Idiopathic orbital inflammatory syndrome: a dermatological perspective

Priyanka Sharma; Jasleen Kaur; Rakesh Kumar Bansal; Gurvinder P Thami

doi:10.4103/ejdv.ejdv_40_18

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CASE REPORT

Year : 2020 | Volume : 40 | Issue : 1 | Page : 59-61

Idiopathic orbital inflammatory syndrome: a dermatological perspective

Priyanka Sharma¹, Jasleen Kaur¹, Rakesh Kumar Bansal², Gurvinder P Thami¹
¹ Department of Dermatology, Government Medical College and Hospital, Chandigarh, Punjab, India
² Department of Ophthalmology, Government Medical College and Hospital, Chandigarh, Punjab, India

Date of Submission	18-Dec-2018
Date of Acceptance	10-Jun-2019
Date of Web Publication	6-Jan-2020

Correspondence Address:
Priyanka Sharma
Department of Dermatology, Government Medical College & Hospital, Sector 32, Chandigarh 160030, Punjab
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ejdv.ejdv_40_18

Abstract

Idiopathic orbital inflammatory syndrome, formerly known as orbital pseudotumor, is a rare benign condition with myriad clinical presentations including periorbital edema, pain, conjunctival chemosis, diplopia, extraocular muscle restriction, and decreased vision. It is a relatively lesser known clinical entity from a dermatological perspective and may present as recurrent periorbital edema as a sole manifestation as in this case, and thus can potentially be mistaken for angioedema. The objective of this case report is to emphasize on the wide range of clinical manifestations and the need for awareness of this entity and thus avoiding its misdiagnosis.

Keywords: angioedema, idiopathic orbital inflammatory syndrome, orbital pseudotumor, recurrent periorbital edema

How to cite this article:
Sharma P, Kaur J, Bansal RK, Thami GP. Idiopathic orbital inflammatory syndrome: a dermatological perspective. Egypt J Dermatol Venerol 2020;40:59-61

How to cite this URL:
Sharma P, Kaur J, Bansal RK, Thami GP. Idiopathic orbital inflammatory syndrome: a dermatological perspective. Egypt J Dermatol Venerol [serial online] 2020 [cited 2021 Apr 17];40:59-61. Available from: http://www.ejdv.eg.net/text.asp?2020/40/1/59/275185

Introduction

Idiopathic orbital inflammatory syndrome (IOIS), formerly known as orbital pseudotumor, is a rare benign condition without identifiable local or systemic cause. It has been reported in all ethnic groups, sexes and most frequently affects young to middle-aged adults [1].

It may develop acutely, subacutely, or insidiously as a diffuse or as a focal inflammatory process affecting any orbital structure and rarely with extraorbital extension [2]. It classically affects one eye and may present as periorbital edema, pain, conjunctival chemosis, diplopia, proptosis, extraocular muscle restriction, or decreased vision [2],[3]. IOIS is a diagnosis of exclusion and is often diagnosed on the basis of response to systemic corticosteroids [1].

Rarely, IOIS may present with periorbital edema as its sole manifestation [4],[5],[6]. In such cases, there is a possibility of confusion with angioedema, both clinically and by its responsiveness to corticosteroids [6]. Here, we describe a patient of IOIS with unilateral periorbital edema as the only symptom, presenting in the dermatology outpatient department of a tertiary care hospital.

Case report

A 51-year-old woman presented with recurrent episodes of swelling over her right eyelid which was associated with mild pain for the last 2 years. She denied antecedent trauma, redness of eyes, restriction of eye movements, diplopia, diminished vision, headache, or fever. There were no complaints of swelling over the other eye or lips, wheals, difficulty in breathing, choking sensation, or change of voice. She denied history of cough and chest pain. There were no precipitating events such as drugs, food, stress, or exercise. She had received short courses of oral corticosteroids with which the swelling used to subside but was followed by recurrence after 2–3 weeks of stopping the treatment.

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Clinical examination showed periorbital edema with mild tenderness over the right side, prominently over the upper eyelid ([Figure 1]). There was absence of conjunctival congestion, restriction of eye movements, and proptosis. Visual acuity was 6/9 in both eyes. Slit-lamp examination showed unremarkable anterior and posterior segments of the eye.

