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.

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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.

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.

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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.

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.

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.

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.

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.