Immunoglobulin A vasculitis with periorbital edema and severe renal involvement: A case report.

Immunoglobulin A (IgA) vasculitis, or Henoch-Schonlein purpura, is the most common systemic vasculitis in children, clinically presenting as palpable purpura in combination with arthritis, gastrointestinal involvement, or kidney injury. Subcutaneous edema is reported in patients with IgA vasculitis, and it commonly affects the lower extremities, especially around joints. Here, we report a case of IgA vasculitis with a rare presentation of edema isolated to the periorbital area in a 7-year-old boy, who subsequently developed crescentic glomerulonephritis with nephrotic range proteinuria. Isolated periorbital edema is an uncommon cutaneous feature of IgA vasculitis.

Cutaneous angiosarcoma presenting as bilateral periorbital edema.

A 66-year-old man presented with chronic bilateral periorbital edema with associated yellowish hue, scattered violaceous smooth macules and contracture of the forehead. He had undergone dental surgery 3 months prior to symptom onset. Laboratory workup for common causes of eyelid edema was unremarkable and MRI of the orbits was unrevealing. The patient did not respond to oral corticosteroids or antibiotics. Punch biopsies were obtained which revealed atypical lymphatic endothelial cells consistent with a diagnosis of cutaneous angiosarcoma.The patient was deemed not to be a surgical candidate and underwent 3 cycles of immunotherapy with limited response. He declined further treatment and transitioned to hospice care. Although cutaneous angiosarcoma uncommonly involves the periorbital region, it should be considered in the differential diagnosis of eyelid edema as early recognition and treatment are critical to prevent rapid intradermal spread and metastases.

Bilateral periorbital leukemia cutis presenting as suspected cellulitis.

Many conditions present with periorbital edema and erythema, mimicking preseptal cellulitis. We report the unique case of a patient with relapsed monoblastic mutant isocitrate dehydrogenase-2 (IDH2) acute myeloid leukemia (AML) who presented with periorbital edema and erythema, unresponsive to antibiotics. Histopathology from punch biopsy was consistent with leukemia cutis. The patient responded rapidly to the initiation of enasidenib, a novel targeted inhibitor of mutant IDH2 enzymes. Our case highlights the importance of considering leukemia cutis in patients with a history of leukemia presenting with periorbital edema and erythema.

Safety of low-dose oral minoxidil for hair loss: A multicenter study of 1404 patients.

BACKGROUND: The major concern regarding the use of low-dose oral minoxidil (LDOM) for the treatment of hair loss is the potential risk of systemic adverse effects. OBJECTIVE: To describe the safety of LDOM for the treatment of hair loss in a large cohort of patients. METHODS: Retrospective multicenter study of patients treated with LDOM for at least 3 months for any type of alopecia. RESULTS: A total of 1404 patients (943 women [67.2%] and 461 men [32.8%]) with a mean age of 43 years (range 8-86) were included. The dose of LDOM was titrated in 1065 patients, allowing the analysis of 2469 different cases. The most frequent adverse effect was hypertrichosis (15.1%), which led to treatment withdrawal in 14 patients (0.5%). Systemic adverse effects included lightheadedness (1.7%), fluid retention (1.3%), tachycardia (0.9%), headache (0.4%), periorbital edema (0.3%), and insomnia (0.2%), leading to drug discontinuation in 29 patients (1.2%). No life-threatening adverse effects were observed. LIMITATIONS: Retrospective design and lack of a control group. CONCLUSION: LDOM has a good safety profile as a treatment for hair loss. Systemic adverse effects were infrequent and only 1.7% of patients discontinued treatment owing to adverse effects.

Sex difference in clinical presentation of patients with infectious mononucleosis caused by Epstein-Barr virus.

