Case Report | Open Access
Systemic Lupus Erythematosus Presented with Bilateral Orbital Edema and Negative Serology
Isolated bilateral periorbital edema with negative serology is an extremely rare presentation of cutaneous lupus erythematosus that may lead to eyelid scarring, infection, or even corneal involvement. The treatment usually comprises a combination of hydroxychloroquine and a tapering dose of systemic steroids. Patients require long-term follow-up as they may develop systemic lupus erythematosus with positive serology later in life. We report a case of a 32-year-old female who presented with chronic bilateral periorbital edema, and the histopathology confirmed cutaneous lupus erythematosus.
Lupus erythematous is a chronic inflammatory autoimmune disease which encompasses a wide range of clinical manifestations affecting multiple organ systems. There are four main types of lupus: neonatal lupus, discoid lupus, drug-induced lupus, and systemic lupus erythematosus . The modified Gilliam grouping system is a modified classification for cutaneous lupus erythematosus which comprises acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CLE) .
Clinically, each type of cutaneous lupus presents with some specific features. For instance, discoid lupus presents with thick, firm, erythematous, scaly, and well-demarcated plaques that have the potential to heal with scarring, alopecia, and hypopigmentation .
The presentation of eyelid edema is extremely rare; the reported incidence is 0.1% as for the presenting manifestation of systemic lupus erythematous with an overall incidence of 4.8% . Diagnosis for these cases is essential to prevent scarring, and long-term follow-up is needed as 5% of the population has the risk of developing systemic lupus erythematosus.
In general, the key characteristic that unites cutaneous lupus erythematosus is the histopathology presentations of the following features: hyperkeratosis, epidermal atrophy, vacuolar interface dermatitis, perivascular, and perifollicular mononuclear cell inflammatory infiltrate, with or without the presence of civatte bodies and follicular plugging .
2. Case Presentation
A 32-year-old African female presented to the emergency department complaining of persisted periorbital swelling, joint pain, and fever. The periorbital swelling was initially noticed 3 months before her presentation with mild pruritus and no visual disturbance. In addition, she was having intermittent joint pain involving the wrists, fingers, and shoulders. The fever was subjective and not documented with any history of sweating.
On presentation, she also complained of a two-week history of sore throat and difficulty in breathing. Paracetamol was the only medicine the patient was taking to manage her symptoms. She denied using any new medication or product and denied any previous history of allergies.
On her arrival, her vitals were normal and stable. Clinical examination revealed bilateral erythematous periorbital edema with violaceous hue, right cervical lymphadenopathy, and mild friction rub on the lower zone of the right lung. She had tenderness of the wrists, proximal metacarpal joints, and shoulders; movement restriction due to pain was noticed. Cardiovascular, breast, and abdominal examinations were uneventful.
Electrocardiography showed a sinus rhythm with a regular rate and no abnormalities. A chest X-ray showed pleural thickening at the right lung base.
The patient was admitted to the internal medicine department for further investigations. Abdominal ultrasound was normal with no abdominal lymphadenopathy. CT chest with contrast showed right lateral pleurodiaphragmatic adhesions and fibrotic bands.
WBC, white blood cell count; HBG, serum hemoglobin; PLT, platelets; Na, serum sodium; K, serum potassium; ALT, alanine transaminase; AST, aspartate aminotransferase; GGT, gamma-glutamyl transferase; ASO, antistreptolysin O; TIBC, total iron-binding capacity; TSH, thyroid-stimulating hormone; TFT, thyroid function test; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein.
ANA: antineutrophil antibody; CTD screen: connective tissue disease screen; anti-ds DNA: anti-double-stranded DNA; ACE: angiotensin-converting enzyme; anti-RNP70 antibody: anti-ribonucleoprotein antibody; anti-Jo1 antibody: myositis-specific autoantibodies directed against the histidyl-tRNA synthetase; anti-Scl-70: anti-topoisomerase antibody; anti-SSA/Ro autoantibodies: anti-Sjögren's-syndrome-related antigen A; anti-U1RNP: anti-ribonucleoprotein.
The echocardiogram showed a normal left ventricular cavity size with a preserved ventricular ejection fraction of 55%, and the systolic pulmonary artery pressure was 21 mmHg.
For this case, it was of interest to draw a list of differential diagnosis that includes sarcoidosis, Still’s disease, systemic lupus erythematosus, rheumatoid arthritis, amyloidosis, chronic blepharitis, eczema, contact dermatitis, psoriasis, and lymphoma. Still’s disease was brought to attention due to an elevated ferritin level; however, there was absence of the classic triad of persistent high-grade fever, joint pain, and a distinctive salmon-colored skin lesion.
Therefore, it remained unclear whether it was systemic lupus erythematosus or amyloidosis until skin biopsy from the eyelid was attempted, and the histopathology results showed degeneration with follicular plugging and dermal inflammatory cell infiltrate. These features were consistent with lupus erythematosus with skin involvement (Figure 1).
The patient was started on prednisolone 40 mg orally once per day with noticeable improvement in periorbital edema and joint tenderness; however, unfortunately, the patient had presented once in the internal medicine clinic after her discharge, while the skin biopsy results were still pending and did not attend further appointments.
Chronic bilateral periorbital edema that poorly responds to topical treatment should raise the suspicion of rare disorders, with possibility of neoplasm or connective tissue disease. Wu et al. reviewed 25 patients presented primarily with periorbital edema with a diagnosis of cutaneous lupus in addition to reviewing 10 cases from the literature. The majority of the patients were females (68%), and only four of the 25 patients had bilateral involvement. Seventy-two percent had their upper eyelid involved. Majority of the patients were misdiagnosed or suspected to have contact dermatitis, dermatomyositis, lymphoproliferative neoplasm, facial cellulitis, and severe angioedema. Our patient was a female with bilateral eyelid erythematous edema. The initial impression was of an allergic reaction and angioedema. Nineteen of the 25 patients as well as our patient had a negative ANA rate in that study (Table 3).
There is a broad implication of shared histological features in lupus erythematosus; 80% of patients had basal cell vacuolar degeneration, 44% had lymphocytic infiltration, and 40% had follicular plugging. Those findings were consistent with our case.
Although our case showed features such as arthritis, pleuritic thickening, and initial lymphopenia, the serologic profile came negative. Wu et al. reported 6 patients, who initially presented with periorbital edema and developed systemic involvement, meeting the American College of Rheumatology criteria for diagnosing systemic lupus. Furthermore, Wu et al. found that 10 cases with lupus erythematosus were reported in the literature with primary presentation of periorbital erythematous edema. Of those, 9 were females, and 6 had a positive ANA. Similarly, cases were misdiagnosed as lymphoproliferative disease, blepharitis, and chalazion.
Isolated bilateral orbital edema is an extremely rare presentation and found possible to be presented before other skin lesions, the challenges with negative serology impact on diagnosis for cutaneous lupus erythematous, and therefore, these cases are treated as chronic eczema or angioedema. It is essential for patients presenting with eyelid lesions and who fail the treatment for eczema or infections to have a full careful skin examination and skin biopsy for diagnosis.
Conflicts of Interest
The authors declare that they have no conflicts of interest.
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Copyright © 2019 Manal Al-Khaldi and Manal Alsabbagh. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.