Devi Jhaveri

A unique case of peroneus brevis/longus myositis in a patient with a STAT3 mutation

Figure 1. Cross-section of patient’s MRI showing extensive enhancement of peroneus brevis and longus muscle bellies on T2 images. E (IgE) syndrome (HIES). These patients experience recurrent infections involving opportunistic organisms of the lung and skin, including staphylococcal abscesses and pneumonias with pneumatoceles.1,2 The syndrome is also characterized by markedly elevated serum IgE levels and may include other features such as joint hyperextensibility, scoliosis, osteopenia, delayed shedding of primary teeth, coarse facial features, and pruritic dermatitis.3e5 The National Institutes of Health has developed a scoring system to classify patients with suspected HIES, with a score of 40 or higher being suggestive of HIES.2 We present a 36-year-old African Americanmanwith the history of a STAT3 mutation and a history of severe infections, including an empyema requiring thoracotomy with surgical lobectomy at age 2 years, knee abscess requiring incision and drainage, right arm abscess requiring incision and drainage, and four pneumonias requiring hospitalization. The patient’s total serum IgE was 2,728 Ku/L, and his HIES National Institutes of Health score was 53, with points for his IgE level, skin abscesses, pneumonias pneumatocele, fractures, characteristic facies, eczema, upper respiratory infections, candidiasis, hyperextensibility, and increased nasal bridge. Genotyping showed a STAT3 mutation involving exon 12, Thr389Ile, which is consistent with the diagnosis of HIES. He presented to the office complaining of persistent right lower leg pain with swelling for the previous 3 days involving the outer aspect of his right ankle. Despite the pain and swelling, he continued to ambulate without difficulty. The patient noted no history of trauma or repetitivemovement to the foot or ankle and had no prior surgery or injury to that area. He did admit to scratching his foot and the development of excoriated areas of his forefoot before the symptoms. Consistent with his presentation of prior severe infections, he did not have fever, chills, or night sweats, butwas requiring oral narcotics around the clock to continue his work. The area of involvement of his right lower leg showed no warmth or erythema, although it was painful to manipulation. A magnetic resonance imaging (MRI) scan revealed inflammation of the peroneus brevis and longus muscle bellies (Fig. 1), and a serum creatine phosphokinase was elevated at 813, suggesting muscle involvement. Because of the patient’s underlying immune status, hewas referred to an infectious disease specialist who reviewed the MRI and started the patient on intravenous vancomycin and cefepime via peripherally inserted central catheter line for presumed bacterial myositis confined to the bellies of the peronius muscles. The patient’s symptoms and clinical presentation improved dramatically within 24 hours of initiation of the antimicrobial treatment, and he completed his therapy with a course of oral

Ocular desensitization in the face of local anesthetic hypersensitivity

Local anesthetics (LAs) have been used in medicine since 1884 when Carl Koller applied cocaine to the cornea. Hypersensitivity reactions to LAs have been associated with a metabolite of ester group anesthetics as well as components found within the LA solutions. True IgE-mediated allergic reactions are very uncommon and contribute to less than 1% of all LA reactions. Rarely individuals demonstrate hypersensitivity to both ester and amide group LAs. We present the first documented case of ocular bupivacaine desensitization in a patient with suspected ester and amide group hypersensitivity. A 52-year-old white female with bilateral central retinal vein occlusion and subsequent retinal edema required intraocular ranibizumab injections every 5 weeks with ocular LA pretreatment. In February 2013, the patient began exhibiting reactions to LAs with development of urticaria, throat fullness, dyspnea, confusion, and fatigue within minutes of subconjunctival injection of an amide anesthetic, lidocaine. Subsequent topical conjunctival application of tetracaine and benoxinate, both members of the ester group of LAs, failed as the patient immediately developed shortness of breath, dysphagia, and increased sputum production. Skin prick testing with full-strength benoxinate HCL revealed erythema and a 3-mm wheal. Within minutes of skin testing, she developed tachycardia and urticaria with upper body erythema and was tremulous. She was treated successfully with multiple doses of intramuscular epinephrine, intravenous methylprednisolone, and normal saline fluid. Following these reactions, a challenge with topical bupivacaine and subsequent subconjunctival bupivacaine injections were tolerated. However, after 3 years of repeated bupivacaine treatments, she developed a transient episode of pruritus, dysphagia, and heart palpitations within minutes of an ocular bupivacaine injection. Within 1 hour of the procedure, the patient developed a second reaction composed of chest tightness and shortness of breath responsive to inhaled albuterol. The patient was reevaluated 2 weeks after this reaction. Baseline serum tryptases and urinary histamine levels at that time were found to be normal. Skin testing was performed to mepivacaine 1%, bupivacaine 0.25%, lidocaine 2%, and prilocaine 4%, all of which were preservative-free. Initial skin prick testing yielded no reaction for all LAs tested. Intradermal skin testing results at 1:100 dilution were negative with the exception of a 3-mm wheal noted to mepivacaine. Full-strength intradermal testing to the 3 remaining LAs revealed a 3-mm wheal with erythema to lidocaine and a 4-mm wheal with erythema to prilocaine and was negative to bupivacaine. One of the main complications of central retinal vein occlusion is legal blindness. Because of the morbidity of the disease, it was crucial for the patient to be continued on ranibizumab injections every 5 weeks with LA pretreatment. Because of the patient’s history of immediate hypersensitivity reactions to multiple LAs and most recently a suspected immediate and delayed hypersensitivity reaction to previously tolerated bupivacaine, an ocular desensitization protocol was designed (Table I) for bupivacaine in an attempt to allow ranibizumab treatment to proceed. Pretreatment with cetirizine 10 mg orally the evening before and the day of the procedure was used to optimize safety and success of the desensitization. Peripheral intravenous access was obtained before the desensitization. The desensitization protocol was started by diluting bupivacaine to a concentration of 1:1000 and began administering 0.03 mL topically. Then, every 15 minutes 0.03 mL of an increased concentration of the diluted bupivacaine was given. Five minutes after the fifth application (see Table I), the patient experienced a brief episode of tachycardia to 158 beats per minute without any additional symptoms. This spontaneously resolved without treatment. As a precautionary measure, the patient did receive 1 dose of diphenhydramine (25 mg intravenously) 5 minutes after the tachycardia resolved. The patient completed the desensitization procedure (see Table I) without further difficulty and ultimately tolerated her ranibizumab injection. She has subsequently undergone several more monthly procedures without adversity using the protocol described above. Of note is the fact that the total dose of bupivacaine administered was 3 to 4 mg, well below the usual cardiotoxic threshold of 1 to 2 mg/kg published in the literature. This is the first documented case of probable ocular desensitization to an LA in a patient with suspected LA hypersensitivity to both ester and amide LAs. Historically, our patient had experienced hypersensitivity reactions to numerous LAs, including bupivacaine, suggestive of an allergic mechanism. Neither prick nor intradermal skin testing fully supported the entire clinical history of multiple LA hypersensitivity reactions in this patient. A topical desensitization protocol was developed that ultimately resulted in our patient being able to safely receive bupivacaine on a repetitive basis. Clinicians may consider this methodology for patients with suspected LA hypersensitivity who require ocular LA injections and for which there are no alternative agents.

