Chapter 23 - Purpura: Mechanisms and Differential Diagnosis Flashcards
How does livedo reticularis differ from retiform purpura?
By the presence of purpura (both are reticular)
List the five pigmentary purpuras.
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
What are the 5 subtypes of pigmented purpura?
Schamberg’s disease Majocchi disease Pigmented purpuric lichenoid dermatitis of Gourerot & Blum Eczematid-like purpura of Doucas & Kapetanakis Lichen aureus
Inflammation of which vascular structures is responsible for the pathogenesis of the pigmentary purpuras?
Inflammation of the capillaries; the reason why capillaritis occurs is unknown
What is the most common subtype of pigmented purpura?
Schamberg’s disease
What are the systemic findings associated with the pigmentary purpuras?
There are none (not even thrombocytopenia)
Which subtype of pigmented purpura is typicaly symptomatic?
Eczematid-like purpura of Doucas & Kapetanakis (pruritis)
Which of the five pigmentary purpuras is the most common?
Schamberg’s disease
True or False: in hypergammaglobunemic purpura of Waldenstrom, IgM RF is elevated
False - IgA and IgG RF are elevated
In what population is Schamberg’s disease most commonly seen?
In middle-aged men, although it can infrequently occur in children
What disease is most commonly associated with hypergammaglobulinemic purpura of Waldenstrom?
Sjogren’s syndrome
In what population does purpura annularis telangiectoides of Majocchi occur?
Most often it occurs in adolescents and young adults, especially in females
True or False: Garnder-Diamond syndrome is usually associated with psychiatric illness
True - Women
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.
True
What viens are envolved in Mondor’s syndrome of superficial thrombophlebitis?
Latercal thoracic vien Thoracoepigastric vien Superior epigastric vien
True or false: lichen aureus usually presents as multiple lesions on the lower extremity.
False; lichen aureus usually presents as a solitary lesion on the lower extremity
List two pigmentary purpuras with a lichenoid infiltrate.
Lichen aureus and pigmented purpuric lichenoid dermatitis of Gougerot and Blum
True or false: topical steroids are only occasionally helpful for the pigmentary purpuras.
True; treatment alternatives include UV light, vitamin C, and rutin (found in buckwheat, citrus peels, and cranberries)
Which two types of cancer is Trousseau’s sign most commonly associated with?
Pancreatic and lung cancer
How long does the aching pain associated with Mondor’s disease usually last?
Usually the aching pain subsides in 10 days, although the cord itself (which may be tender) can persist for months
What percentage of patients with Mondor’s disease have an underlying breast carcinoma?
10%
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.
Gardner-Diamond syndrome
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.
Mondor’s disease
List four causes of Mondor’s disease.
Trauma, surgery, infection, and breast cancer (10% of patient’s with Mondor’s disease have breast carcinoma)
True or false: hypergammaglobulinemic purpura of Waldenstrom is often associated with a connective tissue disease.
True; most often Sjogrens, but sometimes SLE or rheumatoid arthritis
What is the typical presentation of hypergammaglobulinemic purpura of Waldenstrom?
Recurrent crops of purpura preceded by burning and stinging
What are the 6 types of purpura?
a. Petechiae
b. Macular purpura
c. Ecchymoses
d. Palpable purpura
e. Non-inflammatory retiform purpura
f. Inflammatory retiform purpura
How is inflammatory retiform purpura different from non-inflammatory retiform purpura?
Early lesions in inflammatory retiform purpura typically exhibit prominent erythema
List some old and new anticoagulants
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
List variants of pigmented purpuric eruptions
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
What are some laboratory findings in patients with hypergammaglobulinemic purpura of Waldenstrom?
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
What systemic diseases are usually associated with hypergammaglobulinemic purpura of Waldenstrom?
Autoimmune connective tissue disease
Order of likelihood: Sjogren’s syndrome > rheumatoid arthritis, lupus erythematosus > monoclonal gammopathy > lymphoma, multiple myeloma
