Digital ischemia due to Systemic Sclerosis associated to Essential Thrombocythemia: A case report

Abstract

Digital ulcers (DU) are a well-known problem in patients with systemic sclerosis. It is an underestimated complication of the disease causing pain and morbidity. Essential thrombocytosis is another cause of DU. The association of theses two diseases increases the risk of ischemic complications and impairment of hand function which are frequently observed in patients with digital ulcers. This report deals with a 68-year-old patient with rare association of Essential thrombocytosis ,Systemic sclerosis and Raynaud's phenomenon that was refractory to medical treatment of Systemic sclerosis (illoprost,calcium channel blockers) and improved with hydrea R .

Dermatology Eponyms – sign –Lexicon (W)

“…4), specific to the myeloproliferative disorders, refers to the occlusion of the microcirculation by platelets and is characterized by redness, congestion, and painful burning sensations of the extremities. Symptoms are characteristically relieved by cold or elevation of the extremity [3,[16][17][18].…”

Dermatology Eponyms – sign –Lexicon (M)

“…(Fig. 20) [62]. The term erythromelalgia, specific to the myeloproliferative disorders, refers to the occlusion of the microcirculation by platelets and is characterized by redness, congestion, and painful burning sensations of the extremities.…”

Dermatology Eponyms – sign –Lexicon (R). Part 1

“…An early sign of asymmetric gangrene or Raynaud's disease. Also known as acro-asphyxia or Dead-finger sign and sphaceloderma [8,9].…”