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The case of the sassy
blistering woman
Andy J. Chien
University of Washington
Department of Medicine
Division of Dermatolo...
Introducing J.M.
• ID: 65 yo F with two week history of “blisters”
• Admitted initially to St. Joseph’s hospital, but then...
J.M.’s Past Medical History
• Pyoderma gangrenosum since 2001– flares if
prednisone < 20 mg/day. Had skin grafts in 2001 o...
J.M.’s Medication History
• Allergies/Intolerances: codeine, diazepam
• Prednisone 20 mg qd
• Azathioprine 50 mg bid
• Fur...
Physical Examination
• VS: T = 35.9o
C, P = 106, BP = 111/67, RR = 20,
SaO2 = 93% on RA
• Gen: sassy elderly female lying ...
Physical Examination
Physical Examination
Physical Examination
Physical Examination
Physical Examination
Laboratory and diagnostic
studies
• Chem 7: Na = 129, K = 3.4, Cl = 85, HCO3 = 25, BUN = 35, Cr
= 1.1, Glu = 144
• CBC: WB...
Differential diagnosis
• Acute febrile neutrophilic dermatosis
• Pyoderma gangrenosum
• Drug reaction
• Cutaneous malignan...
Histopathology
“Right palm plaque” “Left leg bulla”
Histopathology
Differential diagnosis
• Acute febrile neutrophilic dermatosis
• Pyoderma gangrenosum
• Drug reaction
• Cutaneous malignan...
Sweet’s original report in 1964
• 8 female patients between 32 and 55 seen over 15 years
• Patients were ill
• One or more...
• Martin Luther King wins the Nobel Peace Prize and
the Civil Acts Right is signed by LBJ
• The Palestine Liberation Organ...
• “I Want To Hold Your Hand” tops the charts
• The Rolling Stones begin their first tour of the US
• The mini-skirt debuts...
Sweet’s syndrome
• Fever
• Neutrophil leukocytosis of the blood
• Painful plaques/nodules on the limbs, face and neck
– 0....
Additional features of Sweet’s syndrome
• Pathergy – biopsy sites, trauma, radiation tx, sunburn
• Oral lesions – mucosal ...
Clinical settings of Sweet’s
syndrome
• “Classic” Sweet’s syndrome
• Malignancy-associated Sweet’s
Syndrome (MASS)
• Drug-...
Classic Sweet’s syndrome
• F:M ~ 4:1
• No racial preference
• Ages 30-50 (youngest reported 7 wks old)
• Idiopathic
• Infe...
Malignancy-associated
Sweet’s syndrome
• M=F
• Incidence difficult to establish (7-56%).
Cohen and Kurzrock reviewed 448 p...
Diagnostic criteria for classic Sweet’s
or MASS
Major criteria (both required)
1. Abrupt onset of painful erythematous pla...
Other
Infection
Autoimmune
IBD
Malignancies
Diseases reported in association with Sweet’s syndrome
• AML
• Myelodysplasia
...
Drug-induced Sweet’s
syndrome
• F:M = 3:1
• G-CSF is big culprit
• All-trans-RA, CBZ, hydralazine, OCPs, minocycline,
nitr...
Diagnostic criteria for drug-
induced Sweet’s syndrome
All five criteria required
1. Abrupt onset of painful plaques or no...
Clinical features in Sweet’s syndrome
Adapted from Cohen PR and Kurzrock R, Clin Dermatol (2000) 18, 265-282
Epidemiology
...
Vasculitis and the histopathology of
Sweet’s syndrome
• 28 biopsy specimens from 21 patients satisfying diagnostic
criteri...
Pathogenesis of Sweet’s syndrome
?
The non-infectious neutrophilic dermatoses
• Non-angiocentric
– Psoriasis
– Reiter’s syndrome
– Subcorneal pustular dermat...
Overlapping of the neutrophilic dermatoses
Sweet’s PG Sned-Wilk
Oral involv. ulcers ulcers -- -- ulcers
Eye involv. conjun...
Stem cell
CFU-GEMM
CFU-GM
CFU-G
myeloblast
promyelocyte
myelocyte
metamyelocyte
band
IL-3
IL-6
GM-CSF
G-CSF
IL-17
T
MP
B
N...
