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Abstract: A 39-year-old man with previously undiagnosed scleroderma was admitted to the UCSD Medical Center with bilateral, limb-threatening necrotic lower extremity ulcers extending to underlying fascia and muscle. Rather than amputate the extremities, the patient requested alternative treatment and underwent extensive tissue debridement followed by placement of an equine pericardium xenograft. Subsequent to treatment, the patient underwent weekly examinations and dressing changes without additional treatment. The patient was ambulating without assistance and with complete closure of all wounds in 10 weeks. The patient remained without wound recurrence at a recent 6-month follow-up visit.

Address correspondence to:
Gerit Mulder, DPM, MS, FAPWCA
UCSD Medical Center
200 West Arbor Drive
First Floor, Suite 4
San Diego, CA 92103
Phone: 303-733-4181
Email: gmulder@ucsd.edu

     Wounds associated with scleroderma appear to be of multifactorial etiology.^1,2 They often fail to respond to conventional treatments and continue to be a challenge for wound management clinics. It is not unusual for these wounds to progressively deteriorate, and when present on the lower extremity within this population, to contribute to limb loss.³ A case of a patient with scleroderma associated with painful, large, full-thickness ulcers, and subsequent wound management is presented.

     Systemic sclerosis (SSc) is a chronic systemic disorder of unknown etiology. It is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs most notably the lungs, gastrointestinal tract, heart, and kidneys. The early stage of the disease, associated with prominent inflammatory features, is followed by the development of widespread functional and structural alterations in multiple vascular beds and progressive visceral organ dysfunction due to fibrosis. The pathogenesis is characterized by vasculopathy, cellular and humoral immunity, and progressive visceral and vascular fibrosis in multiple organs. The vasculopathic process affects capillaries, arterioles, and even large vessels in many organs, resulting in reduced blood flow and impaired tissue oxygenation.⁴ The effect of reduced tissue oxygenation resulting from impaired perfusion, coupled with increased inflammatory cells, may rapidly lead to tissue necrosis and associated high levels of pain.

     Cutaneous manifestations include both digital ulcers that result from progressive Raynaud's phenomenon and extremities ulcers found on bony prominences such as the olecranon, malleolus, and calcaneus. These ulcers can be very painful.¹ Many factors appear to contribute to the etiology of these ulcerations. While digital tip ulcers are most commonly caused by ischemia, skin conditions due to fibrosis, contracture, and trauma may contribute to ulcers over bony prominence and joints. Vasculitis also is a major contributor in the large deep, painful, punched-out lower leg and malleolar ulcers.²

Case Report

     A 39-year-old Hispanic man was admitted to the University of California Medical Center with full-thickness ulcers that extended to underlying muscle, tendon, and fascia of the medial and lateral aspect of both ankles, the dorsum of both feet, and the digits of both feet (Figure 1).

     The patient complained of the sudden appearance of these wounds and did not understand why they had developed. He stated the wounds were associated with extreme pain and heavy drainage and had been present for approximately 6 months. The patient had not sought medical treatment prior to admission through the Medical Center Emergency Department. The patient was not on any medications at the time of admission and was unaware that he had any medical problems.