Abstract
Cutaneous lupus erythematosus (CLE) is an autoimmune skin disease that can manifest itself with a variety of skin symptoms. Periorbital erythema, a rare variant of CLE, presents challenges in terms of diagnosis and treatment. Here, we report a case of CLE presenting with periorbital erythema and edema. A 42-year-old female patient presented with complaints of erythema, edema, and scaling on the right eyelid that started four months ago. A skin biopsy was performed on the lesioned skin of the eyelid to differentiate dermatomyositis, cutaneous lupus erythematosus, sarcoidosis, lupus vulgaris, and cutaneous lymphoma. Histopathological examination revealed focal hyperkeratosis and parakeratosis on the surface of the epidermis, vacuolar degeneration in the basal layer of the epidermis, lymphocyte exocytosis with necrotic keratinocytes, edema in the dermis, melanophages, and perivascular, periadnexal lymphocytic reaction. Laboratory tests showed negative antinuclear antibody and anti-dsDNA, but positivity for anti-Ro-52. In the absence of any other complaints, the patient was diagnosed with cutaneous lupus erythematosus presenting with periorbital erythema based on clinical, histopathological, and laboratory findings. Hydroxychloroquine 200 mg/day, topical corticosteroid, and topical tacrolimus were administered. Two months later, significant improvement in the lesions was observed. In conclusion, it should be kept in mind that periorbital erythema can develop as a rare variant of CLE and can be misdiagnosed as contact dermatitis, dermatomyositis, sarcoidosis, or cutaneous lymphoma. Additionally, the ANA and anti-dsDNA antibodies are often found to be negative in these cases. In establishing the diagnosis, firstly considering the disease, followed by histopathological examinations and laboratory tests, is crucial.
Keywords
Introduction
Discoid lupus erythematosus (DLE) is the most common chronic form of cutaneous lupus erythematosus, often localized to sun-exposed areas such as the scalp, neck, and extensor surfaces of the arms. Lesions present as erythematous, scaly plaques with atrophy and telangiectasia, and regress with hyperpigmentation or hypopigmentation. Unilateral and isolated involvement of the eyelid in DLE is quite rare. Periorbital DLE typically manifests as a subtly infiltrated erythematous plaque, and concomitant features such as atrophy, scaling, edema, or madarosis may or may not be present. 1 The accurate diagnosis of DLE can be challenging and time-consuming, as its morphological presentation may deviate from typical DLE lesions. In this report, we present a rare case of cutaneous lupus erythematosus characterized by unilateral and isolated involvement of the eyelid, highlighting the importance of recognizing atypical manifestations in DLE diagnosis.
Case report
A 42-year-old female patient presented with redness, swelling, and scaling on the upper right eyelid that started four months ago (Figure 1(a)). The patient’s history revealed a pregnancy-related pre-eclampsia 15 years ago, followed by membranoproliferative glomerulonephritis (MPGN), and pancreatitis six years ago. Additionally, the patient reported the onset of hepatitis two months after the appearance of eyelid lesions, but no underlying viral or autoimmune cause was identified. She had previously used topical corticosteroids with a preliminary diagnosis of contact dermatitis for the redness and swelling on the eyelid, but experienced no improvement. During the dermatological examination, a 3 × 2 cm erythematous, edematous plaque with mild scaling was observed on the upper right eyelid. When we initially examined the lesion on the eyelid of the patient, we conducted a dermoscopic evaluation. However, at that time, we were unable to capture photographs, and as a result, we could not document the dermoscopic features. Furthermore, due to the regression of the patient’s lesions, we regret to inform that dermoscopic photographs are currently unavailable. We performed a punch biopsy with a preliminary diagnosis of dermatomyositis, cutaneous lupus erythematosus, sarcoidosis, lupus vulgaris, and cutaneous lymphoma. Histopathological examination revealed focal hyperkeratosis and parakeratosis on the surface of the epidermis, vacuolar degeneration in the basal layer of the epidermis, necrotic keratinocytes, lymphocyte exocytosis, edema in the dermis, melanophages, and perivascular and periadnexal