IgG
C3b
Phagocytosis
IL-8
C5a
LTB4
PAF
fMLP
Chemoattractants
and
chemotaxis
IL-1
IL-2
TNFα
G-CSF
GM-CSF
Neutrophil activat...
Classic chemoattractant
Gα
γ
β
GTP
PI3K
Adhesion,
chemotaxis,
ROS
PLC
IP3 DAG
Ca PKC
Adhesion,
ROS
cPLA2
AA
Ras GTP
Raf Rh...
IgG
C3b
Phagocytosis
IL-8
C5a
LTB4
PAF
fMLP
Chemoattractants
and
chemotaxis
CD11/CD18
L-selectin
Adhesion
β1 integrin
PECA...
E and P
selectin
ICAM
sialyl
Lewis X
CD11/CD18 (LAD)
1. “rolling”
PECAM
2. transmigration
β1 and β2
integrins
3. infiltrat...
IgG
C3b
Phagocytosis
IL-8
C5a
LTB4
PAF
fMLP
Chemoattractants
and
chemotaxis
CD11/CD18
L-selectin
Adhesion
β1 integrin
PECA...
IgG
C3b
Phagocytosis
Interference with
phagocytosis:
dapsone
CD11/CD18
L-selectin
Adhesion
β1 integrin
PECAM
Interference with
integrin-mediated adhesion:
-corticosteroids
-dapsone
IL-8
C5a
LTB4
PAF
fMLP
Chemoattractants
and
chemotaxis
IL-1
IL-2
TNFα
G-CSF
GM-CSF
Neutrophil activators
Interference with...
Reactive oxygen species
(NADPH oxidase)
Enzymatic destruction
-cathepsins
-azurocidins/defensins
-elastases
-lysozymes
-ph...
Scientific advances since 1964
• First beta-blocker synthesized (propranalol, 1964)
• First human heart transplant (1967)
...
Treatment of Sweet’s syndrome
• Spontaneous resolution
– 4/8 of women in Sweet’s original report
– 9 published reports fro...
Corticosteroids
• Still the treatment of choice
• Systemic:
– 0.5-1.5 mg/kg/day average from a dozen studies
– Tapered ove...
Other therapeutic agents
• Potassium Iodide (>10 reports/series)
– 300 - 950 mg/day
• Colchicine (5 reports/series)
– 1 – ...
Other therapeutic agents
• Antimicrobials
– Tetracycline class
– Antimicrobials implicated in drug-induced Sweet’s
• Sulfo...
Less exciting, but somebody tried it
• Salicylates
• Clofazimine
– 200 mg qd x 4 wks, then 100 mg qd x 4 wks
– Enhances PM...
• Derm residents: ova awaiting fertilization.
• Dank: alas, also just an unfertilized ovum.
• Kuechle: learning to walk, a...
• Iwamoto: skiing in Switzerland and miserably trying to learn
Swiss-German while speaking only Japanese.
• Berg: having a...
• Sybert: starting tenth grade, fending off would-be suitors, and
falling in love with Mr. Zaccaro, the social studies tea...
Acknowledgments
• Phil Fleckman
• Bob Underwood
Selected References
• Burg, ND and Pillinger, MH (2001) “The neutrophil: function and regulation in
innate and humoral imm...
Sweet's syndrome.ppt
Sweet's syndrome.ppt
Sweet's syndrome.ppt
Sweet's syndrome.ppt
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Transcript of "Sweet's syndrome.ppt"

  1. 1. The case of the sassy blistering woman Andy J. Chien University of Washington Department of Medicine Division of Dermatology
  2. 2. Introducing J.M. • ID: 65 yo F with two week history of “blisters” • Admitted initially to St. Joseph’s hospital, but then transferred to UWMC for further evaluation when KOH prep of blister fluid from heel showed hyphal elements • Blisters began on each heel, then progressed up the legs, and then involved the hands. Started as red spots, progressed to blisters and painful bumps. • 4-day history of eruption on the face • Blisters began 3 days after patient started furosemide for ankle edema • Cold-like symptoms occurred shortly before first blisters appeared • Review of systems unremarkable. No F/C/NS/wt loss. Excellent appetite. No arthralgias/myalgias. Organ-based ROS all negative.
