Abstract
We are presenting the case of a 48 years old female diagnosed at the age of 28 with diffuse cutaneous systemic sclerosis. During the course of disease, despite the vasodilatator treatments and immunosuppresion for visceral involvement, the patient developed multiple infected digital ulcers both in the upper limbs and in the lower limbs, which complicated with wet gangrene, requiring transmetatarsal amputations. At that time, a macrovascular disease or a prothrombotic condition were excluded. Patient also developed infected nondigital lower extremity ulcers, which slowly healed after Alprostadil treatment. The evolution of our patient‘s disease demonstrate that the management of scleroderma vasculopathy represent a challange and a multidisciplinary approach is needed.
Highlights
Digital ulcers (DUs) are a major clinical problem in systemic sclerosis (SSc), occurring in ~30% of the patients each year [1]
Nondigital lower limb ulcers are less frequent in systemic sclerosis, with a prevalence estimated to 4%
There are a number of drug therapies avalaible to prevent and treat DUs [2]
Summary
Digital ulcers (DUs) are a major clinical problem in systemic sclerosis (SSc), occurring in ~30% of the patients each year [1]. Nondigital lower limb ulcers are less frequent in systemic sclerosis, with a prevalence estimated to 4%. In this case, any concomitent pathology (large vessel disease, vasculitis, coagulopathy, thromboembolism and smoking) that could be contributory should be identified [3]. There are a number of drug therapies avalaible to prevent (e.g. phosphodiesterase type-5 inhibitors and endothelin receptor-1 antagonists) and treat (e.g. intravenous iloprost) DUs [2]. The effect of these drugs on nondigital ulcers in systemic sclerosis has not been studied
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