lymphocytic reaction (Figure 2(a) and (b)). In the immunohistochemical examinations, the lymphoid cells were CD3 positive and composed of a mixture of CD4 and CD8 positive cells, with no major antigen loss observed with CD5 and CD7 staining. Aside from intermittent muscle pains reported in the patient's history, there were no other complaints. There was no muscle weakness on physical examination, and apart from the plaque on the eyelid during dermatological examination, no abnormal findings were noted. Laboratory tests, including complete blood count, liver and kidney function tests, erythrocyte sedimentation rate, and CRP values were within normal limits. Antinuclear antibody (ANA) was negative, and in the extractable nuclear antigen (ENA) panel, only anti-Ro52 positivity was detected. Serum complement levels and rheumatoid factor were within normal limits, and due to a previous history of MPGN, there was a trace amount of proteinuria in the complete urine analysis. Muscle enzyme levels such as creatine kinase, aldolase, aspartate aminotransferase, alanine aminotransferase, and lactate dehydrogenase were normal, and electromyography revealed no pathological findings. Based on clinical, histopathological, and laboratory findings, a diagnosis of DLE presenting with periorbital erythema and edema was established. The patient was started on oral hydroxychloroquine 200 mg/day, topical corticosteroid, and topical tacrolimus treatments after ophthalmology consultation. Two months later, a significant improvement in the lesions was observed (Figure 1(b)). The patient is currently under follow-up and has no additional complaints. (a) A 3 × 2 cm erythematous and edematous plaque with mild scaling on the right upper eyelid. (b) Significant regression on the lesion after treatment. (a) Perivascular and periadnexial lymphomononuclear inflammatory cell reaction (H&E x20) and (b) basal vacuolar degeneration and lymphomononuclear cell exocytosis (H&E x100).
Discussion
Although the eyelid involvement in DLE has been reported at a rate of 5%–6%, it is frequently observed bilaterally and in the lower eyelids.2,3 Additionally, DLE lesions in different anatomical locations often coexist. 2 Unilateral, isolated eyelid involvement is quite rare. 4 Diagnosis can be challenging as the clinical appearance may differ from classic DLE lesions. If it is left untreated, it can lead to ophthalmic complications ranging from ectropion, madarosis, scarring, to blindness, emphasizing the importance of accurate diagnosis and prompt initiation of appropriate treatment. 5 In cases reported in the literature, there is a predominance of female gender for DLE presenting with unilateral, isolated periorbital erythema and edema, and the rarest involvement is observed in the upper right eyelid. 4 The average age of patients is 42.5 years, and the time elapsed until diagnosis varies between 2 months and 20 years. 4 Our case involved a 42-year-old female patient, and the duration from the onset of lesions to diagnosis was 4 months. In these patients, laboratory findings often show negative results for ANA and anti-ds DNA.4,6 In cases where ANA is positive, a speckled pattern is often observed, and in some cases, anti-SSA/SSB antibodies can be detected.4,5 In our case, ANA and anti-dsDNA were negative, and only anti-Ro52 positivity was present in the ENA panel. The first-choice agent for treatment is usually oral hydroxychloroquine. Additionally, topical corticosteroids, topical calcineurin inhibitors, intralesional triamcinolone, and oral corticosteroids, as well as immunosuppressive agents such as methotrexate, retinoids, thalidomide, dapsone, and azathioprine, can be used. 4 However, response rates to treatment vary among patients, and recurrences may occur. In this case, treatment included oral hydroxychloroquine at a dose of 200 mg/day along with topical corticosteroids and topical tacrolimus, and significant improvement in the lesions was observed after two months. In conclusion, isolated, unilateral involvement of the eyelid in DLE is a rare entity. We present this case to highlight the importance of considering DLE in the differential diagnosis when encountered with periorbital erythema and edema, emphasizing the rarity of involvement of the upper right eyelid in DLE, and underlining the importance of accurate diagnosis in treatment.
Footnotes
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