  3. 3. J.M.’s Past Medical History • Pyoderma gangrenosum since 2001– flares if prednisone < 20 mg/day. Had skin grafts in 2001 on legs. • “monoclonal gammopathy” of some type with recent bone marrow biopsy • HTN • Hyperlipidemia • Osteoporosis • Fibrocystic breast disease • Active 45 pack-year smoking history • S/P hysterectomy • H/O colonization with MRSA
  4. 4. J.M.’s Medication History • Allergies/Intolerances: codeine, diazepam • Prednisone 20 mg qd • Azathioprine 50 mg bid • Furosemide 40 mg qd (started 17 days ago) • Simvastatin • Potassium chloride • Premarin • ASA • Alendronate • Alprazolam • Celexa • MVI
  5. 5. Physical Examination • VS: T = 35.9o C, P = 106, BP = 111/67, RR = 20, SaO2 = 93% on RA • Gen: sassy elderly female lying in bed in NAD, interactions appropriate. • HEENT: anicteric, OP clear. • Neck: no masses/LAD. Normal JVP. • Abdomen: No masses, no HSM. • LN: No cervical, axillary or inguinal LAD. • Ext: No clubbing. Trace bilateral LE pitting edema. Good peripheral pulses. • Neuro: alert and oriented with no focal deficits noted.
  6. 6. Physical Examination
  7. 7. Physical Examination
  8. 8. Physical Examination
  9. 9. Physical Examination
  10. 10. Physical Examination
  11. 11. Laboratory and diagnostic studies • Chem 7: Na = 129, K = 3.4, Cl = 85, HCO3 = 25, BUN = 35, Cr = 1.1, Glu = 144 • CBC: WBC = 19.6 with neutrophils = 18.03 (92%), Hct = 34 (MCV 91) • Ca/Mg/Phos, LFTs, INR unremarkable • UA and urine culture normal • Blood cultures with no growth • Direct exam of blister fluid on left leg x 3 showed 2-4+ WBCs, no organisms. Bacterial, fungal and viral cultures negative. Viral FA negative. • CXR: emphysematous changes with no masses or infiltrates • EKG: NSR with RAD, LV hypertrophy by voltage criteria
  12. 12. Differential diagnosis • Acute febrile neutrophilic dermatosis • Pyoderma gangrenosum • Drug reaction • Cutaneous malignancy • Infection secondary to immunosuppresion
  13. 13. Histopathology “Right palm plaque” “Left leg bulla”
  14. 14. Histopathology
  15. 15. Differential diagnosis • Acute febrile neutrophilic dermatosis • Pyoderma gangrenosum • Drug reaction • Cutaneous malignancy • Infection secondary to immunosuppresion
  16. 16. Sweet’s original report in 1964 • 8 female patients between 32 and 55 seen over 15 years • Patients were ill • One or more asymmetric erythmatous patches progressing to raised painful plaques (0.5-4 cm) • Some plaques gave the illusion of a “multiocular blister”, some had small central pustules • Active phase was 1-2 months depending on treatment with steroids • No scarring • No other physical exam abnormalities • Infection workups unrevealing • Five of eight had one or more recurrences • Neutrophilic infiltrates seen on pathology • Diff dx: erythema multiforme, erythema elevatum diutinum, pyoderma gangrenosum, erythema nodosum, bromide/iodide eruption • Postulated a steroid responsive reactive process
  17. 17. • Martin Luther King wins the Nobel Peace Prize and the Civil Acts Right is signed by LBJ • The Palestine Liberation Organization is founded • Khruschev is deposed in Moscow and replaced by Brezhnev • Conflict in Vietnam escalates as US warships are attacked in the Gulf of Tonkin • Nelson Mandela given life sentence (but will be freed in 1990 and elected president of S. Africa in 1994) • Japan unveils the “bullet train” (130 mph top speed)
