Chapter 27 - Neutrophilic Dermatoses Flashcards
What types of cells are considered “granulocytes”?
Neutrophils, eosinophils, and basophils
How long do neutrophils circulate in the bloodstream before migrating into tissues? How long do they survive in peripheral tissues?
- Neutrophils circulate in the peripheral blood for 3-12 hours
- Neutrophils survive in the peripheral blood for 2-3 days
What percentage of patients with Sweet’s syndrome have an internal malignancy?
20%
What malignancy is most closely associated with vesiculobullous Sweet’s syndrome?
Myelogenous leukemia
True or false: if left untreated, Sweet’s syndrome usually regresses spontaneously.
True; typically within 5-12 weeks, although recurrence occurs in 30% of patients
What are the four major clinical forms of pyoderma gangrenous?
Ulcerative, bullous, pustular, and superficial granulomatous (vegetative)
What percentage of patients with pyoderma gangrenosum have an underlying systemic disease?
50%
What type of monoclonal gammopathy is seen in 15% of patients with pyoderma gangrenosum?
IgA
What does PAPA syndrome stand for?
Pyogenic arthritis, pyoderma gangrenosum, and acne
Sweet’s syndrome is also known as:
Acute febrile neutrophilic dermatosis
What does MAGIC stand for in MAGIC syndrome?
Mouth And Genital Ulcers with Inflamed Cartilage; basically Behcet’s disease plus relapsing polychondritis
True or false: isotretinoin can be used to treat the severe acne associated with SAPHO syndrome.
True; although close monitoring is required since occasionally isotretinoin can flare SAPHO
What does SAPHO stand for?
Synovitis, Acne, Pustolosis, Hyperostosis, and osteitis
What are the most common sites for bony and articular involvement in SAPHO syndrome?
The anterior chest wall (sternum, clavicle, and ribs) and other axial skeleton sites
Describe the stages of myeloid maturation of neutrophils.
Myeloblast, promyelocyte, myelocyte, metamyelocyte, band, segmented neutrophil. No longer able to divide beyond myelocyte stage.
List the steps of neutrophil migration out of the vasculature.
Margination, rolling, activation and tight adhesion
What are some of the activators of neutrophils?
Chemoattractants, selectin ligands, immune complexes, activated complement
What on the neutrophil is expressed and adheres to what on endothelial cells?
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
What does neutrophilic activation result in?
Degranulation, lysosomal enzyme secretion, oxidative burst, arachidonic acid metabolite production, cytokine secretion, modulation of leukocyte adhesion molecules
What systemic conditions are associated with Sweet’s syndrome?
Infections, malignancies (AML), IBD, autoimmune disorders, drugs, pregnancy. 50% of patients may have idiopathic disease
What type of variant of Sweet’s syndrome is most frequently associated with acute myelogenous leukemia?
Vesiculobullous variant
What type of infections are most commonly associated with Sweet’s?
URTI with Streptococcus spp., GI infection with Yersinia
What are the major criteria of Sweet’s? Hint: there are 2
1) Abrupt onset of usual skin findings; 2) consistent histology. 2 major + 2 minor needed for diagnosis
What are the minor criteria of Sweet’s? Hint: there are 4
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
What are the 4 clinical forms of pyoderma gangrenosum?
Ulcerative, bullous, pustular, superficial granulomatous
What % of patients show signs of pathergy with their pyoderma gangrenosum?
20-30%
What is PAPA syndrome?
Autosomal dominant disorder, CD2 binding protein 1 mutation causes abnormal inflammatory response; stands for pyogenic sterile arthritis, PG, acne
What is the triad of Behcet’s?
Iritis, mucocutaneous ulcers, genital ulcers
What is MAGIC syndrome?
Features of both Behcet’s and relapsing polychondritis; stand for mouth and gential ulcers with inflamed cartilage
What are the neurologic manifestations of Behcet’s?
Acute meningoencephalitis, CN palsies, brainstem lesions, pyramidal/extrapyramidal signs
What is the major criterion for the diagnosis of Behcet’s?
Recurrent oral ulceration; need at least 1 major + 2 minor
What are the minor criteria of Behcet’s? Hint: there are 4
1) Recurrent genital ulceration; 2) eye lesions; 3) cutaneous lesions; 4) positive pathergy test; need at least 1 major + 2 minor
What are the usual sites of osteoarticular lesions in SAPHO?
Sternum, clavicles, ribs, axial skeleton
