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Periodic facial erythema is associated with postural orthostatic tachycardia syndrome (POTS), an abnormal response of the autonomic nervous system. This syndrome of orthostatic intolerance is characterized by tachycardia in the absence of orthostatic hypotension, light-headedness, palpitations, and rarely facial erythema. Resolution of symptoms occurs with recumbence. Here we present a case of periodic facial erythema secondary to POTS.
Primary antiphospholipid syndrome (PAPS) has rarely been reported with C1 INH AAE,and all previous patients had underlying Lymphoproliferative disease (LPD), MGUS or SLE. We report a 79 y.o. woman with type II C1INH AAE of the face and tongue and PAPS with recurrent thrombosis (DVT) and pulmonary embolism (PE). Therapeutically CINH concentrates requiring IV or SQ infusions, and thrombogenic products were excluded. Danazol followed by lanadelumab-flyo with icatibant rescue were selected for therapy.
Studies have examined effects of delivery mode and feeding practices on allergic outcomes, though most have focused on a single condition. The influence of these factors on development of multiple allergic manifestations is not known. We studied the effects of delivery mode and feeding practices on allergic disease burden over time.
Mepolizumab has been shown to reduce asthma exacerbations ≥50% in randomized clinical trials. However, patients in the real world diverge from some of the clinical features and adherence behaviors present at the time of enrollment in clinical trials. This study examines the exacerbation reduction in patients receiving mepolizumab in a managed care claims database and compares a subset that reflects the study entry criteria and treatment adherence in pivotal clinical trials.
Pathogenic variants in NDUFAF3 leading to mitochondrial complex I deficiency are associated with a variety of phenotypes often leading to mortality at an early age. Ichthyosis vulgaris is caused by FLG gene loss-of-function variant. Ichthyosis vulgaris renders increased risk of environmental toxin/irritant exposures causing secondary disease due to impaired functional barrier.
Making and playing with “Slime” is popular among preteens and adolescents1. Ingredients may cause contact and/or irritant dermatitis. We report a case of a 9YO female with Slime associated chronic dermatitis.
There is an increasing incidence of allergic contact dermatitis to corticosteroids. However, this diagnosis is being undetected or misdiagnosed, since topical corticosteroids are the first line treatment for inflammatory skin disorders.
Hypereosinophilic Syndrome (HES) is a group of disorders with persistent peripheral eosinophilia and organ dysfunction. Cutaneous T cell lymphomas (CTCLs) are disorders with clonal T cells invading the skin, and eosinophilia is a poor prognostic factor in CTCL. We present the case of a patient in whom distinguishing between HES and CTCL has been challenging.
Scleromyxedema is a disease of unknown etiology that usually affects middle aged adults and presents with widespread eruptions of firm, waxy papules containing mucin deposits. Extracutaneous manifestations include carpal tunnel syndrome, polyarthritis, dysphagia, and heart failure. Several treatment modalities including IVIG, steroids, and thalidomide have been used but typically it is refractory to medical interventions.
This is the case of 39-year-old man seen ten days after hospitalization in the ICU with a diagnosis of anaphylaxis triggered by Alka Seltzer.
Delayed type IV hypersensitivity drug reactions are common but alternative diagnoses must be considered with an atypical history and in specific patient populations.
Wells syndrome is a rare disease characterized by skin eruption of various morphologies which may vary over time. Histologically there are three distinct phases (acute, subacute and resolution) each with defining characteristics. Integration of clinical features alongside histopathology is imperative for diagnosis. Topical and oral corticosteroids are the most common treatment; however, relapses are common. An effective steroid-sparing option is desired for those needing long-term therapy.
Urticarial vasculitis (UV) is defined by urticarial rash and histopathologic signs of cutaneous vasculitis, often with systemic features. Mild forms of the disease are treated with antihistamines, while severe forms may require systemic immunosuppressive therapies.
The patient is an 18-year-old male with genetically confirmed ichthyosis (KRT10 gene mutation), who presented with atopic findings at age 12 and subsequently developed severe persistent asthma, eosinophilic esophagitis, and multiple anaphylactic food allergies.