Fetal response to intramuscular epinephrine for anaphylaxis during maternal penicillin desensitization for secondary syphilis

Abstract Penicillin desensitization is indicated in pregnant patients with severe allergies to penicillin with syphilis. The immediate effects of intramuscular epinephrine on the fetus during desensitization remain unreported. We describe a pregnant patient with secondary syphilis and penicillin allergy who developed anaphylaxis during penicillin desensitization. Anaphylaxis resolved after administration of intramuscular epinephrine. Throughout the procedure, continuous electronic fetal monitoring showed a stable fetus without a decrease in variability, tachycardia, decelerations, or signs of fetal distress. This case showed that intramuscular epinephrine is effective in treatment of anaphylaxis in a pregnant patient with little to no immediate effects on the fetus.

Stratification of peanut allergic murine model into anaphylaxis severity risk groups using thermography

Murine models are readily used to investigate mechanisms potentially involved in anaphylaxis. Determining successful sensitization with current methods remain potentially lethal, invasive, expensive and/or cumbersome. Here we describe the use of thermography to read intradermal testing to detect peanut allergic sensitization in the murine model and as a first time sensitive tool for anaphylaxis stratification. The relative wheal size in the thermal image can be used to stratify anaphylaxis severity risk groups prior to a challenge. This screening method is nonlethal, inexpensive, minimally invasive and can be carried out expeditiously.

Chronic lymphocytic lymphoma presenting with recurrent demodicidosis

allergy in patients not detected by skin testing. Of the patients with negative skin test reactions but positive chlorhexidine sIgE reactions, 2 had a clinical picture consistent with chlorhexidine allergy. For this reason, we concur with suggestions that a combination of diagnostic methods is advisable in association with the clinical history.3,7 With respect to the high sensitivity and specificity seenwith the chlorhexidine sIgE assay, a positive result in the context of a low total IgE level might be sufficient for a diagnosis of chlorhexidine allergy in some settings. This particularly might be the case where there is a strong clinical history or in cases in which skin testing is unclear or unable to be performed. However, it should be noted that sensitivity and specificity were evaluated using sIgE measurements determined on serum samples collected at the time of clinic attendance for skin testing. Therefore, the timing of a serum test might be relevant to the utility of this protocol, particularly in view of the need for further studies in this area as previously mentioned. Overall, our evaluation of sIgE testing to chlorhexidine by the ImmunoCAP method shows this to be a reliable diagnostic method to use in addition to skin testing in the investigation of perioperative allergy. Adjustment of the cutoff threshold also can increase sensitivity without significantly affecting specificity. However, results should be evaluated with caution in the presence of total IgE levels higher than 500 kU/L and particularly at levels higher than 2,000 kU/L.

Hypogammaglobulinemia treated with infusion therapy associated with cortisol deficiency

contain a C-terminal carbohydrate binding module, family 20, domain.3 In summary, we have identified glucoamylase I precursor in N. crassa CM using human scFv antibodies. Glucoamylase I precursor is recognized by IgE from some individuals with bakers’ asthma and mold allergy, and its presence in vaccines expressed in N. crassa needs to be controlled. Besides glucoamylase, antibody NC65 recognizes a-amylase from A. oryzae and possibly S. cerevisiae, and may be useful as a tool for allergen detection.