“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 levofloxacinMid-May 2006 – small errythematous bump develops on hand, topical antiseptic appliedEarly June 2006 – large ulcer present, hospitalized at OSH – started on vancLate 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 % RApleasant, NAD, well appearingPERRL, EOMI, sclera clearOP pink and moist, no exudative lesionssuppleno cervical, supraclavicular, axillary LADRRR, with 3/6 systolic murmur loudest at LUSBCTA bilat no c/wsoft, nontender, normoactive bs, no masses/organomegalyno CVA tendernessno 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, punched-out ulceration (? biopsy site), and violaceous wound edges, desquamative wound edges. L antecubital with pustule. No tenderness, warmth, or induration.

Labs

1434.5

10129

13

0.9 36.18.6

12.2277

MCV 108MCHC 37

Diff – 6.1 – 1.4 – 0.7 – 0.2 – 0

101

Ca 9.3Mg 2.2Phos 4.2B12 318Folate 4.7ACE 7ANA negativeDS DNA negative

ESR 41CRP 5.2SPEP – decrease in gamma globulinsTotal Protein 6.9Monoclonal 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.  Thedifferential 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

UnknownInitial hypotheses of immune complex vasculitis, T-cell activation, or altered neutrophil function were not confirmed with experimental dataCurrent 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 UpToDateAvailable 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 lesionsEye involvement with conjuntivitis, episcleritis, iridocyclitis1/3rd of pts have arthralgias, myalgia, arthritis – asymmetric, nonerosive, mostly knees and wristsInternal 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 findingsElevated ESRElevated CRPPeripheral neutrophilia with 70% band formsDecreased hemoglobinSlight increase in alk phos, AST, GGT+ANCA rarely

Proposed Diagnostic Criteria

MajorAbrupt onset of typical cutaneous lesions

Histopathology consistent with Sweet’s

MinorAntecedent 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 seriesPrednisone is standard treatmentOther options include

NSAIDSDapsonePotassium IodideColchicineDoxycyclineClofazzimine

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 SyndromeCase Reports

Reviews

Differential Diagnosis

Drug Therapy