Hypereosinophilic vasculitis: A case report

Abstract

Introduction: The Revised International Chapel Hill Consensus Conference 2012 subdivides vasculitides based on combinations of features that separate different forms of vasculitis into definable categories. Hypereosinophilic vasculitis with sparing of the respiratory tract and renal system is a rare presentation that is yet to be described in the Revised International Chapel Hill Consensus Conference 2012 report that addresses nomenclature of vasculitides. This is a condition that involves a vascular injury due to either a primary eosinophilic vasculitis or an underlying connective tissue disease and it predisposes patients to a prothrombotic state.

Patient concerns: A 39-year-old patient presented with left hand digital ischemia, preceded by Raynaud phenomenon, and vasculitic rash. For 3 months, he was having digital ischemia affecting the left 2nd and 3rd digits in the form of pallor and gangrenous discoloration with a preceding history of a pinpoint pruritic rash affecting his lower limbs that extended to involve the trunk and upper limbs over a short period of time and responded to only a tapering dose of oral steroids. Examination revealed a delayed capillary refill in all left-hand digits and a weak left radial pulse but no bruit. The rest of his peripheral vascular examination was unremarkable.

Diagnosis: Investigations revealed an absolute eosinophilic count of 4.34 K/μL and erythrocyte sedimentation rate of 44 mm/h. A magnetic resonance angiogram showed a beaded appearance of the left ulnar artery distally and the radial artery branches in the left hand and subsequently was diagnosed with hypereosinophilic vasculitis.

Interventions: He was started on oral prednisone of 1 mg/kg daily orally tapering done as well as azathioprine for maintenance.

Outcomes: Two weeks postdischarge, the patient was seen in the outpatient department where his ischemic symptoms improved, and his skin rash healed. Noticed improvement in his splinter hemorrhages was also detected. He continued to do well on 2 years follow-up CONCLUSION:: This case reflects the importance of frequent reevaluation for vasculitic diseases criteria and nomenclature. Hypereosinophilic vasculitis with absent respiratory and renal involvement is a rare presentation with scarce evidence to guide treatment.

Figure 1

Ischemic changes in the form of unilateral pallor of the left hand in addition to multiple splinter hemorrhages and digital infarcts.

Figure 2

Splinter hemorrhage on the 2nd through the 5th left digits and distal ulceration on the tip of left 3rd and 4th digits.

Figure 3

Scattered pinpoint healing vasculitic rash over the lower limbs.

Figure 4

Beaded appearance of the left ulnar artery and the radial artery branches in the left hand on magnetic resonance angiography (MRA).

Figure 5

Approach to the diagnosis of Raynaud phenomenon adapted from Wigley FM. Raynaud phenomenon. New England Journal of Medicine 2002 26;347(13):1001–8.

Figure 6

Diagnostic approach in a patient presenting with cutaneous vasculitis adapted Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, Scocco GL, Pujol RM. Cutaneous vasculitis: a diagnostic approach. Clinical and Experimental Rheumatology 2003 1;21(6; SUPP/32):S85–8.

Figure 7

Diagnostic criteria for hypereosinophilic syndrome.