  18. 18. • “I Want To Hold Your Hand” tops the charts • The Rolling Stones begin their first tour of the US • The mini-skirt debuts and is a hit worldwide • Sidney Poitier wins an Oscar for his performance in “Lilies of the Field” (Best picture? “Tom Jones”) • Caldecott Medal awarded to Maurice Sendak for “Where the Wild Things Are” • Cassius Clay wins the world heavyweight title • Summer Olympics held in Tokyo, winter Olympics held in Innsbruck • St. Louis beats the Yankees (4-3) for the World Series title (Bob Gibson is the MVP)
  19. 19. Sweet’s syndrome • Fever • Neutrophil leukocytosis of the blood • Painful plaques/nodules on the limbs, face and neck – 0.5 – 12 cm in size, enlarge over days-weeks – red-purple in color – single or multiple, often asymmetric – can be bullous/vesicular in appearance • Histologically dense infiltrates with mature PMNs – usually infiltrate in superficial dermis, +/- eos and lymphs – can be diffuse or perivascular – papillary edema common – epidermis usually spared; occasional spongiosis, exocytosis, subcorneal pustule • No evidence of infection • Responds to corticosteroids
  20. 20. Additional features of Sweet’s syndrome • Pathergy – biopsy sites, trauma, radiation tx, sunburn • Oral lesions – mucosal ulcers, swollen tongue • Eyes – conjunctivitis, scleritis, iritis, glaucoma, hemorrhage • Bones – arthralgias, aseptic osteomyelitis, aseptic osteitis, neutrophils in synovial aspirates • CNS – aseptic meningitis, neuro sxs, central pareses, pysch sxs • Liver – lab abnormalities • Kidney – lab abnormalities • Intestines – lower GI sxs • Lungs – lesions on bronch, abnormalities on CXR • Musculoskeletal – myalgias
  21. 21. Clinical settings of Sweet’s syndrome • “Classic” Sweet’s syndrome • Malignancy-associated Sweet’s Syndrome (MASS) • Drug-induced Sweet’s syndrome
  22. 22. Classic Sweet’s syndrome • F:M ~ 4:1 • No racial preference • Ages 30-50 (youngest reported 7 wks old) • Idiopathic • Infections (Strep - URI, Yersinia -GI) • Pregnancy • Inflammatory bowel disease
  23. 23. Malignancy-associated Sweet’s syndrome • M=F • Incidence difficult to establish (7-56%). Cohen and Kurzrock reviewed 448 patients with Sweet’s; 20% had evidence of a solid or hematologic tumor • Hematologic malignancies: AML most common • Solid tumors: GU, breasts, GI tract
  24. 24. Diagnostic criteria for classic Sweet’s or MASS Major criteria (both required) 1. Abrupt onset of painful erythematous plaques or nodules 2. Dense neutrophilic infiltrate (without evidence of LCV?) Minor criteria (two of four required) 1. Fever > 38o C 2. History of associated illness or malignancy 3. Response to treatment with potassium iodide or steroids 4. Three of four lab abnormalities: - WBC > 8000 - Neutrophils > 70% - ESR > 20 - Positive CRP Cohen PR and Kurzrock R, Clin Dermatol (2000) 18, 265-282 Su WP and Liu HN, Cutis (1986) 37, 167-174
  25. 25. Other Infection Autoimmune IBD Malignancies Diseases reported in association with Sweet’s syndrome • AML • Myelodysplasia • CML • Multiple myeloma • Solid tumors (GU, GI and breast) • Crohn’s • Ulcerative colitis • Rheumatoid arthritis • SLE • Thyroid disease (Grave’s, Hashimoto’s) • Post-infection (bacterial, fungal, parasitic) • Behcet’s • Sarcoidosis • Erythema nodosum • Pregnancy
  26. 26. Drug-induced Sweet’s syndrome • F:M = 3:1 • G-CSF is big culprit • All-trans-RA, CBZ, hydralazine, OCPs, minocycline, nitrofurantoin, TMP-SMX • M.W. Cobb: JAAD 1989, “Furosemide-induced eruption simulating Sweet’s syndrome” – Clinical and histological features of Sweet’s syndrome, but no fever
