Case Report |
Corresponding author: Lyubomir Dourmishev ( l_dourmishev@mail.bg ) © 2022 Lyubomir Dourmishev, Kremena Nikolova , Lyubka Miteva.
This is an open access article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Citation:
Dourmishev L, Nikolova K, Miteva L (2022) Cutaneous manifestations of aortoiliac occlusive disease: two cases and review of the literature. Folia Medica 64(4): 682-687. https://doi.org/10.3897/folmed.64.e64221
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Aortoiliac occlusive disease (AIOD) is non-inflammatory obstructive vasculopathy commonly affecting patients with advanced atherosclerosis, diabetes mellitus, or elevated cholesterol levels, and subjects with other risk factors such as cigarette smoking.
Two Caucasian patients (a 55-year-old woman and a 56-year-old man), with ulcerous cutaneous lesions of AIOD are reported. In both cases, medical history comprises initial lower limb claudication, multiple painful ulcers along the legs and absence of superficial femoral artery pulse. Severe obstruction of both infrarenal aorta and iliac arteries on the left side was demonstrated by contrast angiography and Doppler ultrasonography.
The evolution of the disease showed some characteristic findings, including pyoderma gangrenosum-like ulcerations as the initial cutaneous manifestation of AIOD, multiple painful ulcers along the lower extremities, and aorto-iliac occlusive disease due to atherosclerosis.
Early diagnosis and surgical reconstruction of vessels in patients with AIOD improved quality of life and limb salvage rates.
aortoiliac occlusive disease, pyoderma gangrenosum-like lesions, skin ulcers
Aortoiliac occlusive disease (AIOD) is a segmental non-inflammatory obstructive vasculopathy commonly occurring in the infrarenal aorta and iliac arteries. It affects mainly patients with advanced atherosclerosis, diabetes mellitus, or hypercholesterolemia, and those with other risk factors such as cigarette smoking, age, family history, race etc.[
Cutaneous manifestation varied from nonspecific inflammatory lesions through ulcers to gangrene of low extremities and the diagnosis might be difficult.[
We present two Caucasian patients with similar medical histories in whom we suspected severe obstruction of the infrarenal aorta and iliac arteries because of the clinical and cutaneous features, which was verified by contrast angiography and Doppler ultrasonography.
A 55-year-old woman was admitted to the Department of Dermatology, Alexandrovska University Hospital in Sofia with pustular lesions on the abdomen, and painful multiple ulcers located on the postoperative inguinal scar, the buttocks, and internal part of the left thigh. Pathergy reaction was positive and the working diagnosis was pyoderma gangrenosum (PG). The patient had a history of allergic reactions to non-steroidal anti-inflammatory drugs (NSAID) and house dust mites and concomitant arterial hypertension, diabetes mellitus, and ischemic heart disease, including past myocardial infarction.
Physical examination revealed vesiculopustular skin lesion on the abdomen (Fig.
Methylprednisolone (1 mg/kg/24 h) was started for PG and the dose was reduced step-wise over the next 2 months and combined with local ozone therapy. Therapy resulted in healing of the ulcers on the inguinal fold and internal aspect of the thigh and allowed completion of the surgical treatment of AIOD. Thromboendarterectomy of the femoral artery and bypass of the left aorto-femoral artery with an 8-mm prosthesis was performed in the cardio-vascular clinic. At the same time, a necrectomy of left thumb was performed. Treatment continued with pentoxifylline and acenocoumarin to complete resolution of the ulcers. The concomitant diabetes, cholesterol, and hypertension were controlled with adequate medications.
A 56-year-old male patient was admitted to our Department of Dermatology with a 3-year history of persistent swelling of both calves. He complained of formation of small ulcers followed by spontaneous re-epithelization; however, six months earlier, deep painful nonhealing ulcerations had appeared on the anterior aspect of his legs. The patient was a heavy smoker (>30 cigarettes/day). Previous treatment with diosmin and local re-epithelization agents was ineffective.
Physical examination revealed claudication after walking 15 feet and diffuse livedo reticularis, purpuric and pigmented maculae on both lower extremities. The patient’s left leg was slightly thinner than the right. Four deep ulcerations, two around the left knee, one pretibial, and one around the left medial ankle (Fig.
Routine laboratory investigations were within the normal ranges except for the slightly elevated aspartate transaminase (46 U/l) and creatine (130 mmol/l). Doppler ultrasonography measured decreased pulsations of the left posterior tibial artery (40 mm/Hg) compared with the right (110 mm/Hg) and normal blood pressure (RR 130/90 mm/Hg) of the left brachial artery. Abdominal CT revealed homogenous hepato- and splenomegaly. Angiography found occlusion of the bifurcation of the left iliac artery and an enlarged, corkscrew lineal artery. The right femoral artery, however, was normally visualised due to collateral arteries. One month later, the obstruction of the iliac artery was surgically removed. Maintenance therapy was pentoxifylline, verapamil, and acetylsalicylate with local iodine povidone dressings.
Histopathology of skin presenting acanthosis in epidermis, abscess formation and dense lymphocytic infiltration around blood vessels with extravasation of erythrocytes in dermis (H&E) ×200.
Arterial ulcers on the left leg, petechiae and reticular cyanotic erythema of the skin in in 56-year-old male.
Aortoiliac occlusive disease is large-vessel vasculopathy characterised by degenerated stenotic atheromatous obstruction, primarily affecting the infrarenal aorta and iliac arteries.[
Claudication in our patients was an alarming sign. The constellation of intermittent claudication, erectile dysfunction, and absent femoral pulses in men is termed Leriche syndrome (LS), named after the surgeon who first described the condition in 1923.[
Our first patient presented with vesiculopustular lesions on the abdomen and postoperative cicatrix compatible with PG. Pyoderma gangrenosum following surgery has been repeatedly reported.[
The evolution of the disease in our patients showed some characteristic cutaneous findings: (a) ulcerations and pustules sometimes resembling PG; (b) multiple deep and painful ulcers with callous borders and necrotic or fibrinous bottom, along the lower extremities; (c) petechial and purpuric lesions; and (d) livedo reticularis. All of these cutaneous findings were associated with angiography and Doppler ultrasonography, confirming arterial occlusion; aorto-iliac arterial reconstruction significantly promoted wound healing.
AOID should be distinguished from other low limb ischemic disorders such as peripheral arterial occlusive disease, superficial femoral artery occlusive disease, chronic lower extremity ischemia, peripheral emboli, and lower extremity atheromatous emboli syndrome.[
Collaboration between the dermatologist and vascular surgeon can shorten the diagnostic process and might be essential for ensuring optimal management of patients with aorto-iliac occlusive disease. Early determination of the diagnosis and surgical reconstruction of vessels should improve both the patient’s quality of life and limb salvage rates. Patients need to be given education on lifestyle modification and risk to prevent progression of the disease, including smoking cessation, increased physical activity, and diet modification.