Livedo reticularis as a cutaneous manifestation of type 1 diabetes mellitus: A case report

INTRODUCTION

Livedo reticularis (LR) is characterized by a mottled, lace-like pattern of reddish-blue to violaceous, cyanotic skin discoloration, most commonly involving the extremities and occasionally the trunk.^1,2 The mottling seen in LR, which gives it the name of ‘livedo,’ is because of stagnation of deoxygenated blood in the cutaneous vasculature, which has its peculiar microanatomy.^2,3 It is postulated that blood supply to healthy skin is organized into cone or hexagon-shaped units, with each unit centered around a cutaneous arteriole at the apex.^1,4 When there is a decrease in blood flow through these arterioles or impaired venous drainage, it can manifest as LR.^1,4 In clinical settings, LR is most often associated with vasospasm or hypercoagulable states, while less commonly drugs like amantadine and endocrine disorders like diabetes mellitus (DM) may lead to the development of LR. Here, we report a rare association of LR with type 1 DM. This case adds to existing knowledge by documenting LR as a possible cutaneous manifestation of uncontrolled type 1 diabetes. It highlights the potential role of diabetic microangiopathy in the pathogenesis of LR and aims to increase clinical awareness of this uncommon presentation.

CASE REPORT

An 18-year-old girl presented to our outdoor clinic with mottled discoloration of skin over the lower extremities [Figure 1]. The patient was a known case of type 1 DM since the age of 8 years and was on subcutaneous insulin therapy for the same. The lesions began 5 months back and were persistent and progressive in nature. They involved the lower limbs bilaterally from the dorsal aspect of the foot to midcalf. The patient denied any history of fever, itching, or pain in the extremities, although the patient had complaints of occasional tingling and numbness in the lower limbs. The patient gave no history of any drug intake in the past 6 months other than her daily insulin injections. Her vitals were stable with a blood pressure of 118/74 mm Hg, pulse rate of 88 beats/min, saturation of 98% on room air, and a random blood glucose of 259 mg/dL. On examination, the lesions were reticular (lacy) with purplish discoloration and areas of pale skin in the center. The lesions were non-tender, cyanotic lesions with central areas of seemingly normal skin with no evidence of ulceration, fissuring, scaling, or dryness. Systemic examination was unremarkable. On laboratory workup, her hemoglobin was 11.6 gm/dL (reference range 12-14gm/dL), hemoglobin A1c was 11.3% (reference range <6.5%) with normal liver and kidney profile. Her coagulation profile tests, such as prothrombin time / international normalized ratio, activated partial thromboplastin time, and thrombin time, were normal; viral markers were negative. The patients thyroid function was within normal limits, along with negative rheumatoid arthritis factor, anti-nuclear antibody, and antiphospholipid antibody tests with normal calcium, ferritin, ceruloplasmin, and haptoglobin levels [Table 1]. Histopathology of the lesion was normal except for mild hyalinization and minimal inflammation. After ruling out the presence of vasculopathy of autoimmune origin, we hypothesized that it is most likely secondary LR associated with uncontrolled type 1 DM. Her insulin regimen was revised, and the patient was informed of proper diabetic diet and lifestyle modifications for better glycemic control. The lesions improved gradually with better glycemic control.

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Figure 1: Showing mottled discoloration of skin over lower extremities (livedo reticularis) in the patient. (a) anterior aspect (arrows), (b) lateral view (arrow), and (c) posterior aspect (arrow).

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DISCUSSION

LR may occur in the absence of an underlying systemic disorder, as seen in physiological, primary, and idiopathic forms, or it may be associated with various systemic diseases, in which case it is termed secondary LR.¹ Physiologic LR, also called cutis marmorata, is most commonly seen in neonates and young females, appearing on exposure to cold and resolving completely on warming.⁵ Primary LR follows a fluctuating course with appearance and resolution being independent of exposed temperature and with no underlying systemic disease.⁶ Idiopathic LR is persistent, with no systemic association, and does not resolve on warming.⁶ Secondary LR is commonly associated with hypercoagulable states, most frequently antiphospholipid antibody syndrome (APS), in which LR is postulated to be the initial clinical manifestation in ∼40% of patients with primary APS.^1,7 Other associations are haematological causes like cryoglobulinemia, multiple myeloma, polycythemia vera, essential thrombocythemia, lymphomas; vasculitis like rheumatoid vasculitis and polyarteritis nodosa; connective tissue disorders like systemic lupus erythematosus and dermatomyositis; infections like hepatitis C; endocrine diseases like hypercalcemia, hypothyroidism, and diabetes.^1,2 Management of secondary LR is directed at treating the underlying cause.^1,2 LR is a relatively uncommon and poorly studied manifestation in patients with diabetes. Diabetic autonomic neuropathy frequently leads to dysfunction of the cardiac and peripheral sympathetic nervous systems, resulting in impaired reflex activity, which may manifest as postural hypotension and peripheral arteriolar dilatation.^8,9 Additionally, sympathetic dysfunction in diabetes is associated with increased arteriovenous shunting, leading to a secondary reduction in capillary bed perfusion. Both arteriolar dilatation and enhanced arteriovenous shunting are believed to contribute to the development of LR in patients with diabetes.^8,9

The clinical, laboratory, and histopathology profile of our patient suggested that, in this case, LR was associated with uncontrolled type 1 DM and was not related to any other systemic cause. This case is unique due to the rare occurrence of LR in an adolescent with type 1 DM, a non-classical association. The major uncertainty during management was whether LR represented an early manifestation of an evolving autoimmune or hypercoagulable disorder, despite initially negative investigations. However, the lesions improved with better glycemic control, supporting the association with uncontrolled diabetes.

This article aims to bring this rare complication of diabetes to light so that it can improve our understanding of the association and complications of this disease. The key lesson for physicians is that LR in a patient with diabetes is not always due to vasculitis or thrombophilia; uncontrolled diabetes itself may be a contributing factor. Although systematic exclusion of serious secondary causes is essential, once these are ruled out, clinicians should focus on assessing glycemic control and diabetic microvascular disease.