  27. 27. Diagnostic criteria for drug- induced Sweet’s syndrome All five criteria required 1. Abrupt onset of painful plaques or nodules 2. Dense neutrophilic infiltrate (without evidence of LCV?) 3. Fever > 38o C 4. Temporal relation between drug onset and clinical onset OR temporally related recurrence after re- challenge with drug 5. Temporal relation between withdrawal of drug OR resolution with systemic corticosteroids Adapted from Cohen PR and Kurzrock R, Clin Dermatol (2000) 18, 265-282 and Walker DC and Cohen PR, J Am Acad Dermatol (1996) 34, 918-923
  28. 28. Clinical features in Sweet’s syndrome Adapted from Cohen PR and Kurzrock R, Clin Dermatol (2000) 18, 265-282 Epidemiology -Female 80 50 (33/66) 59 71 -Prior URI/GI infxn. 75-90 16 (11/69) 20 21 -Recurrence 30 69 (37/54) 41 67 Symptoms -Fever > 38C 80-90 88 (50/57) 79 100 -Musculosk. involv. 12-56 26 (18/69) 34 21 -Ocular involv. 17-72 7 (5/69) 15 21 Lesion locations -Upper extrem. 80 89 (58/65) 97 71 -Head/neck 50 63 (41/65) 52 43 -Trunk/back 30 42 (27/65) 33 50 -Lower extrem. Infreq. 49 (32/65) 48 36 -Oral mucous memb. 2 12 (8/65) 3 7 Lab findings -Neutrophilia 80 47 (29/62) 60 38 -Elev. ESR 90 100 (25/25) 95 100 -Anemia Infreq. 68 (40/49) 50 50 -Abnormal renal fxn. 11-50 15 (10/69) 7 0 Classic Heme Solid Drug- Malignancy Tumor Induced
  29. 29. Vasculitis and the histopathology of Sweet’s syndrome • 28 biopsy specimens from 21 patients satisfying diagnostic criteria for Sweet’s syndrome (classic or MASS) • Vasculitis: fibrinoid necrosis and intramural inflammation • Vasculitis as defined found in 6/28 specimens (21%) • Evidence of RBC extravasation and intramural inflammatory cells seen in 21/28 specimens (79%) • Specimens with vasculitis examined by IF for presence of IgG, IgM, IgA and C3 with appropriate controls • No immune complex deposition detected with Sweet’s samples • Proposal: vasculitis can be seen as an epiphenomenon in Sweet’s syndrome Malone JC et al., Arch Dermatol (2002) 138, 345-349.
  30. 30. Pathogenesis of Sweet’s syndrome ?
  31. 31. The non-infectious neutrophilic dermatoses • Non-angiocentric – Psoriasis – Reiter’s syndrome – Subcorneal pustular dermatosis (Sneddon-Wilkinson) – Acne fulminans – Neutrophilic eccrine hidradenitis – Blastomycosis-like pyoderma (pyoderma vegetans) • Angiocentric – Vessel wall destruction (leukocytoclastic vasculitis, polyarteritis nodosa) – No vessel wall destruction • Acute febrile neutrophilic dermatosis (Sweet’s) • Pyoderma gangrenosum • Behcet’s disease • Pustular vasculitis • Bowel-associated dermatosis-arthritis syndrome (BADAS) • Rheumatoid neutrophilic dermatosis • Pyostomatitis vegetans • Pustular eruption of ulcerative colitis • Neutrophilic dermatosis of the dorsal hand • Familial Mediterranean fever
  32. 32. Overlapping of the neutrophilic dermatoses Sweet’s PG Sned-Wilk Oral involv. ulcers ulcers -- -- ulcers Eye involv. conjunctivitis keratitis, -- keratitis, uveitis, Iritis, scleritis vasculitis vasculitis vasculitis Associations heme malig. heme malig. heme malig. heme malig. heme malig. IBD IBD IBD IBD e. nodosum e. nodosum e. nodosum sarcoid sarcoid thyroid dz. thyroid dz. thyroid dz. RA RA RA RA SLE SLE SLE Behcet’s Behcet’s PG PG Sweet’s, PG Clin. Appear. papules ulcers pustules papules ulcers, nodules nodules Sweet’s-like, PG-like EED Behcet’s Pathergy yes yes -- -- yes Joint involv. arthritis arthritis arthritis arthritis arthritis Histopath superficial ulcer subcorneal sup/mid dermis LCV dermis pustules LCV
  33. 33. Stem cell CFU-GEMM CFU-GM CFU-G myeloblast promyelocyte myelocyte metamyelocyte band IL-3 IL-6 GM-CSF G-CSF IL-17 T MP B N E KC all-trans retinoic acid
  34. 34. IgG C3b Phagocytosis IL-8 C5a LTB4 PAF fMLP Chemoattractants and chemotaxis IL-1 IL-2 TNFα G-CSF GM-CSF Neutrophil activators
