A Review of the Risk of Cow's Milk Protein Allergy in Oral Medication.

Immunoglobulin E (IgE)-mediated cow's milk allergy (CMA) is a common food reaction resulting from the consumption of cow's milk protein (CMP). The clinical manifestations of CMA include mild to severe urticaria, skin-manifested hypersensitivity reactions, and anaphylaxis. Food allergies may affect 8% of children and 10% of adults. The Federal Food, Drug, and Cosmetic Act (FD&C Act) requires that the label of a food must declare the presence of a "major food allergen" (MFA) contained in the food or ingredient. The Food and Drug Administration (FDA) generally regards milk protein concentrate (MPC) as safe for human consumption and use. The increasing use of MPC in formulations raises the need for its revelation in prescription and on labels of over-the-counter drugs. This review investigates oral and topical (including mucosal) preparations containing MPC for dermatologic and other uses and their therapeutic impact. Our findings suggest that for the adult population, the risk of serious cow's milk protein allergy (CMPA) from medications is minimal.

Silver-impregnated hydrofiber dressing followed by delayed surgical closure for management of infants born with giant omphaloceles.

OBJECTIVE: We successfully employed silver-impregnated hydrofiber dressing for management of giant omphaloceles (GO) followed by delayed surgical closure. STUDY DESIGN: Between 2005 and 2008, eight consecutive GO infants were cared for at Driscoll Children's Hospital. Four patients had additional congenital anomalies including Beckwith-Wiedemann (n = 1), tetralogy of Fallot (n = 1), pulmonary hypoplasia (n = 1), and ruptured omphalocele (n=1). Infants underwent amnion epithelization using a silver-impregnated hydrofiber dressing over the course of several months followed by delayed surgical closure. Mean ± SD of parameters including maternal age, gestational age, infant weight, size of GO, preoperative intubation, preoperative hospitalization, time to epithelization, days to surgical closure, postoperative hospitalization, postoperative intubation and months of follow-up were studied. RESULTS: Five patients underwent successful closure, 2 were lost to follow-up and 1 was lost because of withdrawal of support. The maternal age, gestation age and weight of infant were 28 ± 5.3 years, 34 ± 4 weeks and 2.5 ± 0.62 kg, respectively. The GO size was 11 cm in length and 11 cm in width, respectively. Preoperative hospitalization days were 78 ± 74 days. Preoperative intubation was 3.5 ± 3.1 days with 2 neonates requiring tracheostomy and home ventilation owing to additional congenital abnormalities. Time to epithelization was 2.9 ± 0.9 months. Days to surgical closure and postoperative hospitalization were 331 ± 119 days and 5 ± 3.4 days, respectively. Average follow-up was 37 ± 27 months. No treatment associated morbidities are noted. CONCLUSIONS: Silver-impregnated hydrofiber mediated epithelization of GO followed by delayed surgical closure is safe for management of infants.

Case of 7p22.1 Microduplication Detected by Whole Genome Microarray (REVEAL) in Workup of Child Diagnosed with Autism.

Introduction. More than 60 cases of 7p22 duplications and deletions have been reported with over 16 of them occurring without concomitant chromosomal abnormalities. Patient and Methods. We report a 29-month-old male diagnosed with autism. Whole genome chromosome SNP microarray (REVEAL) demonstrated a 1.3 Mb interstitial duplication of 7p22.1 ->p22.1 arr 7p22.1 (5,436,367-6,762,394), the second smallest interstitial 7p duplication reported to date. This interval included 14 OMIM annotated genes (FBXL18, ACTB, FSCN1, RNF216, OCM, EIF2AK1, AIMP2, PMS2, CYTH3, RAC1, DAGLB, KDELR2, GRID2IP, and ZNF12). Results. Our patient presented features similar to previously reported cases with 7p22 duplication, including brachycephaly, prominent ears, cryptorchidism, speech delay, poor eye contact, and outburst of aggressive behavior with autism-like features. Among the genes located in the duplicated segment, ACTB gene has been proposed as a candidate gene for the alteration of craniofacial development. Overexpression of RNF216L has been linked to autism. FSCN1 may play a role in neurodevelopmental disease. Conclusion. Characterization of a possible 7p22.1 Duplication Syndrome has yet to be made. Recognition of the clinical spectrum in patients with a smaller duplication of 7p should prove valuable for determining the minimal critical region, helping delineate a better prediction of outcome and genetic counseling.

Introduction to autoinflammatory syndromes and diseases.

Autoinflammatory syndromes and diseases are a group of disorders of innate immunity. This group has grown rapidly in recent years as a result of research advancements in molecular biology and genetics. These diseases often present with skin manifestations and the dermatologist may not recognize the constellation of symptoms and medical history as a systemic inflammatory disease. Dermatologists would benefit from a deeper understanding of these diseases and the new treatments available for them.

Pralatrexate (Folotyn).