In a patient with long-standing history of atopic dermatitis (AD) presenting with increased frequency, severity, and diffuse nature of recent flares, it is important to thoroughly search for a precipitating factor
Allergic fungal sinusitis is caused by a hypersensitivity reaction to fungal allergens in atopic individuals, which can cause significant pathology.
Sluder’s neuralgia (also known as sphenopalatine neuralgia) consists of neuralgic, sensory and gustatory manifestations attributed to irritation of sphenopalatine ganglion, usually from infection, deformity or scarring. Pain is associated with nasal and/or sinus congestion, swelling or erythema of nasal mucosa, tearing and facial erythema. Medical therapy for sinus decongestion can usually alleviate symptoms.
Bacterial cultures of nasal secretions from the middle meatus, obtained by rhinoscopy, may mirror the microbial environment within the sinuses. In theory, nasal mucus blown from the nose may produce a more accurate culture result than a swab of nasal mucous membranes. It is common to find antibiotic resistant bacteria as a cause of chronic sinusitis. Antibiotic sensitivity testing of bacterial isolates can aid in directing therapeutic choices. Pantoea agglomerans (formerly Enterobacter agglomerans)...
Find co-morbid conditions such as immune deficiency that facilitate ongoing disease activity in patients with CRS w NP.
We discuss the rapid progression of the symptoms and the multidisciplinary approach to treatment of this allergic condition. rhinosinusitis and allergic fungal polyposisAn elderly veteran was diagnosed with nasal
Chronically ill patients with recurring rhinosinusitis and asthma forget feeling well. This results in "brain fog” and making poor health care choices. Patients want to participate in their health care decisions.
Eosinophilic myocarditis is a rare form of myocarditis caused by a range of diseases. Though the underlying etiology can guide therapy, the mainstay of treatment is systemic corticosteroids, with limited options for refractory disease.
64 year old male with polymyositis previously treated with rituximab 5 years prior presented with hypogammaglobulinemia. Polymyositis was poorly controlled and the patient needed clearance prior to restarting rituximab. The patient had no significant infection history. Screening laboratories included a low IgG of 610, low IgM of 31 and protection to 4/14 post vaccination pneumococcal titers.
Urticarial vasculitis is a small vessel vasculitis characterized by urticarial lesions which can leave bruising upon resolution. It often causes pain and pruritus and can be difficult to treat with traditional medications thereby adversely affecting quality of life.
Dupilumab is currently approved for the treatment of asthma and atopic dermatitis. Dupilumab targets the interleukin 4 receptor alpha subunit, thereby inhibiting IL-4 and IL-13, which decreases Th2 inflammation and may cause incidental improvement in other allergic diseases.
This report highlights a patient with no prior history of urticaria, who developed chronic urticaria after discontinuing omalizumab treatment for asthma.
Patients with multiple conditions are managed by individual specialists with their own focused treatment endpoints who do not always coordinate care. How do we optimize treatment for a complex patient with multiple active atopic conditions?
Chronic eosinophilic pneumonia (CEP) has a 50% response rate to oral (OCS). There is no consensus regarding the use of biologic agents as therapeutic alternatives for patients with steroid-dependent CEP unresponsive to OCS.
Aspirin-exacerbated respiratory disease (AERD) consists of nasal polyposis, asthma, chronic rhinosinusitis and hypersensitivity reactions with aspirin or other COX-1 inhibitor, such as non-steroidal anti-inflammatory drugs (NSAIDS). Aspirin therapy after desensitization is a treatment option for AERD patients with worsening nasal polyposis or recurring polyps after surgery despite conventional therapy.
Sjögren's syndrome is an autoimmune disease characterized primarily by decreased exocrine gland function leading to eye and mouth dryness. Extraglandular manifestations occur less frequently.
Patients with a tracheostomy with unexplained persistent airway or peristomal inflammation are increasingly referred for allergy assessment. While plastic allergy is relatively rare, some tracheostomy tubes contain metal, and other topical products and dressing materials are used which have the potential to induce delayed-type (Type IV) hypersensitivity (DTH).