  35. 35. Classic chemoattractant Gα γ β GTP PI3K Adhesion, chemotaxis, ROS PLC IP3 DAG Ca PKC Adhesion, ROS cPLA2 AA Ras GTP Raf Rho Rac Membrane ruffling, ROS MEK MAPK Adhesion, transcription, aggregationAdapted from Burg ND and Pillinger MH (2001) Clin Immunol 99, 7-17
  36. 36. IgG C3b Phagocytosis IL-8 C5a LTB4 PAF fMLP Chemoattractants and chemotaxis CD11/CD18 L-selectin Adhesion β1 integrin PECAM IL-1 IL-2 TNFα G-CSF GM-CSF Neutrophil activators 12-HETE Keratinocytes
  37. 37. E and P selectin ICAM sialyl Lewis X CD11/CD18 (LAD) 1. “rolling” PECAM 2. transmigration β1 and β2 integrins 3. infiltration Adapted from Von Den Driesch (2000) Clin Dermatol 18, 233-244
  38. 38. IgG C3b Phagocytosis IL-8 C5a LTB4 PAF fMLP Chemoattractants and chemotaxis CD11/CD18 L-selectin Adhesion β1 integrin PECAM Reactive oxygen species (NADPH oxidase) Enzymatic destruction -cathepsins -azurocidins/defensins -elastases -lysozymes -phospholipases -glycosidases IL-1 IL-2 TNFα G-CSF GM-CSF Neutrophil activators
  39. 39. IgG C3b Phagocytosis Interference with phagocytosis: dapsone
  40. 40. CD11/CD18 L-selectin Adhesion β1 integrin PECAM Interference with integrin-mediated adhesion: -corticosteroids -dapsone
  41. 41. IL-8 C5a LTB4 PAF fMLP Chemoattractants and chemotaxis IL-1 IL-2 TNFα G-CSF GM-CSF Neutrophil activators Interference with Chemotaxis: - potassium iodide - colchicine - tetracycline Inhibtion of TNFα: thalidomide
  42. 42. Reactive oxygen species (NADPH oxidase) Enzymatic destruction -cathepsins -azurocidins/defensins -elastases -lysozymes -phospholipases -glycosidases Inhibits secretion of neutrophil lysosome: - colchicine Scavenge ROS: -dapsone Inhibit generation Of ROS: -thalidomide -tetracycline -erythromycin -metronidazole -sulfasalazine (also induces PMN apoptosis)
  43. 43. Scientific advances since 1964 • First beta-blocker synthesized (propranalol, 1964) • First human heart transplant (1967) • Restriction endonucleases discovered (1968) • Interleukin-1 is identified (1972) • Acyclovir is developed (1972) • Computer assisted tomography invented (1973) • DNA sequencing is developed (1977) • In vitro fertilization is accomplished (1978) • Polymerase chain reaction developed (1983) • HIV identified (1984) • Dolly is cloned (1996) • Human genome working draft is completed (2000) • Fleckman declares pathogenesis of Sweet’s still unclear (2002)
  44. 44. Treatment of Sweet’s syndrome • Spontaneous resolution – 4/8 of women in Sweet’s original report – 9 published reports from 1964-1997 – 33/208 (16%) with spontaneous resolution • Cure underlying condition – infection, cancer • Discontinue offending medications • No randomized control trials exist for documenting the effectiveness of pharmacologic interventions
  45. 45. Corticosteroids • Still the treatment of choice • Systemic: – 0.5-1.5 mg/kg/day average from a dozen studies – Tapered over 1 – 12 weeks – Pulsed methylprednisolone 250 – 1000 g/day x 3 days • Topical: – Fluocinonide, diflorasone – Primary or adjuvant therapy • Intralesional: – TAC at 3 – 10 mg/cc • ACTH
  46. 46. Other therapeutic agents • Potassium Iodide (>10 reports/series) – 300 - 950 mg/day • Colchicine (5 reports/series) – 1 – 1.5 mg qd/qod • Cyclosporine (5 case reports/series) – 2 – 10 mg/kg/day • NSAIDS (8 reports/series) – Indomethacin, 150 – 750 mg qd/qod – Not always useful
  47. 47. Other therapeutic agents • Antimicrobials – Tetracycline class – Antimicrobials implicated in drug-induced Sweet’s • Sulfones (9 reports/series) – Dapsone 100 – 200 mg qod – Sulfapyridine also used for IBD • Infliximab – Crohn’s disease