INTRODUCTION: There are few studies on sex difference in patients with infectious mononucleosis caused by Epstein-Barr virus (EBV-IM). We performed a retrospective study to evaluate the sex difference in clinical presentation of patients with EBV-IM. METHODS: We performed a single-center retrospective study evaluating >14-year-old patients with serologically confirmed EBV-IM during 2006-2017. We compared the patients' age, symptoms, physical findings, and laboratory data between male and female patients. To adjust for confounding factors, we performed a logistic regression analysis based on the results of univariate comparisons. RESULT: Of the 122 eligible patients (56 male and 66 female, ratio: 1:1.2), the median ages were 26 years old (interquartile range [IR], 22-31.5 years old]) and 22 years old (IR, 20-25 years old) for males and females, respectively (p < 0.001). Headache was significantly more prevalent in males (25.0% vs. 10.6%, p = 0.036). Leukocyte count was also significantly higher in males (11,400/mm3 [IR, 7,600-14,100/mm3] vs. 9,400/mm3 [IR, 6,600-11,600/mm3], p = 0.021). The prevalence of periorbital edema (male: 3.6% vs. female: 18.1%, p = 0.012) and severity of transaminase elevation were significantly higher in females. The regression analysis evaluating clinical characteristics of male patients showed that age >30 years old, headache, and leukocyte >11,000/mm3 had high odds ratios. CONCLUSION: Our single-center retrospective study suggests that older age of onset, headache, and leukocytosis are more likely to be characteristics of male patients with EBV-IM. Our study also underscores the importance of periorbital edema as a clue for early diagnosis of EBV-IM, especially in female patients.

Silicone oil migration into the orbit.

PURPOSE: To report a case in of intravitreal silicone oil migration into the inferior orbit. Silicone oil tamponade is commonly used in retinal detachment repair and extrusion into the orbital tissues is very rare. METHODS: A 70-year-old male with a remote history of repair of a right ruptured globe and retinal detachment surgery presented with progressive right lower eyelid edema. There was a known history of intravitreal silicone oil instillation and removal. An elective right inferior orbitotomy with excisional biopsy was performed. RESULTS: Histopathology confirmed the presence of silicone oil intermixed with necrotic fat. The patient had excellent cosmetic and functional outcome. CONCLUSION: The presence of silicone oil within the orbital fat may be a result of increased intraocular pressure and extrusion through presumably water-tight sclerotomy sites.

Dosage-dependent role of Rac1 in podocyte injury.

Activation of small GTPase Rac1 in podocytes is associated with rodent models of kidney injury and familial nephrotic syndrome. Induced Rac1 activation in podocytes in transgenic mice results in rapid transient proteinuria and foot process effacement, but not glomerular sclerosis. Thus it remains an open question whether abnormal activation of Rac1 in podocytes is sufficient to cause permanent podocyte damage. Using a number of transgenic zebrafish models, we showed that moderate elevation of Rac1 activity in podocytes did not impair the glomerular filtration barrier but aggravated metronidazole-induced podocyte injury, while inhibition of Rac1 activity ameliorated metronidazole-induced podocyte injury. Furthermore, a further increase in Rac1 activity in podocytes was sufficient to cause proteinuria and foot process effacement, which resulted in edema and lethality in juvenile zebrafish. We also found that activation of Rac1 in podocytes significantly downregulated the expression of nephrin and podocin, suggesting an adverse effect of Rac1 on slit diaphragm protein expression. Taken together, our data have demonstrated a causal link between excessive Rac1 activity and podocyte injury in a dosage-dependent manner, and transgenic zebrafish of variable Rac1 activities in podocytes may serve as useful animal models for the study of Rac1-related podocytopathy.

Periorbital Emphysema Due to Traumatic Pneumothorax.

Periorbital emphysema is rare and associated with facial trauma. Its main distinguishing feature is crepitation on palpation of the edema. It resolves spontaneously in a few days, but there are cases of orbital compartment syndrome that can lead to loss of vision. Here we present the case of a 55-year-old male who came to the emergency department for bilateral periorbital edema associated with non-steroidal anti-inflammatory drug (NSAID) usage, for pain following a fall from a ladder. He was treated with antihistamines and corticosteroids, for presumed allergic reaction, but without response, and subsequently developed acute onset dyspnea. Chest x-ray revealed a left pneumothorax in the context of chest trauma. Chest CT scan after drain placement shows extensive subcutaneous emphysema. In the differential diagnosis of periorbital edema, in addition to allergic, inflammatory, and systemic causes, the traumatic ones should not be excluded.

Gene polymorphisms affect postoperative imatinib plasma levels and edema in adults with gastrointestinal stromal tumor.

Aim: To assess the role of genetic polymorphisms in postoperative imatinib concentrations and edema in patients with gastrointestinal stromal tumor. Methods: The relationships between genetic polymorphisms, imatinib concentrations and edema were explored. Results: Carriers of the rs683369 G-allele and rs2231142 T-allele had significantly higher imatinib concentrations. Grade ≥2 periorbital edemas were related to the carriership of two C-alleles in rs2072454 with an adjusted odds ratio of 2.85, two T-alleles in rs1867351 with an adjusted odds ratio of 3.42 and two A-alleles in rs11636419 with an adjusted odds ratio of 3.15. Conclusion: rs683369 and rs2231142 affect the metabolism of imatinib; rs2072454, rs1867351 and rs11636419 are related to grade ≥2 periorbital edemas.