M358 SUCCESSFUL OFF-LABEL USE OF OMALIZUMAB IN THE TREATMENT OF A PATIENT WITH SYSTEMIC MASTOCYTOSIS
Systemic mastocytosis (SM) is an accumulation of mast cells in tissue. Patients with SM may have clinical manifestations involving cutaneous, gastrointestinal, musculoskeletal, cardiovascular, neurological systems. Patients are also more likely to have anaphylaxis following a Hymenoptera sting. Current treatment for SM includes antihistamines, antileukotriene agents, Oral cromolyn. Imatinib may be used in patients without D816V mutation. Omalizumab is not currently approved for use in SM.
A 47 year old gentleman presented to a local ED with five days of substernal chest pain. He had ST depressions and a troponin of 18, however cardiac catheterization was negative. Eosinophils were found to be 8000.
Mastisol® is commonly used in operating rooms and other healthcare settings to enhance adhesive integrity. Although an etiology for hardware failure was not identified, component(s) of Mastisol® appeared to distinctly cause cutaneous symptoms following PT.
Hypereosinophilic syndrome (HES) is characterized by an eosinophil level above 1500 and tissue damage, typically affecting multiple organs. Cardiac involvement is associated with a poor prognosis. High-dose mepolizumab therapy has been successful in treating patients with HES.
A 65-year-old female with a metal allergy developed a refractory diffuse pruritic rash with fatigue three months after having a right total knee replacement. Our case is the first reported case of omalizumab successfully treating contact dermatitis to metal joint prosthesis.
Hereditary alpha-tryptasemia (HAT) is an autosomal dominant disorder characterized by excess copies of the alpha-tryptase gene TPSAB1. There is elevated baseline tryptase and associated symptoms, including flushing, hives, irritable bowel syndrome and dysautonomia. We describe a patient with HAT and interstitial cystitis (IC) in whom bladder-directed therapy was effective in treating systemic symptoms.
Hereditary alpha-tryptasemia is a recently described disorder characterized by increased copy numbers of the TPSAB1 gene resulting in elevated tryptase. We present a novel case of hereditary alpha-tryptasemia in which the patient had both TPSAB1 duplication and c-kit mutation.
Allergic conjunctivitis typically presents with symptoms of bilateral eye pruritus, tearing, hyperemia, and eyelid edema. Ocular symptoms have been reported in as high as 40% of the population. We describe a case of allergic conjunctivitis with progressive unilateral eye symptoms.
While uncommon, anaphylaxis to coconut has been described1–7. We report three cases of anaphylaxis during physician supervised coconut oral food challenge (OFC).
Frequently, patients with atopic dermatitis (AD) avoid otherwise tolerated foods due to concerns for possible flares of AD, or sensitization found on blood and/or skin prick testing (SPT). Most studies in patients with AD demonstrate poor predictive value when comparing food allergy (FA) testing and immediate reactions during oral food challenge (OFC). Furthermore, avoidance of some foods carries a risk of developing IgE-mediated FA.
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated gastrointestinal food hypersensitivity that presents with profuse, repetitive vomiting and associated dehydration. Common triggers are cow’s milk, soy and cereals. FPIES due to fish and shellfish is rare and most commonly presents in older children and adults. We report the youngest patient with FPIES due to shrimp.
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food hypersensitivity. Diagnosis may be delayed due to a combination of nonspecific symptoms, the absence of definitive diagnostic tests, and an overall lack of familiarity with FPIES. We report a severe case of unrecognized FPIES in a newborn.
There is increased recognition of development of food allergy through solid organ transplantation. We present a patient without known food allergy, who acquired peanut sensitization after bilateral lung transplantation from his organ donor.
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Abstract Background Severe skin reactions, mostly following medication use, are rare and can be associated with high mortality. A suitable treatment approach that is able to reduce mortality is needed. Methods Recent publications on this topic were reviewed and evaluated. Results ...
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Abstract PBC is a chronic progressive autoimmune disorder involving the destruction of intrahepatic small bile ducts, cholestasis, fibrosis, and ultimately cirrhosis if left untreated. It is largely driven by the autoimmune response, but bile acids and the intestinal microbiota are implicated in disease progression as well. The only drugs licensed for PBC are UDCA and OCA. UDCA as a first-line and OCA as a second-line therapy are safe and effective, but the lack of response in...
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