Patient

1

2

3

4

5

6

Sex

F

F

F

M

F

F

Race

C

C

AA

C

AA

C

Age at Ulcer Onset

58

24

53

38

33

64

Ssc clinical

subtype

Limited

Diffuse

SSc/Rheumatoid Arthritis overlap

Limited

SSc/SLE

overlap

Limited

Antibodies

ANA + without titer,

ANA 1:1280 diffuse

ANA 1:1280 speckled

ANA 1:640, diffuse

ANA 1:10,240, speckled

ANA 1:640, speckled

dsDNA 13

Scl 70 6.9

CCP 165

RF 17

+RNP, +Smith, +cryoglobulins +RF

+RNA pol III

Organ

involvement

Raynaud’s, digital ulcerations,

ILD

Raynauds,

digital ulcerations,

ILD

Raynaud’s GERD,

arthritis,

skin vasculitis

Sclerodactyly

Raynaud’s, digital ulcerations,

ILD, PAH, pericardial effusion, sclerodactyly, calcinosis,

gastric dysmotility,

oral ulcers

Raynaud’s, digital ulcerations, GERD, sclerodactyly, calcinosis, sicca symptoms

Hypercoagulable

state

mildly elevated cardiolipin IgM; homozygous MTHFR mutation (C667T),

Mildly elevated anticardiolipin IgM, upper extremity

arterial embolism

None

None

None

mildly elevated protein C,

mildly elevated Factor VIII

Vascular

disease

Mild PAD

Microvascular disease only

None

None

venous hypertension

None

Hypertension

No

No

Yes

Yes

No

Yes

Diabetes

No

No

No

No

No

No

Ulcer location

Bilateral

legs and feet

Bilateral toes

R lateral ankle

L medial ankle

Bilateral lower anterior legs

Bilateral lower anterior legs

Biopsy

Epidermal ulceration with fibrosis and sclerosis. deep dermis with evidence of collagen fiber thickening and sclerosis concerning for persistent ulceration in the setting of systemic sclerosis.

-

No evidence of vasculitis, histology consistent with edge of a wound

-

Medium vessel vasculitis, neutrophilic infiltrate in dermis

Dermal sclerosis and mixed inflammation. Lymphocytic infiltrate. Decreased elastin fiber content in the areas of sclerosis. The sclerosis noted could be consistent with a prior history of scleroderma

Immunosuppressants

MMF

plaquenil

MTX, SSZ, infliximab, rituximab

None

MMF, plaquenil, prior cyclophosphamide, mycophenolic acid, prednisone (prior to development of wound)

MMF

Ulcer Treatment

Topical only

IV antibiotics, hyperbaric oxygen.

IV antibiotics. Frequent debridement. Rituximab for wound

Frequent debridements. Multiple rounds of antibiotics.

multiple rounds of antibiotics, skin graft, cyclophosphamide, rituximab.

antibiotics mycophenolic acid, pentyoxyphylline for vascular component

Topical treatments

profore wraps, topical gentamycin, unna boot, topical mupirocin

No topical treatments used

collagenase, miconazole, clotrimazole, ammonium lactate

Clobetasol, metronidazole gel, topical mupirocin, santyl, silvadine

wound vac, silicone border, kerlix, and coban, tefla dressing

medihoney, triamcinolone, vaseline gauze