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“It’s my hand, doc…”
ID Case Conference
10-3-07
Gretchen Shaughnessy, MD
CC: L hand wound
52 year old gentleman with HTN who presents in June 07 for a
second opinion of a left hand wound.
He states that since October 2006 he’s had recurrent ulcers on
his left hand. He thinks he’s had about 6 since October, they
usually last about a week and clear up with antibiotics. In
the past he’s been on amicycline, augmentin, nad
levofloxacin. The lesions appear to be getting more severe
and in April of 2007 he was hospitalized for 4 days, given IV
vancomycin, and then discharged on levofloxacin.
In mid-May 2007 he noticed a small errythematous bump on his
left 2nd MTP joint. He began scratching it and using a topical
antiseptic but it progressed and opened with pus and grew in
diameter.
His PCP prescribed levofloxacin and referred him to
dermatology. The dermatologist added minocycline but the
wound continued to progress, turning black at the edges and
growing in size.
HPI (cont)
He was admitted to Nash Regional in June 2007
where a biopsy of the lesion was inconclusive.
Blood cultures, wound cultures, biopsy cultures for
fungal, bacterial, and AFB were all negative.
Left hand film revealed no evidence of osteomyelitis.
HIV was negative. He was started on Vancomycin,
Primaxin, and Biaxin during his course and was
ultimately d/c'd on biaxin and doxycycline with a
preliminary diagnosis of Mycobacterium marinum
because of occupational exposure.
Given the pt's overall minimal improvement, he
presented to UNC ER for a second opinion.
HPI Timeline
October 2006 – started getting ulcers on L hand,
each lasts a few weeks, seems to resolve in a few
weeks.
April 2006 – ulcer is larger than usual, doesn’t
respond to outpatient antibiotics, patient is
hospitalized and improves with vanc, d/ced on
levofloxacin
Mid-May 2006 – small errythematous bump
develops on hand, topical antiseptic applied
Early June 2006 – large ulcer present, hospitalized
at OSH – started on vanc
Late June 2006 – goes to UNC ED for second
opinion.
PMH
Back Surgery many years ago – no
hardware left in place
HTN – well controlled on meds
Recurrent hand ulcers as described in
HPI
NKDA
Social History
Lives in Rocky Mount with wife.
Works in pressure washing and typically wears
gloves but occasionally tears in them.
He denies recent travel, denies livestock and
exoctic animal exposure; they do have 3 dogs in
the home. Denies any fists fights/hand trauma.
Denies known interaction with military or middle
east travelers.
Denies tobacco, alcohol, or drugs.
No significant family history.
Medications
Vasotec 20mg bid
Ziac 10mg daily
Norvasc 10mg daily
ABX (started 6/17)
Biaxin 500mg bid
Doxycyline 100mg bid
ROS
The pt denies fevers, chills but since
October pt endorses 27lb wt loss and
intermittent left arm tingling.
He denies palpable LAD, chest pain,
shortness of breath. He denies
abdominal pain, n/v/d, bloody stools.
He denies change in UOP, dysuria,
hematuria.
Physical Exam
BP 201 / 97-->161/83. HR: 71. RR: 19. Temp: 37.1 oral. O2
saturation: 98 % RA
pleasant, NAD, well appearing
PERRL, EOMI, sclera clear
OP pink and moist, no exudative lesions
supple
no cervical, supraclavicular, axillary LAD
RRR, with 3/6 systolic murmur loudest at LUSB
CTA bilat no c/w
soft, nontender, normoactive bs, no masses/organomegaly
no CVA tenderness
no LEE, 2+ radial/dp pulses. Left hand wound described in
skin exam, mildly tender to palpation.
A&Ox3, CN II-XII grossly intact.
Skin exam
4cm x 4cm ulcer at 2nd MTP joint. Central
yellowish granulation, dark purple edges.
Dry, mildly erythematous. Nontender.
Derm consult description:
3 cm wound with 1.5 cm central, punchedout ulceration (? biopsy site), and
violaceous wound edges, desquamative
wound edges.
L antecubital with pustule.
No tenderness, warmth, or induration.
Labs
143 101 13
4.5 29 0.9
101
Ca 9.3
Mg 2.2
Phos 4.2
B12 318
Folate 4.7
ACE 7
ANA negative
DS DNA negative
8.6
12.2
277
36.1
Diff – 6.1 – 1.4 – 0.7 – 0.2 – 0
MCV 108
MCHC 37
ESR 41
CRP 5.2
SPEP – decrease in gamma globulins
Total Protein 6.9
Monoclonal component – IgG kappa
but conc too low to quantify
Discussion
Pathology
Skin, left hand, biopsy
- Neutrophilic dermatosis with overlying
pseudoepitheliomatous hyperplasia
The AFB, GMS, and Gram stains are
negative for infectious organisms. The
differential diagnosis includes Sweet's
syndrome.
