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Table of Contents
CASE REPORT
Year : 2023  |  Volume : 11  |  Issue : 1  |  Page : 56-59

An interesting case of peripheral arterial disease as presentation in systemic lupus erythematosus


1 Department of General Medicine, Basaveshwara Teaching and General Hospital, M R Medical College, Gulbarga, Karnataka, India
2 S B Medical Center, Gulbarga, Karnataka, India

Date of Submission16-Jun-2021
Date of Decision02-Dec-2021
Date of Acceptance18-Dec-2021
Date of Web Publication05-Jan-2023

Correspondence Address:
Dr. Sangram Biradar
S B Medical Center, #14, Lahoti Enclave, Station Road, Gulbarga, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajim.ajim_63_21

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  Abstract 


Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease. Among the cutaneous manifestations of SLE, peripheral arterial disease and digital gangrene are considered very rare. Digital ulcers are relatively rare in SLE and never present as an initial manifestation of disease. The presence of an extremity ulcer is one of the more obvious clinical signs that can be due to ischemia (peripheral arterial disease). Digital gangrene is seen mostly at a late stage of the disease proposing that a long history of SLE is a considered risk factor. Only 0.2% of patients with SLE presented initially as digital necrosis. This is a case report of a 55-year-old male patient who presented with primarily with acute painful localized dry digital gangrene associated with bilateral lower limbs. Serology was positive for SLE. Doppler study of both the lower limbs vessels showed digital artery of second toe of both feet shows reduced flow on doppler study. Disarticulation of the toes was adviced as treatment.

Keywords: Dry gangrene, peripheral arterial disease, systemic lupus erythematosus


How to cite this article:
Kulkarni S, Biradar S. An interesting case of peripheral arterial disease as presentation in systemic lupus erythematosus. APIK J Int Med 2023;11:56-9

How to cite this URL:
Kulkarni S, Biradar S. An interesting case of peripheral arterial disease as presentation in systemic lupus erythematosus. APIK J Int Med [serial online] 2023 [cited 2023 Feb 6];11:56-9. Available from: https://www.ajim.in/text.asp?2023/11/1/56/367262




  Introduction Top


Peripheral artery disease (PAD), specifically atherosclerotic disease leading to peripheral artery obstruction, may be silent or present with a variety of symptoms and signs indicative of extremity ischemia. Atherosclerotic disease often involves the arteries providing flow to the lower extremities, referred to as lower extremity PAD. This is a case report of a 55-year-old male patient who presented with primarily with acute painful localized dry digital gangrene and ulcers associated with bilateral lower limbs and serology are positive for systemic lupus erythematosus (SLE).

SLE is a chronic autoimmune disease of unknown cause that can affect virtually any organ of the body. Immunologic abnormalities, especially the production of a number of antinuclear antibodies (ANA), are a prominent feature of the disease. Serologic findings are important in suggesting the possibility of SLE, with some antibodies (e.g., anti-double-stranded DNA [anti-dsDNA] and anti-Smith [anti-Sm]) highly associated with this condition.

Among the cutaneous manifestations of SLE, peripheral arterial disease and digital gangrene are considered to be very rare.

Digital gangrene is seen mostly at a late stage of the disease proposing that a long history of SLE is a considered risk factor. Only 0.2% of patients with SLE presented initially as digital necrosis.


  Case Report Top


A 55-year-old male patient presented with complaints of bilateral lower limb swelling and pain, discoloration of second toe on both sides, and fever for 8 days. The patient noticed multiple ulcers on all the toes associated with serous discharge and discoloration of second toe on both sides, which was painful and nonfoul-smelling. Pain was localized to bilateral lower limb, pricking type with intermittent episodes of numbness and pins and needles sensation, severe enough to cause restriction of daily activities, difficulty to walk and present even during sleep. The patient gave a history of joint pain of both knees for 2 years, aching type, with joint stiffness usually more in the early morning hours, which relieved on medication and swelling and pain of both wrist joints for 2 years. The patient is a chronic smoker, with 1 pack/day for 20 years (20 pack-years). Patient gave history of multiple trauma to the foot for which he has taken Ayurvedic treatment. There was no prior history of similar presentation.

After admission, his vitals were pulse rate = 72 bpm, BP = 110/70, and saturation of 98% at room air.

On examination, multiple ill-defined DUs over the back of all the toes, with seropurulent discharge, of 1 cm × 2 cm, with round edges, irregular margins, no foul smell, associated with blackish discoloration of second and third toes, tenderness present, movements of great toe, fourth and fifth toes are intact, no edema, no local rise in temperature, with dry, scaly shiny skin with hair loss and thickened nails.

The [Figure 1], [Figure 2], [Figure 3] shows the multiple digital ulcers.
Figure 1: This image shows gangrene of the second toe of the left foot and ill-defined ulcer with an irregular margin

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Figure 2: This image shows gangrene of the second toe on the right foot and ill-defined ulcer

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Figure 3: This image shows edema of the foot

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All the peripheral pulses such as dorsalis pedis, posterior tibial, popliteal, femoral, radial, and brachial are felt.

There was no peripheral nerve enlargement, glove, and stocking type of anesthesia. There was no hypopigmented, hypoanesthetic patches over the body. Respiratory, cardiovascular, and nervous system examination is normal. The patient had tenderness of both knee joints.

[Figure 1] shows gangrene of the second toe of the left foot and ill-defined ulcer with an irregular margin. [Figure 2] shows gangrene of the second toe on the right foot and ill-defined ulcer. [Figure 3] shows edema of the foot.