  48. 48. Less exciting, but somebody tried it • Salicylates • Clofazimine – 200 mg qd x 4 wks, then 100 mg qd x 4 wks – Enhances PMN phagocytic activity – Red-gray skin pigmentation, drug-related icthyosis • Etretinate – Good response in patient with agnogenic myeloid metaplasia • IFN-alpha – Systemic and IL in patient with CML • Danazol • Pentoxifylline • Antihistamines • Cytotoxic agents – AZA, MTX, cyclophosphamide, chlorambucil
  49. 49. • Derm residents: ova awaiting fertilization. • Dank: alas, also just an unfertilized ovum. • Kuechle: learning to walk, awaiting first ballet lesson. • Sidbury: learning to walk, soon to be forced by older brother to play basketball left-handed. • Hornung: in a Southern California grocery store, asking her mother when she was going to turn four. • Kern: cynical New York second-grader. • Kirby: starting high school in Seattle. • Milner: dreaming of Bonaparte and waiting to start his residency in dermatology at the UW
  50. 50. • Iwamoto: skiing in Switzerland and miserably trying to learn Swiss-German while speaking only Japanese. • Berg: having a smashing time as a three year-old in London, not yet planning on a career in medicine. • Lantz: happy 4-year old Republican in Vancouver, Washington • Argenyi: going through sixth grade in Budapest, Hungary, insisting that he was a “very well-behaved boy”. • Colven: pimping fellow pre-schoolers on “Mother Goose Nursery Rhymes” reading assignment.
  51. 51. • Sybert: starting tenth grade, fending off would-be suitors, and falling in love with Mr. Zaccaro, the social studies teacher. • Still a virgin… • Fleckman: demonstrating with Mario Savio (1942-1996) and the Free Speech Movement at Berkeley, birthplace of the “sit-in”. During 1964, a skeptical young Phil develops a distrust of news media coverage. • Olerud: awarded first of two team MVP awards, one year before being awarded “John Olerud Award” and First Team All- American. Courting future Mrs. Olerud. • Raugi: about to start senior year of high school with abundant hair on both head and legs, doing research for $1.25/hour at Palo Alto, trying to figure out where Vietnam was, developing a passion for golf, disdaining Beatlemania, and spending countless hours watching… • …BULLFIGHTING FROM TIJUANA?!?
  52. 52. Acknowledgments • Phil Fleckman • Bob Underwood
  53. 53. Selected References • Burg, ND and Pillinger, MH (2001) “The neutrophil: function and regulation in innate and humoral immunity” Clin Immunol 99, 7-17. • Callen, JP (2002) “Neutrophilic dermatoses” Dermatol Clin 20, 409-419. • Cohen, PR and Kurzrock, R (2000) “Sweet’s syndrome: a neutrophilic dermatoses classically associated with acute onset and fever” Clin in Dermatol 18, 265-282. • Cohen, PR and Kurzrock, R (2002) “Sweet’s syndrome: A review of current treatment options” Am J Clin Dermatol 3, 117-31. • Cronstein et al. (1992) “A mechanism for the antiinflammatory effects of corticosteroids: the glucocorticoid receptor regulates leukocyte adhesion to endothelial cells and expression of ELAM-1 and ICAM-1” Proc Natl Acad Sci USA 89, 9991-9995. • Miyachi, Y (2000) “Pharmacologic modulations of neutrophils” Clin in Dermatol 18, 369-373. • Schroder, J-M (2000) “Chemoattractants as mediators of neutrophilic tissue recruitment” Clin in Dermatol 18, 245-263. • Sweet, RD (1964) “An acute febrile neutrophilic dermatosis” Br J Dermatol 79, 351-356. • Von Den Driesch, P (2000) “Polymorphonuclears: Structure, function and mechanisms of involvement in skin disease” Clin in Dermatol 18, 233-44.
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