Treatment of Periorbital Edema in a Patient With Systemic Lupus Erythematosus During Pregnancy: A Case Report Written With the Assistance of ChatGPT.

Systemic lupus erythematosus (SLE) is an autoimmune disease that has a wide range of manifestations and can affect nearly every organ system. Skin manifestations are a common finding in SLE. They are often photosensitive and can be exacerbated by exposure to ultraviolet light. Here, we discuss the case of a 34-year-old African American woman who presented with periorbital edema while 12 weeks pregnant. This case highlights the importance of avoiding sun exposure in patients with SLE and the challenge of treating SLE during pregnancy.

Episcleritis and periorbital edema secondary to VEXAS syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described autoinflammatory entity caused by a UBA-1 gene mutation. Among the most frequent symptoms it produces fever, cytopenias, polychondritis, pulmonary infiltrates and up to 40% ocular involvement such as periorbital edema, uveitis, episcleritis, scleritis and retinal vasculitis. Patients respond to high doses of corticosteroids, however, many end up being refractory to them and to the classic immunosuppressants. We described the case of a 77-year-old male patient with ocular involvement in the form of episcleritis and periorbital edema who was later diagnosed with VEXAS Syndrome. The patient, after failing treatment with immunosuppressants, is currently receiving treatment with oral steroids and tocilizumab. Ophthalmologist must be aware of the ophthalmological affectation of autoinflammatory diseases and especially of this new entity described as the VEXAS Syndrome.

Systemic Lupus Erythematosus Presenting With Severe Hypothyroidism and Extensive Pericardial Effusion in a Child.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, which may be associated with other autoimmune diseases, like autoimmune hypothyroidism. Both disorders can involve the cardiovascular system and cause pericardial effusion with cardiac tamponade. Herein, we describe a young eight-year-old female patient who initially presented with periorbital edema, cold intolerance, fatigue, and papular skin rash that was present on the face and the chest and was found to have significant pericardial effusion along with bilateral pleural effusion. Further laboratory investigation done in the hospital revealed severe hypothyroidism and positive SLE antibodies (antinuclear antibodies [ANA], antidouble strand DNA [anti-ds-DNA], and Sjögren's syndrome antibodies A and B [SS-A and SS-B]). She was administered levothyroxine and pulse methylprednisolone, which significantly improved her condition. She was discharged on maintenance therapy with regular follow-ups with a multidisciplinary team.

Dermatomyositis Masquerading As Generalized Body Swelling: A Case Report.

Dermatomyositis (DM) is a systemic autoimmune disease that primarily affects the skin and muscles. The hallmark skin manifestation is a violaceous rash on the face, neck, shoulders, upper chest, and extensor surfaces of the arms and legs, which is often accompanied by edema and can be exacerbated by exposure to sunlight. Generalized limb edema and dysphagia are rare presentations of dermatomyositis. Here we present a case of a 69-year-old woman presenting with generalized limb swelling, periorbital swelling, and dysphagia which was diagnosed as dermatomyositis based on a combination of clinical, laboratory, and imaging findings. The patient had an absence of complaints of limb weakness and a predominance of complaints of edema and dysphagia which posed a diagnostic challenge. The patient was treated with high-dose steroids and immunosuppressive therapy, leading to a significant improvement in her symptoms. Edematous dermatomyositis has been associated with underlying malignancy in 25% of the cases and this warrants close follow-up and malignancy screening for such patients. In some cases, subcutaneous edema might be the only manifestation of the disease. This case underscores the importance of recognizing DM as a potential differential diagnosis in patients presenting with generalized edema and dysphagia, particularly in the initial absence of classic skin findings. This rare presentation of dermatomyositis may be a hallmark of a severe form of the disease and requires prompt recognition and aggressive treatment.

Periorbital Edema as the Initial Manifestation of Pediatric Systemic Lupus Erythematosus.

Periorbital edema as a sole initial manifestation, without any evidence of other significant cutaneous or systemic involvement, is rare in systemic lupus erythematosus (SLE). We report a 16 year-old female who presented with bilateral periorbital edema as the sole initial manifestation of SLE. As the disease progressed, a kidney biopsy was performed demonstrating lupus nephritis stage II. This report emphasizes the importance of the index of suspicion of SLE as one of the differential diagnosis of patients with periorbital edema in the relevant clinical context.