After reviewing the slides from the OSH,
dermatology gave this patient the diagnosis
of Sweet’s Syndrome
Sweet’s Syndrome
One of the neutrophilic dermatoses
Pathologic dx - Intense epidermal
and/or dermal inflammatory infiltrate
composed mostly of neutrophils with
no evidence of infection or vasculitis
First described in 1964 by Dr. Sweet
in eight female patients
Pathogenesis
Unknown
Initial hypotheses of immune complex
vasculitis, T-cell activation, or altered
neutrophil function were not confirmed with
experimental data
Current hypothesis is cytokine
dysregulation
Sweet's syndrome has been described as
a complication of granulocyte-colony
stimulating factor (G-CSF) therapy,
supporting this theory
Associated Conditions
20-25% of patients with Sweet's
syndrome have an associated
malignancy
Most hematopoietic – MDS or AML
~15% of malignancy associated Sweet’s
syndrome are assocated with solid
tumors
genitourinary, breast, and gastrointestinal
tract most common
Associated Conditions (cont)
Bacterial infections — vaccinations, streptococcus,
mycobacterium, Yersinia, typhus, salmonella
Viral infections — cytomegalovirus, chronic active hepatitis,
HIV
Drugs — lithium, furosemide, hydralazine, oral
contraceptives, minocycline, imatinib, mesylate and
tmp/smx.
Autoimmune and collagen vascular diseases — rheumatoid
arthritis, systemic lupus erythematosus, mixed connective
tissue disease, Hashimoto thyroiditis, Sjögren's syndrome,
Behcet's disease
Inflammatory bowel disease — Crohn's disease, ulcerative
colitis
Other — pregnancy, complement deficiency, subacute
necrotizing Iymphadenitis, and Poem's syndrome
Primary Features
Cutaneous eruption consisting of
erythematous papules and plaques
a dermal nonvasculitic neutrophilic
infiltration on biopsy
Fever
Peripheral neutrophilia
Dermatologic Findings
Erythematous to violaceous tender papules
which enlarge to form plaques with an
irregular pseudovesicular surface
True pustulation and blistering can occur
Plaques usu a few centimeters in diameter.
May cause pain or burning sensation, not
pruritic.
Most common areas – dorsum of hands,
face, neck, upper extremeties.
More Images
See UpToDate
Available online at UNC Health Sciences
Library [on campus only]
Systemic symptoms
Fever in 40-80%
Can be intermittent
URI or flu-like illness can precede cutaneous
lesions
Eye involvement with conjuntivitis, episcleritis,
iridocyclitis
1/3rd of pts have arthralgias, myalgia, arthritis –
asymmetric, nonerosive, mostly knees and wrists
Internal organ involvement can include
neutrophilic alveolitis, sterile osteomyelitits, ARF,
involvement of kidneys, liver, pancreas,
neurologic, and psychiatric changes.
All viseral manifestations repsond to corticosteroid
therapy.
Lab Findings
Nonspecific findings
Elevated ESR
Elevated CRP
Peripheral neutrophilia with 70% band
forms
Decreased hemoglobin
Slight increase in alk phos, AST, GGT
+ANCA rarely
Proposed Diagnostic Criteria
Major
Abrupt onset of typical cutaneous lesions
Histopathology consistent with Sweet’s
Minor
Antecedent fever of infection
Accompanying fever, arthralgias, conjunctivitis,
or underlying malignancy
Leukocytosis
Good response to steroids but no response to
antibiotics
* Two major and two minor criteria are needed for diagnosis.
Adapted from Su, WP, Liu, HN, Cutis 1986; 37:167.
Treatment
All treatment based on uncontrolled
nonrandomized case series
Prednisone is standard treatment
Other options include
NSAIDS
Dapsone
Potassium Iodide
Colchicine
Doxycycline
Clofazzimine
Our Patient’s Course
Last seen in derm clinic 8-07
Took 8 week course of dapsone, skin
lesions resolved completely
Now on a prolonged taper of dapsone
per derm clinic
No associated condition yet
diagnosed
Search PubMed
Sweet’s Syndrome
Case Reports
Reviews
Differential Diagnosis
Drug Therapy