Raynaud's sendromlu hastada el yanığı
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Introduction Raynaud’s phenomenon (RP) is characterized by episodic digital blanching, cyanosis and rubor of the fingers and toes following exposure to cold and subsequent rewarming. Its overall incidance among the general population in the colder regions of the United States and Northern Europe is reported to be 10 to 15 % (1,2). Most patients have a mild form of the disorder, severe forms are rare. RP may exist independently (primary) or in association with an underlying disease (secondary), most commonly systemic sclerosis (3). The pathophysiologic features include vasospasm, endothelial cell changes, vessel obstructive features, and hemorrheologic factors. RP is the initial manifestation of disease in 70% of patients with systemic sclerosis, in whom it may be present for many years before the development of the connective tissue disease. Patients with primary RP need only conservative management and should be reassured that digital ischemia and loss of tissue occur extremely rarely (3). In the present case report a patient with RP is described who developed total finger necrosis following burn. Case Report A 39-year-old woman suffered deep and deep-dermal burns below 1 % TBSA, affecting the dorsal surface of 2nd, 3rd and 4th fingers of right hand and left wrist. The burn occured after the woman had fallen asleep while smoking. Her health background showed severe Raynaud’s disease for 5 years. She had smoked about 30 cigarettes a day for many years. The patients had frequent episodes of Raynaud’s disease. She was under treatment with calcium channel blockers before burn. On admission the patient was afebrile, with normal haemodynamic parameters. Laboratory tests including complete blood count, general biochemistry, coagulation screening, lipit levels, urine analysis, inflammatory markers were within normal limits. Rheumatoid factor, antinuclear factor, extractable nuclear antibodies, anticardiolipin, cryoglobulins and cold agglutinins were normal. Initial physical examination revealed that dorsal surface of 2nd, 3rd and 4th fingers of right hand were burned. There were no burn on palmar surface. Both hands with all fingers blanched on the first day. On the second day, cyanosis and rubor were shown. Ischemia and infarction were observed on the 3rd postburn day. During this period, the hands and the patient room was kept warm, calcium channel blocker, aspirin, low-molecular-weight heparin and potent vasodilatories such as Iloprost were used. The treatments were all ineffective. After six days burn necrosis was evident for three fingers and amputation was made (Figures 1). Full thickness skin graft was applied distally to the right hand after 20 days. The skin graft was not well vascularized. After debridement, split thickness skin graft was re-applied. This graft was partially vascularized (Figures 2) and the wound was healed by the secondary epithelialization (Figures 3). Discussion RP is common among patients who have systemic vasospastic disorders. Patients with RP have a higher incidence of migraine and angina (4). RP can affect many areas, including the surface of the nose, ears, tongue, nipples, fingers and toes (4). In the RP, the vascular mechanism is related to vasospasm that is triggered by cold. Repeated attacks of vasospasm lead to the release of damaging endotelial products, such as factor VIII, von Willebrand’s factor and endothelins (4). Skin burn wounds are known to cause alterations in the dermal vasculature. Vascular structures in the reticular layer and the subdermal plexus are often destroyed in the center of deep burns, while at the periphery microcirculation might be critical and can easily deteriorate as a consequence of increased interstitial pressure due to edema. It is well known that, thermal trauma causes two different types of injury in the tissue. Immediate and irreversible injury at the burn side is the first type. The second type is a delayed and reversible injury in the surrounding area. Recently several investigators have focused on this delayed and reversible injury which is also called progressive injury. Progressive dermal ischaemia may develop in the zone of stasis and consequently vascular occlusion develops, beginning from the zone of coagulation. Burns, usually with reperfusion injuries (5), cause a complex oxygen-derived free radical driven injury response, as well (6). After the impairment in physiological mechanisms for free-radical scavenging by the reduction in vascular supply (7) free radicals are allowed to cause endothelial and other cell damage, which results in further ischaemia and necrosis (8). At this point, an accompanying RP may accelerate and enhance the injury due to the burn of the deep dermal tissue. RP and finger necrosis after treatment of some malignancies such as non-hodgkin lymphoma and myeloproliferative disorders with bleomycin, vincristine and interferon have been reported (9-11). It is also well documented that some drugs such as ergot alkaloids, methysergide, beta blockers and chemotherapeutics may cause or exaggerate RP (12). Cocaine-induced RPand ischaemic finger necrosis has also been reported (13). The mechanism of cocaine induced vascular complications is multifactorial, one of them is to cause vasoconstriction and thrombosis of arteries in several tissues. Severe RP often leads to finger tip ulceration or even gangrene. The relationship between RP and high serum levels of anticentromere antibodies and digital necrosis without sclerodactyly has been reported (14). Thromboangiitis obliterans with RP has been reported (15). Treatment is often difficult and amputation rates of up to 70 % have been reported in this disease. RP and gangrene following snake envenomation (16), aerosol spray induced cold injury in a patient with RP (17). Hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and RP complicated by digital gangrene has been reported(18). Patients with RP generally respond well to conservative measures, such as keeping warm and avoiding exposure to cold. Elimination of the provoking factor is of principal importance in secondary RP due to physical or pharmacological triggers. Calcium channel blockers are the drugs of choice when these are not effective. Digital ischaemia due to RP can be reversed with intravenous infusions of iloprost or calcitonin gene related-peptide (12). In resistant cases, digital sympathectomy is an option (19). All medical treatment were found to be ineffective and an inevitable amputation was performed in our case. Either severe RP or effect of burn in the vascular pathophysiology were severe progressed in our patient. The combination of the cigarette smoking, burn and RP were supposed to have a synergistic effect on digital vasoconstriction and fingers necrosis. Its pathophysiology is thought to be multifactorial most likely caused by both vascular and burn mechanisms. As a result, RP with burn and smoking caused impairment in the vascular perfusion of the fingers. References
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