Recurrent Cutaneous Eosinophilic Vasculitis: A Rare Entity.

Recurrent cutaneous necrotizing eosinophilic vasculitis is a rare entity described by Chen et al. It has a benign course without any systemic involvement. However, often long-term treatment with systemic steroids is required. The exact etiopathogenesis remains unknown. We report a female patient, who presented with recurrent pruritic purpuric papules and plaques affecting different body parts of long duration. Disease was well controlled with low dose systemic steroids, but invariably recurred on its withdrawal. Histology revealed the features of eosinophilic vasculitis. Subsequent detailed evaluation ruled out systemic involvement, underlying diseases, and any precipitating factors. Hence, a diagnosis of recurrent cutaneous eosinophilic vasculitis was made. Patient showed excellent response to prednisolone, and on tapering the drug, it was found that she needed a maintenance dose of 5 mg/day. We did not come across any previous reports of recurrent cutaneous eosinophilic vasculitis from India.

What was known?

Recurrent cutaneous eosinophilic vasculitis is a rare entity manifesting as pruritic papules and plaques without any systemic involvement and without any apparent cause.

Introduction

Recurrent cutaneous (necrotizing) eosinophilic vasculitis is a rare eosinophilic vasculitis of unknown etiology that runs a chronic course.[12] This benign condition remains limited to the skin manifesting as pruritic purpuric papules and plaques with angioedema.[3] It shows good response to systemic steroids; but almost always needs long-term maintenance therapy.[2] Here, we report a female patient with recurrent cutaneous eosinophilic vasculitis who required low dose prednisolone to maintain disease control.

Case Report

Forty-five-year-old female patient attended our institution with intensely pruritic, persistent red raised lesions over forearms, legs, palms, and soles of 5 years duration. Fever and constitutional symptoms were conspicuously absent. She did not give a history of bronchial asthma, allergic disorders, or any other systemic diseases. No food item or drug was found to precipitate the lesions. With the diagnosis of erythema multiforme, she was treated at a nearby hospital with prednisolone 20 mg daily to which her skin lesions responded. Tapering the dose of prednisolone to 2.5 mg always brought out recurrences; hence, she was continued on 5 mg prednisolone for the past 5 years. One month before attending our outpatient department, the patient stopped prednisolone as per the advice of her clinician in an attempt to withdraw steroids which resulted in exacerbation of skin lesions. As no control could be achieved with antihistamines and emollients, she was referred to us.

Clinical examination revealed multiple, discrete, and confluent purpuric papules and plaques distributed over forehead [Figure 1], neck, abdomen, and limbs. Some of the lesions showed an annular pattern. In addition, she had angioedema affecting lips [Figure 2]. Systemic examination revealed no abnormality.

Figure 1

Erythematous, purpuric discrete, and confluent papules and plaques on the forehead of a patient with recurrent cutaneous eosinophilic vasculitis

Figure 2

Angioedema of lips in recurrent cutaneous eosinophilic vasculitis

Complete hemogram, absolute eosinophil count, peripheral smear analysis, bleeding and clotting time, and prothrombin time with international normalized ratio, urine microscopy, and renal and liver function tests were within normal limits. Serology was negative for infections due to mycoplasma and herpes simplex, hepatitis A, B, and C, and human immunodeficiency viruses. Serology for antinuclear antibody profile and rheumatoid factor were negative. Chest radiography, electrocardiogram, and ultrasonogram of abdomen and pelvis were within normal limits. Biopsy was taken from a lesion on the right upper arm and was sent for histopathology analysis and immunofluorescence study. Histology revealed perivascular inflammatory infiltrate [Figure 3] predominantly composed of eosinophils with fibrinoid necrosis of vessel walls and extravasation of erythrocytes [Figure 4] conclusive of eosinophilic vasculitis. Immunofluorescence was negative for immunoglobulins G, A and M, and C3.

