Onychodystrophy as the Presenting Sign of Steal Syndrome

Abstract

A man in his 70s presented to the dermatology nail clinic with a 1-month history of worsening onychodystrophy, leukonychia, and pain in his left fifth finger. Physical examination revealed a cool hand and absent radial pulse. Ischemia was suspected, and the patient was sent to the emergency department where the diagnosis of steal syndrome was made and his previously required arteriovenous fistula was ligated. This case highlights the clinical features of steal syndrome, that nail changes should be recognized as clinical features, and that urgent triage of these patients to vascular surgery is of critical importance.

Established Facts

• Steal syndrome is a complication of upper extremity arteriovenous access for hemodialysis and can present with cutaneous manifestations including erythema, ischemic ulcers, and gangrene.

Novel Insights

• Nail changes have not been previously described as a component of steal syndrome but were a critical component of the diagnosis and urgent triage of this patient and should therefore be added to the literature.

Introduction

Steal syndrome, also known as access-related hand ischemia, is a potentially limb-threatening complication of upper extremity arteriovenous access creation for hemodialysis (Fig. 1). Due to derangements in hemodynamics after arteriovenous access creation, digital perfusion is reduced in up to 80% of cases; however, few become symptomatic [1]. Presenting symptoms of steal syndrome include distal limb pain, paresthesia, weakness, and in severe cases gangrene [2]. We report a case of steal syndrome that presented as painful onychodystrophy.

Fig. 1.

Anatomy of steal syndrome. a Normal vascular anatomy of the arm. Arterial (red), superficial venous system draining into axillary vein (blue). b Vascular anatomy of the arm following brachiobasilic arteriovenous fistula creation leading to reduced distal perfusion.

Case

A 70-year-old man was triaged urgently to the dermatology nail clinic with what he stated was a 1-month history of worsening nail changes and pain in the left fifth fingernail. At the time of his referral, images of that nail were uploaded to his electronic medical record by his primary care physician. When contacted to schedule an appointment in the nail clinic, the patient asked if he could be seen as soon as possible because “this thing is going to kill me” referring to severe hand pain and nail changes. The patient's significant concern was heard and, despite the seemingly chronic appearance of his nail changes, he was urgently seen in the nail clinic due to concern for malignancy or infection.

On examination in the nail clinic, the patient's left fifth fingernail was thickened and white-yellow-brown in color (Fig. 2). In addition, fingernails of the left second, third, and fourth digits exhibited proximal leukonychia (Fig. 3). His left hand was cool, swollen, erythematous, and exquisitely tender with pain rated as 10/10. A left radial pulse was not appreciated. Importantly, the patient previously required dialysis via a left brachiobasilic arteriovenous fistula prior to renal transplantation due to complications of type II diabetes mellitus. This combination of signs and symptoms raised concern for ischemia. The patient was sent urgently to the emergency department for further evaluation. In the emergency department, vascular surgery confirmed the diagnosis of steal syndrome, and the patient urgently underwent ligation of his fistula. Postoperatively, he continued to have pain and ultimately developed fourth and fifth digit contractures and distal fifth-digit gangrene (Fig. 4).

Fig. 2.

Left fifth finger at presentation to dermatology nail clinic: left fifth finger with nail bed hyperkeratosis and leukonychia. Nail clippings were subsequently resulted as negative for fungi on PAS stain.

Fig. 3.

Left hand at presentation to dermatology nail clinic: The hand as well as the third, fourth, and fifth fingers were edematous. Second, third, and fourth fingernails had proximal leukonychia. Fifth finger with onychodystrophy, nail bed hyperkeratosis, and leukonychia. The left hand was also swollen and erythematous in comparison to the right hand.

Fig. 4.

Left fifth digit ulceration: distal left fifth digit ulceration and gangrene 1 week following fistula ligation.

Discussion

This case adds to the literature the fact that onychodystrophy is among the presenting signs of steal syndrome. In addition to the nail changes described above, cutaneous manifestations of steal syndrome include erythema, ischemic ulcers, and gangrene which are important for dermatologists to recognize as they may be the physicians to which patients with steal syndrome present [3]. When steal syndrome is suspected, urgent assessment by vascular surgery is critical in order to expedite any necessary revascularization and prevent loss of digit(s), hand, and/or function.

We also present this case to add to the literature a dramatic presentation of onychodystrophy with white-yellow-brown discoloration of the nail which appeared to be due to nail bed hyperkeratosis as a direct consequence of digit hypoxia. Hyperkeratosis and pincer deformities have previously been described in patients with arteriovenous access, thought to be due to tissue hypoxia of the nail beds due to microvascular changes [4, 5]. Nail thickening, hyperkeratosis, leukonychia, ischemic ulcers, and melanonychia have also been described in necrotic carpal tunnel syndrome due to autonomic dysfunction leading to digital ischemia, and these changes have been reversed with surgical treatment of carpal tunnel syndrome [6]. Notably, our patient's leukonychia involved only the hand affected by steal syndrome and occurred acutely with the onset of pain a month prior to presentation. Additionally, our patient experienced concurrent leukonychia of fingers 2, 3, and 4 of the left hand that temporally aligned with the onset of pain due to steal syndrome and hypoxemia of the nail bed.

Leukonychia has previously been reported as a sign of digit hypoxia. In 1997, Hutchinson and Amin [7] reported a case of a mountain climber with bands of leukonychia that corresponded with the amount of time that he was hypoxic at high altitude. As leukonychia arises from alterations in the nail matrix and grows out over the period of months, we speculate that our patient was experiencing subclinical hand ischemia for months prior to presentation [8].

Last, our case emphasizes the critical importance of in person examination when symptoms of a nail disorder are out of proportion to physical findings as viewed on submitted images. Dermatologist review of photos of this patient's left fifth fingernail in the setting of severe and disproportionate nail pain resulted in an expedited in-person evaluation where a complete examination could be performed of the entire hand and the combined features of steal syndrome could be identified. Onychodystrophy is a presenting sign of digit ischemia and dermatologists have a critical role in diagnosing and appropriately triaging these patients.

Statement of Ethics

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

The authors have no sources of funding to declare.

Author Contributions

Colleen M. Morken: drafted/edited the manuscript. Sarah Mortimer: case idea and drafted/edited the manuscript. Richard Denney: edited the manuscript. Molly A. Hinshaw: case idea and drafted/edited the manuscript.