular)

Question 2

List the five pigmentary purpuras.

Answer 2

1) Schamberg’s disease
2) Purpura annularis telangiectoides of Majocchi
3) Pigmented purpuric lichenoid dermatitis of Gougerot and Blum
4) Eczematid-like purpura of Doucas and Kapetanakis
5) Lichen aureus

Question 3

What are the 5 subtypes of pigmented purpura?

Answer 3

Schamberg’s disease Majocchi disease Pigmented purpuric lichenoid dermatitis of Gourerot & Blum Eczematid-like purpura of Doucas & Kapetanakis Lichen aureus

Question 4

Inflammation of which vascular structures is responsible for the pathogenesis of the pigmentary purpuras?

Answer 4

Inflammation of the capillaries; the reason why capillaritis occurs is unknown

Question 5

What is the most common subtype of pigmented purpura?

Answer 5

Schamberg’s disease

Question 6

What are the systemic findings associated with the pigmentary purpuras?

Answer 6

There are none (not even thrombocytopenia)

Question 7

Which subtype of pigmented purpura is typicaly symptomatic?

Answer 7

Eczematid-like purpura of Doucas & Kapetanakis (pruritis)

Question 8

Which of the five pigmentary purpuras is the most common?

Answer 8

Schamberg’s disease

Question 9

True or False: in hypergammaglobunemic purpura of Waldenstrom, IgM RF is elevated

Answer 9

False - IgA and IgG RF are elevated

Question 10

In what population is Schamberg’s disease most commonly seen?

Answer 10

In middle-aged men, although it can infrequently occur in children

Question 11

What disease is most commonly associated with hypergammaglobulinemic purpura of Waldenstrom?

Answer 11

Sjogren’s syndrome

Question 12

In what population does purpura annularis telangiectoides of Majocchi occur?

Answer 12

Most often it occurs in adolescents and young adults, especially in females

Question 13

True or False: Garnder-Diamond syndrome is usually associated with psychiatric illness

Answer 13

True - Women

Question 14

True or false: the lesions of eczematid-like purpura of Doucas and Kapatenakis are usually pruritic, while the lesions of pigmented purpuric lichenoid dermatitis of Gougerot and Blum are usually asymptomatic.

Answer 14

True

Question 15

What viens are envolved in Mondor’s syndrome of superficial thrombophlebitis?

Answer 15

Latercal thoracic vien Thoracoepigastric vien Superior epigastric vien

Question 16

True or false: lichen aureus usually presents as multiple lesions on the lower extremity.

Answer 16

False; lichen aureus usually presents as a solitary lesion on the lower extremity

Question 17

List two pigmentary purpuras with a lichenoid infiltrate.

Answer 17

Lichen aureus and pigmented purpuric lichenoid dermatitis of Gougerot and Blum

Question 18

True or false: topical steroids are only occasionally helpful for the pigmentary purpuras.

Answer 18

True; treatment alternatives include UV light, vitamin C, and rutin (found in buckwheat, citrus peels, and cranberries)

Question 19

Which two types of cancer is Trousseau’s sign most commonly associated with?

Answer 19

Pancreatic and lung cancer

Question 20

How long does the aching pain associated with Mondor’s disease usually last?

Answer 20

Usually the aching pain subsides in 10 days, although the cord itself (which may be tender) can persist for months

Question 21

What percentage of patients with Mondor’s disease have an underlying breast carcinoma?

Answer 21

10%

Question 22

This psychogenic purpura usually affects females with an underlying psychiatric illness. It’s characterized by painful ecchymoses with odd/irregular borders at the sites of trauma.

Answer 22

Gardner-Diamond syndrome

Question 23

This condition is a superficial thrombophlebitis that affects the anterolateral thoracoabdominal wall. It presents as a painful cord-like thrombosed vein, and occurs secondary to trauma, surgery, infection, or breast cancer.

Answer 23

Mondor’s disease

Question 24

List four causes of Mondor’s disease.

Answer 24

Trauma, surgery, infection, and breast cancer (10% of patient’s with Mondor’s disease have breast carcinoma)

Question 25

True or false: hypergammaglobulinemic purpura of Waldenstrom is often associated with a connective tissue disease.

Answer 25

True; most often Sjogrens, but sometimes SLE or rheumatoid arthritis

Question 26

What is the typical presentation of hypergammaglobulinemic purpura of Waldenstrom?

Answer 26

Recurrent crops of purpura preceded by burning and stinging

Question 27

What are the 6 types of purpura?

Answer 27

a. Petechiae
b. Macular purpura
c. Ecchymoses
d. Palpable purpura
e. Non-inflammatory retiform purpura
f. Inflammatory retiform purpura

Question 28

How is inflammatory retiform purpura different from non-inflammatory retiform purpura?

Answer 28

Early lesions in inflammatory retiform purpura typically exhibit prominent erythema

Question 29

List some old and new anticoagulants

Answer 29

Old: heparin, LMWH (dalteparin, enoxaparin, tinzaparin), coumadin

New: nematode anticoagulant peptide/NAPc2, active site blocked factor VIIa, danaparoid, razaxaban, activated protein C, hirudin, argatroban, dabigatran

Question 30

List variants of pigmented purpuric eruptions

Answer 30

a. Schamberg’s disease
b. Purpura annularis telangiectoides of Majocchi
c. Pigmented purpuric lichenoid dermatitis of Gougerot & Blum
d. Eczematid-like purpura of Doucas & Kapetanakis
e. Lichen aureus

Question 31

What are some laboratory findings in patients with hypergammaglobulinemic purpura of Waldenstrom?

Answer 31

a. Polyclonal hypergammaglobulinemia
b. ↑ESR
c. Mild anemia
d. Anti-Ro/SS-A Ab
e. Anti-La/SS-B Ab
f. IgG rheumatoid factor
g. IgA rheumatoid factor
h. Not IgM rheumatoid factor

Question 32

What systemic diseases are usually associated with hypergammaglobulinemic purpura of Waldenstrom?

Answer 32

Autoimmune connective tissue disease

Order of likelihood: Sjogren’s syndrome > rheumatoid arthritis, lupus erythematosus > monoclonal gammopathy > lymphoma, multiple myeloma