Figure 3

Skin biopsy showing perivascular inflammatory infiltrate in recurrent cutaneous eosinophilic vasculitis (H and E, ×100)

Figure 4

(a) High power view of the biopsy specimen from the skin lesions of recurrent cutaneous eosinophilic vasculitis showing the eosinophils infiltrating the walls of small dermal vessels and fibrinoid necrosis of vessel walls (H and E, ×400). (b) Another section of the same biopsy revealing eosinophils invading the vessel walls with fibrinoid necrosis and extravasation of erythrocytes (H and E, ×1000)

Serum cryoglobulins and antineutrophil cytoplasmic antibodies were negative. Serum complement level (C1q, C3, and C4) and immunoglobulin profile were within normal limits. Stool microscopy for cysts and ova was negative. Radiography of paranasal sinuses, computerized tomogram of the thorax, and nerve conduction study (peripheral neuropathy can be a manifestation of idiopathic hypereosinophilic syndrome or Churg–Strauss vasculitis) returned normal results.

Since a detailed evaluation ruled out any underlying systemic disease and since there was no systemic involvement, we arrived at a diagnosis of recurrent cutaneous eosinophilic vasculitis. She received prednisolone 30 mg/day orally. Her skin lesions resolved in 7–10 days. On tapering steroids, the patient remained symptom free up to a dose of 5 mg daily. Trying to reduce the dose to 2.5 mg precipitated a flare up; hence, she was advised to continue prednisolone at 5 mg/day. Now, after 4 months patient is well maintained on 5 mg prednisolone with no more recurrences.

Discussion

Chen et al. in 1994 described three patients who presented with pruritic purpuric papules, annular urticarial plaques, and angioedema.[1] These skin lesion showed chronic relapsing course without any systemic involvement. Histology revealed necrotizing vasculitis of dermal small vessels with prominent eosinophilic infiltration. This new eosinophil mediated syndrome was named as recurrent cutaneous eosinophilic vasculitis. It has remained a rare disease with very few cases reported worldwide.

The chronic course, the clinical picture, and the histopathology feature pointed to the diagnosis of recurrent cutaneous eosinophilic vasculitis in our patient. Peripheral blood eosinophilia, which is documented as a common finding of recurrent cutaneous eosinophilic vasculitis was conspicuously absent in our case. A similar finding was documented by Tsunemi et al.[4]

Lack of peripheral blood eosinophilia persisting for more than 6 months and absence of systemic involvement ruled out the possibility of idiopathic hypereosinophilic syndrome in our patient.

Patient giving no history of bronchial asthma, lack of peripheral blood eosinophilia, and absent extravascular granuloma on histology were against a diagnosis of Churg–Strauss vasculitis.

Eosinophilic vasculitis without any leukocytoclasia observed on biopsy performed on a 4 days old lesion in our patient excluded urticarial vasculitis. Well's syndrome was ruled out by the vasculitis histology.[4]

Erythema multiforme was unlikely in our patient since the two common precipitating infections (herpes simplex and mycoplasma) were ruled out, and since she was not on any medications prior to the disease onset. Histologically also erythema multiforme differs by featuring keratinocyte necrosis, interface dermatitis, and dermal infiltrate composed of mixed inflammatory cells.

The exact etiopathogenesis of recurrent cutaneous eosinophilic vasculitis remains unclear to this date. Abnormal eosinophil mediated-immune response against an unidentified antigen is proposed to play a role in the pathogenesis of recurrent cutaneous eosinophilic vasculitis.[5] It is postulated that interleukin-5, C4, and platelet-activating factor released by eosinophils may increase vascular permeability and induce purpuric papules and angioedema.[6]

So far no systemic associations are noted for recurrent cutaneous eosinophilic vasculitis, though a positive serology for antinuclear antibody is documented in occasional cases.[57] Li et al. reported a case of recurrent cutaneous eosinophilic vasculitis in a patient with psoriasis, but no correlation was established between the two.[6]

This rare vasculitis responds well to systemic steroids, but often requires long-term maintenance dose as observed in our patient.[2] Other therapeutic options found useful are systemic tacrolimus, indomethacin, and suplatast tosilate (a drug found useful in atopic dermatitis).[567]

We did not come across any previous reports of recurrent cutaneous eosinophilic vasculitis from India, and we found only one other case of the same that manifested without peripheral blood eosinophilia.

We report this case to highlight the existence of this rare entity and to underscore the need for long-term systemic steroid therapy.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

What is new?

We did not come across any previous reports of recurrent cutaneous eosinophilic vasculitis from India, and there is only one other instance when it has been reported without peripheral blood eosinophilia.