Understanding the Zebras of Wound Care: An Overview of Atypical Wounds

Elizabeth Ansert, DPM, MBA, MA; Anthony Tickner, DPM, FACCWS, FAPWCA, FAPWH; Donald Cohen, DPM; Weldon Murry, DPM; Samuel Gorelik, DPM


Wounds. 2022;34(5):124-134. 

In This Article


Large epidemiologic studies have shown that up to 79.7% of leg ulcers have a vascular etiology (venous, peripheral arterial disease, or mixed).[3] Approximately 20% to 23% of patients have wounds caused by other, more complex vascular etiologies.[3] Two atypical complex vasculopathies are livedoid vasculopathy and thromboangiitis obliterans.

Livedoid vasculopathy is uncommon. This distinct hyalinizing vascular disease affects only 1 in 100 000 individuals per year.[52] The pathogenesis of livedoid vasculopathy is not fully understood, but it is related to intraluminal thrombosis of the dermal microvessels, resulting in occlusion and tissue hypoxia.[53] Ulcerations caused by livedoid vasculopathy are characterized by atrophic plaques with hyperpigmented borders and telangiectatic vessels (Figure 10). The dorsal aspect of the foot and ankle are most often affected.[56] The aim of treatment modalities is to improve microcirculation, prevent infection, and provide wound care and pain control.[54] After these ulcerations heal, they form white atrophic scars.

Figure 10.

(A) Skin ulcer of the left thigh covered with thick eschar measuring 8cm x 6cm at presentation; (B) vessel with thickened wall and proliferating myofibroblastic cells and irregular demarcation, (hematoxylin and eosin stain, x40 magnification); and (C) size reduction of the ulcer (2.3cm x 1.5cm) following treatment with oral cyclosporine and intravenous prostanoids. Used with permission; Gracia-Cazaña T, de la Fuente S, Concellón M, Ara-Martín M. Lower extremity skin ulcer associated with neurofibromatosis type 1: a case report. Wounds. 2019;31(10):e65-e67.

Thromboangiitis obliterans, also known as Buerger disease, is a nonatherosclerotic, segmental, inflammatory disease that most commonly affects the small-sized to medium-sized arteries and veins of the extremities. This disease is characterized by highly cellular and inflammatory occlusive thrombus with relative sparing of the blood vessel wall.[55] It affects men more commonly than women and is associated with cigarette smoking. Thromboangiitis obliterans is most prevalent in the Mediterranean, Middle East, and Asia because of the homemade cigarettes with raw tobacco smoked in those geographic areas.[56]

Patients typically present with reports of claudication of the extremities (Figure 11), but pain during rest and ischemic ulceration may occur.[57] Erythrocyte sedimentation rate and C-reactive protein level are usually normal, and autoantibodies are typically negative.

Figure 11.

Sudden onset of dry, painful digital dry gangrene in a younger male (unknown age) who was a current smoker. The underlying etiology was thought to be Buerger disease and claudication of the digital arteries resulting from thrombi.

Smoking cessation is the mainstay of treatment, but pharmacologic intervention has shown promising results. Nicotine replacement is not recommended because of the persistence of claudication symptoms even after smoking cessation. Intravenous iloprost was compared with aspirin in 133 patients with thromboangiitis obliterans and critical limb ischemia.[58] At 28-day follow-up, 85% of the patients treated with iloprost (58/68) showed improved ulcer healing and pain relief, compared with only 17% of patients (11/65) in the aspirin group.[58]