T-cell lymphoma accounts for 10% to 15% of all cases of non-Hodgkin lymphoma in the United States (approximately 5000 to 6000 cases a year). Peripheral T-cell lymphoma (PTCL) comprises a subgroup of rare and aggressive non-Hodgkin lymphomas that develop from T cells in different stages of maturity outside of the thymus. Cutaneous T-cell lymphoma is a subgroup that falls within the T-cell lymphoma population but is classified differently than other PTCLs. Most cases of CTCL are considered indolent and can often be treated with less aggressive therapies. Eight percent to 55% of CTCL cases undergo transformation, and once this transformation occurs, the disease acts similarly to other PTCLs and its classification changes to that of a PTCL. Transformed CTCL requires aggressive systemic therapy. Pralatrexate is the first Food and Drug Administration-approved drug for relapsed and refractory PTCL and has also gained compendia approval for treatment of CTCL. Pralatrexate is an antifolate chemotherapeutic inhibitor of dihydrofolatereductase. It has a high affinity for the one carbon-reduced folate carrier, which leads to better cellular internalization of the drug and has a greater antitumor effect than methotrexate. Several clinical trials have been conducted to evaluate the use of this drug in PTCL and other malignancies such as non-small cell lung cancer. This review offers focused information for dermatologists about pralatrexate and its use as a novel treatment for relapsed or refractory PTCL.

Topical vitamin D analogs available to treat psoriasis.

Psoriasis is a chronic and currently incurable inflammatory skin disease, affecting 2% to 3% of the US population. The cost of care in the United States for hospitalizations, outpatient physician visits, phototherapy, prescription therapies, and over-the-counter medications is estimated to be more than $650 million per year. Guidelines developed by the American Academy of Dermatology in 2009 state that approximately 80% of these patients with psoriasis have mild to moderate disease that can be managed with topical agents, including corticosteroids and vitamin D analogs. Topical vitamin D analogs provide "steroid-sparing" effects and a favorable safety profile. Many experts, including a recent consensus conference, classify topical vitamin D agents as first-line therapy for psoriasis either as monotherapy or in combination with topical steroids due to a synergistic, complementary effectiveness. Vitamin D analogs are an indispensable component of the current physician's armamentarium for psoriasis treatment. This review, therefore, is oriented to give a comprehensive understanding of this group of drugs and display the available clinical data for each formulation.

Hydrocortisone butyrate 0.1% cream (proprietary lipid rich cream vehicle) does not significantly suppress hypothalamic-pituitary-adrenal axis and is effective in pediatric patients 3 months and older with extensive atopic dermatitis.

Only a few corticosteroids for topical use have been proven safe and effective in pediatric populations down to 3 months of age. The authors examined the systemic safety (adrenal suppression potential) of topically applied hydrocortisone butyrate 0.1% cream (proprietary lipid rich cream vehicle) in the treatment of moderate to severe atopic dermatitis in pediatric patients aged 3 months to 6 years and 12 years to 18 years. An open-label hypothalamic-pituitary-adrenal axis suppression study was conducted wherein the sole treatment was 0.1% proprietary lipid rich cream vehicle. A total of 65 patients with moderate to severe atopic dermatitis and body surface area involvement of at least 25% were included in the treatment phase of the study based on the requirement that these patients had normal baseline cortisol and hypothalamic-pituitary-adrenal system function. All signs and symptoms of atopic dermatitis showed progressive improvement beginning with day 8 through the day 29 evaluation. Pruritus showed the greatest improvement, with a decrease in grade of 1.3 at day 8, and continued to show improvement at day 29, with a decrease of 1.8 from baseline. The percent body surface area affected at baseline averaged 40.5%, and it decreased significantly to a mean of 6.5% at day 29. Only 5 (8%) of the 63 patients showed laboratory evidence of adrenal suppression at the end of the treatment evaluation. None of these ever demonstrated any clinical signs or symptoms of adrenal suppression. This study adds hydrocortisone butyrate 0.1% cream, to the short list of corticosteroids that have been proven effective and safe by the cosyntropin suppression test in children 3 months and older with widespread atopic dermatitis.

Hydrocortisone butyrate 0.1% lipocream in pediatric patients with atopic dermatitis.

Only a few corticosteroids for topical use have proven safe and effective in pediatric populations down to 3 months of age. The authors report the results of a study designed to assess the efficacy and safety of hydrocortisone butyrate (HCB) 0.1% in lipocream (LCr) vehicle in infants and children. A total of 264 boys and girls 3 months to less than 18 years old, with stable, mild to moderate atopic dermatitis affecting at least 10% body surface area applied HCB 0.1% in LCr or LCr alone twice daily for up to 1 month without occlusion. Primary end-points included: percent of patients who achieved treatment success based on physician global assessments. Secondary endpoint included: difference in pruritus and Eczema Area and Severity Index (EASI) at day 29. Treatment was significant (P < 0.001) for HCB 0.1% LCr over vehicle. No serious nor significant adverse events were reported. Results are representative of a short duration treatment for a chronic disease. HCB 0.1% in LCr is more effective than its vehicle in pediatric populations down to 3 months of age without significant adverse events when used twice a day for up to 1 month.