Chapter 27 - Neutrophilic Dermatoses

Question 1

What types of cells are considered “granulocytes”?

Answer 1

Neutrophils, eosinophils, and basophils

Question 2

How long do neutrophils circulate in the bloodstream before migrating into tissues? How long do they survive in peripheral tissues?

Answer 2

• Neutrophils circulate in the peripheral blood for 3-12 hours
• Neutrophils survive in the peripheral blood for 2-3 days

Question 3

What percentage of patients with Sweet’s syndrome have an internal malignancy?

Answer 3

20%

Question 4

What malignancy is most closely associated with vesiculobullous Sweet’s syndrome?

Answer 4

Myelogenous leukemia

Question 5

True or false: if left untreated, Sweet’s syndrome usually regresses spontaneously.

Answer 5

True; typically within 5-12 weeks, although recurrence occurs in 30% of patients

Question 6

What are the four major clinical forms of pyoderma gangrenous?

Answer 6

Ulcerative, bullous, pustular, and superficial granulomatous (vegetative)

Question 7

What percentage of patients with pyoderma gangrenosum have an underlying systemic disease?

Answer 7

50%

Question 8

What type of monoclonal gammopathy is seen in 15% of patients with pyoderma gangrenosum?

Answer 8

IgA

Question 9

What does PAPA syndrome stand for?

Answer 9

Pyogenic arthritis, pyoderma gangrenosum, and acne

Question 10

Sweet’s syndrome is also known as:

Answer 10

Acute febrile neutrophilic dermatosis

Question 11

What does MAGIC stand for in MAGIC syndrome?

Answer 11

Mouth And Genital Ulcers with Inflamed Cartilage; basically Behcet’s disease plus relapsing polychondritis

Question 12

True or false: isotretinoin can be used to treat the severe acne associated with SAPHO syndrome.

Answer 12

True; although close monitoring is required since occasionally isotretinoin can flare SAPHO

Question 13

What does SAPHO stand for?

Answer 13

Synovitis, Acne, Pustolosis, Hyperostosis, and osteitis

Question 14

What are the most common sites for bony and articular involvement in SAPHO syndrome?

Answer 14

The anterior chest wall (sternum, clavicle, and ribs) and other axial skeleton sites

Question 15

Describe the stages of myeloid maturation of neutrophils.

Answer 15

Myeloblast, promyelocyte, myelocyte, metamyelocyte, band, segmented neutrophil. No longer able to divide beyond myelocyte stage.

Question 16

List the steps of neutrophil migration out of the vasculature.

Answer 16

Margination, rolling, activation and tight adhesion

Question 17

What are some of the activators of neutrophils?

Answer 17

Chemoattractants, selectin ligands, immune complexes, activated complement

Question 18

What on the neutrophil is expressed and adheres to what on endothelial cells?

Answer 18

Beta 2 integrins (leukocyte function associated antigen 1/LFA-1, macrophage antigen /Mac-1) on neutrophils; intercellular adhesion molecule 1/ICAM-1 on endothelial cells

Question 19

What does neutrophilic activation result in?

Answer 19

Degranulation, lysosomal enzyme secretion, oxidative burst, arachidonic acid metabolite production, cytokine secretion, modulation of leukocyte adhesion molecules

Question 20

What systemic conditions are associated with Sweet’s syndrome?

Answer 20

Infections, malignancies (AML), IBD, autoimmune disorders, drugs, pregnancy. 50% of patients may have idiopathic disease

Question 21

What type of variant of Sweet’s syndrome is most frequently associated with acute myelogenous leukemia?

Answer 21

Vesiculobullous variant

Question 22

What type of infections are most commonly associated with Sweet’s?

Answer 22

URTI with Streptococcus spp., GI infection with Yersinia

Question 23

What are the major criteria of Sweet’s? Hint: there are 2

Answer 23

1) Abrupt onset of usual skin findings; 2) consistent histology. 2 major + 2 minor needed for diagnosis

Question 24

What are the minor criteria of Sweet’s? Hint: there are 4

Answer 24

1) Preceded by infection, vaccine, accompanied by associated malignancy or inflammatory disorder or drug exposure or pregnancy; 2) fever, constitutional signs and symptoms; 3) leukocytosis; 4) good response to steroids. 2 major + 2 minor needed for diagnosis

Question 25

What are the 4 clinical forms of pyoderma gangrenosum?

Answer 25

Ulcerative, bullous, pustular, superficial granulomatous

Question 26

What % of patients show signs of pathergy with their pyoderma gangrenosum?

Answer 26

20-30%

Question 27

What is PAPA syndrome?

Answer 27

Autosomal dominant disorder, CD2 binding protein 1 mutation causes abnormal inflammatory response; stands for pyogenic sterile arthritis, PG, acne

Question 28

What is the triad of Behcet’s?

Answer 28

Iritis, mucocutaneous ulcers, genital ulcers

Question 29

What is MAGIC syndrome?

Answer 29

Features of both Behcet’s and relapsing polychondritis; stand for mouth and gential ulcers with inflamed cartilage

Question 30

What are the neurologic manifestations of Behcet’s?

Answer 30

Acute meningoencephalitis, CN palsies, brainstem lesions, pyramidal/extrapyramidal signs

Question 31

What is the major criterion for the diagnosis of Behcet’s?

Answer 31

Recurrent oral ulceration; need at least 1 major + 2 minor

Question 32

What are the minor criteria of Behcet’s? Hint: there are 4

Answer 32

1) Recurrent genital ulceration; 2) eye lesions; 3) cutaneous lesions; 4) positive pathergy test; need at least 1 major + 2 minor

Question 33

What are the usual sites of osteoarticular lesions in SAPHO?

Answer 33

Sternum, clavicles, ribs, axial skeleton