Figure 1 Periorbital edema right side, predominantly over the upper eyelid.

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The complete blood count, erythrocyte sedimentation rate, liver, renal and thyroid function tests, and C4 and C1 esterase inhibitor levels were within normal limits. Chest radiograph was normal. Noncontrast computerized tomography of the orbit showed enlargement of right superior rectus in a tubular configuration with paraseptal thickening indicating orbital myositis ([Figure 2]). Rest of the structures including extraocular muscles, optic nerves, Tenon’s capsule, and the left orbit were unremarkable. On the basis of the above finding, diagnosis of IOIS was made and the patient was given oral prednisolone 60 mg. With the treatment, her swelling and pain subsided completely by 4 weeks ([Figure 3]).

Figure 2 Noncontrast computerized tomography orbit showing right side superior rectus muscle enlargement in a tubular configuration (black arrow) and paraseptal thickening (white arrow).

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Figure 3 Complete resolution of swelling at 4 weeks of follow-up.

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Discussion

IOIS is a non-neoplastic idiopathic inflammatory process which may involve any orbital tissue such as the lacrimal glands, extraocular muscles, orbital fat, sclera, and the optic nerve [1]. It is often classified on the basis of the structure involved in the orbit such as idiopathic dacryoadenitis, orbital myositis, optic perineuritis, tendonitis, and episcleritis [2]. Depending on the anatomic target, degree of inflammation and fibrosis, it may present with a wide range of inflammatory manifestations including a palpable mass, eyelid edema, eye pain, redness, diminished ocular motility, and decreased visual acuity [3]. It is a diagnosis of exclusion; therefore, a detailed evaluation should be done to rule out other systemic or ocular diseases presenting with orbital inflammation or mass like thyroid ophthalmopathy, sarcoidosis, orbital cellulitis, vasculitis, and orbital tumors [6],[7]. IOIS, when presenting as just periorbital swelling with minimal pain and ocular signs/ symptoms, may mimic angioedema [4],[5],[6]. However, the classical unilateral distribution, absence of wheals and other mucosal swellings, slower response to oral steroids, and prolonged duration of IOIS aid in differentiating it from angioedema [6].

Orbital imaging is a useful tool for the characterization and localization of inflammatory process within the orbit. The radiological findings of IOIS are diffuse orbital mass, extraocular muscle and optic nerve enlargement, uveoscleral thickening, enhancement of Tenon’s space and proptosis based on the site and extent of inflammation [8]. Noncontrast computerized tomography orbit in our case showed enlargement of superior rectus muscle in a tubular configuration. Isolated extraocular muscle enlargement with sparing of other orbital tissues is characteristically seen in idiopathic orbital myositis which is a subgroup of IOIS [9]. This radiological feature is useful to differentiate IOIS from Graves’ ophthalmopathy, as the latter is usually bilateral and involves orbital fat and extraocular muscle with tendon sparing giving a spindle-shaped configuration of enlarged muscles [7]. There is no standard histopathological classification of IOIS. Mombaerts et al. [2] have described various histopathological variants based on the nature of cellular infiltrate, vascular changes, stromal component, and the orbital structures involved ([Table 1]). IOIS usually shows chronic inflammatory infiltrate with a predominance of T-helper cells. Less commonly, granulomatous inflammation or eosinophil-dominant infiltrate may be seen [10],[11]. The stromal changes in IOIS may range from edema to extensive sclerosis. Some authors have considered the sclerosing variant of IOIS as a distinct immune-mediated clinicopathological entity similar to retroperitoneal fibrosis [12]. The vascular changes may vary from perivascular lymphocytic infiltrate and endothelial proliferation to frank vasculitis [2]. Systemic corticosteroids are the mainstay of therapy in IOIS and yield a dramatic improvement in all symptoms and findings. However, relapses are common during the course of the disease after the withdrawal of the treatment, thus prolonged therapy with gradual tapering is required [7].

Table 1 Histopathological classification of idiopathic orbital inflammatory syndrome (by Mombaerts et al. [2])

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This case highlights the wide range of clinical manifestations of IOIS and its potential for misinterpretation as angioedema when it presents as periorbital swelling only with minimal ocular symptoms and signs, and thus, the need for awareness of this ophthalmological entity among the dermatologists.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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