[Table 1] Investigations for complete blood picture and Autoantibodies.
Table 1: Investigations for complete blood picture and autoantibodies

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Routine investigations showed raised erythrocyte sedimentation rate of interest 120 mm/h and C-reactive protein of 16.0.

Doppler study of both the lower limbs vessels showed digital artery of second digits of both feet shows reduced flow. External iliac, common and superficial femoral, popliteal, and tibial arteries are normal in caliber flow velocity and spectral waveform in bilateral lower limbs. Dorsalis pedis vessels are normal. Doppler study of lower limbs shows subcutaneous edema of both feet.

For the evaluation for the cause of the peripheral arterial disease and gangrene, after ruling out thrombosis and embolism, serology for connective tissue disorder was done.

Serology was positive for antinuclear antibody, anti-dsDNA antibody (chemiluminescence) and rheumatoid factor, negative for anti-cyclic citrullinated peptide antibodies.

The patient was started on low-molecular heparin subcutaneously, dual antiplatelets, analgesics, antibiotics, and disarticulation of the toes.


  Discussion Top


PAD, specifically atherosclerotic disease leading to peripheral artery obstruction, may be silent or present with a variety of symptoms and signs indicative of extremity ischemia.[1] Atherosclerosis is the leading cause of PAD in patients >40 years old. Other causes include thrombosis, embolism, vasculitis, fibromuscular dysplasia, entrapment, cystic adventitial disease, and trauma. The highest prevalence of atherosclerotic PAD occurs in the sixth and seventh decades of life.

The primary sites of involvement are the abdominal aorta and iliac arteries (30% of symptomatic patients), the femoral and popliteal arteries (80%–90% of patients), and the more distal vessels, including the tibial and peroneal arteries (40%–50% of patients). Involvement of the distal vasculature is most common in elderly individuals and patients with diabetes mellitus.

In patients with severe arterial occlusive disease in whom resting blood flow cannot accommodate basal nutritional needs of the tissues, critical limb ischemia may develop. Patients complain of rest pain or a feeling of cold or numbness in the foot and toes. Frequently, these symptoms occur at night when the legs are horizontal and improve when the legs are in a dependent position. With severe ischemia, rest pain may be persistent.

This is a case report of a 55-year-old elderly male, smoker, who presented with acute painful localized dry digital gangrene and ulcers associated with bilateral lower limbs and serology are positive Despite being a common occurrence in connective tissue diseases, digital ulcer and gangrene development are only occasionally seen in patients with SLE.

In SLE, a variety of clinical manifestations such as Raynaud's phenomenon, acrocyanosis, livedo patterns, erythematous or violaceous macules, and papules or necrosis are triggered by heterogeneous pathophysiological mechanisms such as vasospasm, vasculitis, or thromboembolism. Endarteritis, although rare, is an important complication of SLE, in which vasculopathy affects arteries in the extremities. Poor perfusion leads to ischemia, with necrosis and infarction of the digits.[2]

Peripheral digital gangrene has been described as a rare manifestation of SLE. It occurs only in 1.3% of SLE patients.[3]

SLE is a chronic autoimmune disease of an unknown cause that can affect any organ in the body by producing autoantibodies and consequently inducing inflammation.[4] Although it can present by any means, the usual primary presentation of SLE includes arthritis, rash, and fever.[3] There have been limited reports regarding digital dry gangrene as an initial clinical presentation of SLE. This complication, which may lead to severe ischemic necrosis and amputation, is suggested to be the result of poor perfusion that is usually caused by vasculitis, vasospasm, thromboembolism, or atherosclerosis.[3]

The European League against Rheumatism/the American College of Rheumatology classification criteria for SLE requires the presence of a positive ANA as an entry criterion. Additive criteria consist of seven clinical (i.e., constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, and musculoskeletal) and three immunologic (i.e., antiphospholipid antibodies, complement proteins, and SLE-specific antibodies) categories, each of which are weighted from 2 to 10.

Patients are classified as having SLE with a score of 10 or more points. SLE in this patient was diagnosed as our patient had 4 out of 11 criteria and they were positive ANA test, positive anti-dsDNA test, joint tenderness, and fever.

The suggested treatment of digital gangrene includes corticosteroids, immunosuppressants, lipid-lowering agents, anticoagulation, and the patient was advised for disarticulation of the gangrenous toes.


  Conclusion Top


SLE is a chronic autoimmune disease of an unknown cause that can affect any organ in the body by producing autoantibodies and consequently inducing inflammation.[4] Peripheral digital gangrene has been described as a rare manifestation of SLE. It occurs only in 1.3% of SLE patients.[3]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gerhard-Herman MD, Gornik HL, Barrett C, Barshes NR, Corriere MA, Drachman DE, et al. 2016 AHA/ACC Guideline on the Management of Patients With Lower Extremity Peripheral Artery Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2017;135:e726. Epub 2016 Nov 13.  Back to cited text no. 1
    
2.
Shetty VB, Rao S, Krishnamurthy PN, Shenoy VU. Peripheral gangrene during infancy: A rare presentation of systemic lupus erythematosus. Arch Dis Child 2001;85:335-6.  Back to cited text no. 2
    
3.
Dubois EL, Arterberry JD. Gangrene as a manifestation of systemic lupus erythematosus. JAMA 1962;181:366-74.  Back to cited text no. 3
    
4.
Liu A, Zhang W, Tian X, Zhang X, Zhang F, Zeng X. Prevalence, risk factors and outcome of digital gangrene in 2684 lupus patients. Lupus 2009;18:1112-8.  Back to cited text no. 4
    


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