Clinicians are keenly aware of the importance of evaluating the foot for sentinel clues to the early manifestations of diabetes, neuropathies, venous disease, arterial disease, other vascular pathologies, and infections. As wound care specialists, we should also be attentive to disease processes that may cause ulcerations and even autoamputation in the hands/feet.
There are some vascular/pathologic disease processes that predispose to chronic wounds, significantly affecting the function of the hands and feet, causing loss of function and morbidity.
Buerger Disease. The eponym “Buerger disease” refers to thromboangiitis obliterans (TAO),1 which is an inflammatory vasculopathy that is described as a segmental inflammatory disease affecting the small- and medium-sized arteries, veins, and nerves of the arms and legs.1 It manifests as ischemia and inflammation of the small vessels, intermittent claudication, segmental thrombosing vasculitis, and phlebitis. With TAO, chronic pain (rest pain) and a strong association with gangrene and chronic ulceration of the digits exist. Although the causation is not known, smoking tobacco is strongly implicated especially in males before age 40 to 45 years.1 The only effective treatment is smoking cessation1 and perhaps increasing exercise tolerance. The ulcers associated with TAO appear in the great toe and fingers and are associated with “autoamputation” of the distal gangrenous digits.1
Raynaud Disease and Raynaud Phenomenon. There are 2 types of Raynaud: (1) Raynaud disease (RD; primary) and (2) Raynaud phenomenon (RP; secondary). Raynaud disease is a primary idiopathic vasospasm usually triggered by sudden changes in ambient temperature (cold), affecting the fingers and the toes. There are varying estimates of prevalence for RD, 11% in young women and 8% in men.2 The prevalence of RD is lower in warmer climates.2 Typical color changes in the skin associated with coldness are characterized as a spectrum, from a blanching episode to cyanotic hues, which are transitory, followed by a gradual reperfusion (reactive hyperemia).3 As blood flow returns to the affected areas, usually the digits, they may appear red with symptoms of throbbing, tingling, burning, and numbness. The transient vascular changes associated with RD have been hypothesized to occur because of an exaggerated vasoconstrictive response of the digital arteries to cold temperatures, blunt trauma, or emotional stress.2 The exact etiology of the “vasospastic” episodes associated with RD is not known, and irreversible tissue injury does not occur in RD.2 Treatment strategies focus on avoiding cold exposure, wearing gloves, and avoiding smoking. Pharmacologic interventions aimed at vasodilatation have been suggested, albeit without evidence-based recommendations.2 Conflation exists about the taxonomy of the primary (benign) RD versus RP; remember that the “phenomenon” is the most serious owing to its association with other diseases and secondary complications, such as scleroderma, lupus, and other immunologic conditions. In “secondary” RP (SRP), the more salient manifestations include older patients who potentially have underlying, often serious rheumatologic and autoimmune diseases. The cardiovascular manifestations include vasospasm of the coronary arteries, as in Prinzmetal angina. In contrast to RD, the episodes of SRP are more dramatic, painful, and asymmetric and can be associated with ischemic skin lesions3 (ulcerations and auto amputations of the digits). Superficial ulceration or deep-tissue necrosis with gangrene and autoamputation is a manifestation as well. The patients are often seen by rheumatologists for connective-tissue disease (eg, arthritis and abnormal lung function).3 They also have specific autoantibodies and demonstrate microvascular disease on microscopy of nail-fold capillaries.2 Treatment strategies for SRP include a focus on the underlying cause, including vasodilators, calcium-channel blockers, and antiplatelet therapy with aspirin. Smoking cessation is a must, and supportive wound treatment also is indicated.
Diabetic autoamputation. Uncontrolled type 1 diabetes presents special considerations in wound management. Persons with type 1 diabetes suffer from a host of skin conditions, including bacterial infections, carbuncles, furuncles, and nail infections. Fungal infections in the interregional areas of the groin and genital-perianal regions are common. Vitiligo or patches of white or lighter skin appear occasionally and are thought be an autoimmune response. Painless diabetic blisters (bullous diabeticorum) may occur on the digits, and about a third of persons with type 1 diabetes have digital sclerosis, which manifests as thick, tight, waxy skin that develops on the backs of the hands. The finger joints stiffen and become difficult to move, but less often the skin on the toes is affected as well. Rarely, knees, ankles, or elbows may stiffen. Neurotrophic ulcerations or mutilating fingertip ulcer of the digits may progress to autoamputation.4,5 There remains a paucity of literature about the merits of aggressive surgical amputation versus autoamputation of the digits.5
The vasodysfunctions discussed manifest a continuum, with some similarities and distinct differences, ranging from benign to more serious consequences, such as chronic ischemic wounds. The evidence for pharmacologic therapy is a work in progress. Supportive therapy and smoking cessation are the sine qua non of rehabilitation.
1. Olin JW. Thromboangiitis obliterans (Buerger’s disease). N Engl J Med 2000; 343: 864–9.
2. Butendieck RR, Murray PM. Raynaud disease. J Hand Surg Am 2014; 39 (1): 121–4.
3. Wigley FM. Clinical practice. Raynaud’s phenomenon. N Engl J Med 2002; 347: 1001–8.
4. Dubbioso R, Nolano M, Mazzarella R, Rivellese AA, Manganelli F. Mutilating fingertip ulcers in uncontrolled type 1 diabetes mellitus. Neurol Sci 2014; 35: 123–4.
5. Fikri R, Bicknell CD, Bloomfield LM, et al. Awaiting autoamputation: a primary management strategy for toe gangrene in diabetic foot disease. Diabetes Care 2011; 34 (8): e134.