Contrasting presentations of the same disease: A comparison of two cases of amyloidosis presenting with eyelid involvement.

Purpose: Localized amyloidosis can affect numerous tissues throughout the body and can also affect a variety of peri-ocular tissues including the conjunctiva, extra-ocular muscles, peri-orbital soft tissue, and lacrimal gland. We report two cases of amyloidosis presenting with eyelid involvement. Observations: The first case represented a more subtle presentation of skin thickening with a pre-septal cellulitis, while the second case had a dramatic presentation of edema evolving into tissue dehiscence and spontaneous hemorrhage with ongoing angioedema and systemic coagulopathy. Conclusions and importance: The two cases of biopsy-proven orbital/peri-ocular amyloidosis demonstrate the different clinical presentations that may go from the subtle to dramatic, depending on which peri-ocular tissues are affected and to what degree. Standards for treatment of amyloidosis remain conservative initially with surgery or radiation recommended only for refractory cases, but additional therapies are under investigation. Clinicians should have high clinical suspicion for amyloidosis with findings such as skin thickening or significant periorbital edema and should always consider tissue biopsy and further workup for amyloidosis if the findings worsen or do not resolve with treatment of more common conditions such as cellulitis.

Langerhans Cell Histiocytosis of the Orbit Presenting as Periorbital Cellulitis.

Langerhans cell histiocytosis (LCH) is a rare disease of the reticuloendothelial system. It is characterized by misguided differentiation of myeloid dendritic cell precursors. LCH most commonly presents in childhood and the clinical presentation is dependent on the site and extent of organ involvement. We report a case of orbital Langerhans cell histiocytosis in a 12-year-old boy who presented with left periorbital cellulitis with subsequent proptosis. He later underwent a successful left orbital-frontal-temporal craniotomy and is currently undergoing postoperative chemotherapy.

Lupus-Induced Myopic Shift.

While ophthalmic manifestations of lupus are common, a myopic shift is a rare manifestation of systemic lupus erythematosus (SLE). An acute myopic shift is defined as a progressive worsening of nearsighted vision within a short time frame. Here, we describe the unique presentation of a young woman with a lupus-induced acute myopic shift. The patient presented with blurry vision and bleeding gums with a previous abnormal lymph node biopsy to rule out ocular lymphoma or leukemia. Her baseline prescription prior to coming in was -4.0D in both eyes. Upon exam, she presented with vision worsening: -7.0D in the right eye and -8.2D in the left eye. After completing blood laboratory tests, it became clear that she had pancytopenia, kidney damage, and elevated inflammatory markers pointing towards lupus. A diagnosis of acute binocular myopic shift induced by systemic lupus erythematosus was made.

Massive periorbital edema following hematopoietic stem cell transplantation.

Purpose: To describe a case of severe, bilateral periorbital edema after hematopoietic stem cell transplantation. Observations: A three-year old girl with metastatic neuroblastoma underwent the second of two tandem autologous peripheral blood stem cell transplants, complicated by engraftment syndrome. On post-engraftment day 11, she developed acute onset of severe periorbital edema. She was soon thereafter diagnosed with transplant-associated thrombotic microangiopathy with significant volume overload requiring treatment with eculizumab and etanercept. Periorbital edema resolved after four days with concurrent treatment of her underlying condition. Conclusions and Importance: We report an ocular manifestation related to complications of hematopoietic stem cell transplantation. This highlights a non-infectious etiology of eyelid swelling in the post-transplant, immunocompromised population.

Valproate-induced Periorbital Edema.

INTRODUCTION: Valproate is an antiepileptic medication that can be used to manage behavioral symptoms associated with Alzheimer's dementia. We present a rare case of valproate-induced periorbital edema. CASE: A 76-year-old man came to the emergency room with agitation and aggression. He was medically cleared and referred to a psychiatric facility where he was treated with haloperidol. When he developed drug-induced parkinsonism, the haloperidol was stopped, and the patient was started on valproate 250 mg twice daily. The day after valproate administration, the patient developed periorbital edema. After ruling out other causes of periorbital edema, adverse drug reaction was suspected. Valproate was discontinued, and the edema rapidly resolved within five days. CONCLUSION: Periorbital edema is a rare side effect of valproate. It can occur in patients who are being treated with valproate for behavioral changes in Alzheimer's disease. The edema resolves with discontinuation of the medication.