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 |