Multicenter Canadian case series of pediatric patients less than 12 years of age with moderate-to-severe atopic dermatitis treated with dupilumab.

BACKGROUND: Dupilumab is approved for moderate-severe atopic dermatitis (AD) in patients aged ≥6 months by the US Food and Drug Administration and Health Canada; however, there are little real-world data because providers have limited practical experience with this recently approved therapy. OBJECTIVES: To describe the real-world effectiveness and safety in patients aged <12 years with moderate-severe AD currently receiving or previously having received dupilumab. METHODS: A multicenter retrospective study was conducted at six Canadian sites. Cases were divided into Group 1 ≤2 years old, Group 2 >2 to <6 years old, and Group 3 ≥6 to <12 years old. Medical history and details of dupilumab treatment were collected. The primary outcome was to measure the improvement in eczema area and severity index. Secondary outcomes examined included the children's dermatology life quality index/infant's dermatitis quality of life, peak pruritus numerical rating scale, and delay to dupilumab access for patients who were considered off-label for dupilumab due to their age. RESULTS: Sixty three pediatric patients (37 males) with moderate-to-severe AD were included; the mean age was 6.4 years old (range: 2-11) when dupilumab treatment was started. Overall, 75% (36/48) achieved EASI-75% and 71% (34/48) achieved EASI-90. EASI-75 and EASI-90 were achieved in 90% (17/19) and 73% (12/19) in patients <6 years old, and 76% (22/29) and 59% (17/29) in patients >6 years old, respectively. No serious adverse events were reported. CONCLUSIONS: Dupilumab is safe and effective for patients under the age of 12. However, even for experienced providers, access to the medication was challenging.

Successful treatment of exfoliative cheilitis with dupilumab: A case report.

This case report describes the first known successful treatment of exfoliative cheilitis with the interleukin-4 receptor alpha antagonist dupilumab in an adult patient. With no response to topical tacrolimus, hydrocortisone, dapsone, crisaborole or systemic doxycycline, low dose isotretinoin, or methotrexate, she had resolution with off-label dupilumab after 4 months of treatment.

An unusual case of pediatric embryonal rhabdomyosarcoma with subsequent diagnosis of neurofibromatosis type 1.

A 10-month-old girl presented with a 4-month history of a rapidly growing lesion on the lower lip. Initial assessment and Doppler ultrasound supported a diagnosis of pyogenic granuloma. However, emergent biopsy revealed an embryonal rhabdomyosarcoma, a highly malignant tumor commonly associated with cancer-susceptible syndromes including neurofibromatosis type 1 (NF1). Despite having no apparent clinical features of NF1 at initial presentation, she was later found to have multiple café-au-lait spots and a subsequent diagnosis of NF1 was made.

Management of congenital cutaneous candidiasis in a healthy term baby: A case report.

The management of congenital cutaneous candidiasis in a healthy term neonate of normal birth weight is unclear. Often, healthy term neonates undergo extensive evaluation followed by systemic treatment, which may not be clinically warranted. Here, we present a case of a healthy term neonate with congenital cutaneous candidiasis, whose work-up was minimally invasive and was successfully treated with one oral dose of antifungals and topical antifungals, as well as a review of the literature.

Pediatric Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Halted by Etanercept.

We report a case of an 11-year-old female with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) overlap, most likely triggered by sulfamethoxazole-trimethoprim, who was treated with the combination of methylprednisolone, cyclosporine, and etanercept. Her condition stabilized and her skin involvement did not progress after the addition of etanercept. To our knowledge, this is the first report of etanercept for pediatric SJS/TEN.

Patterns of skin disease in a sample of the federal prison population: a retrospective chart review.

BACKGROUND: Dermatology in vulnerable populations is under-researched. Our objective was to analyze the most commonly referred skin diseases affecting the Correctional Service Canada inmates in Ontario. METHODS: An observational, cross-sectional, retrospective chart review of inmate patients seen from 2008 until 2013 was performed. Two groups of patients were included in the analysis: those assessed in-person, and those evaluated by e-consult. RESULTS: In the in-person patient group, the 3 most common diagnoses were acne, psoriasis and other superficial mycoses. For the e-consult group, the 3 most frequent diagnoses were acne, psoriasis and rosacea. There was a clear bias toward more inmates being seen in-person where the service was provided (Collins Bay Institution) than from other correctional institutions in Eastern Ontario. INTERPRETATION: Most of the skin diseases that affected the incarcerated population studied were common afflictions, similar to those affecting the general population, which is in agreement with other studies. Future studies investigating skin diseases in male and female inmates across Canada would bestow more generalizable data.

Contact Cryoprobe Sterilization Practices: A Patient Quality and Safety Issue for Dermatologists.

BACKGROUND: Cryotherapy is a commonly employed treatment modality in dermatology. There is literature to suggest that sterilization of the contact cryotherapy probe is needed for proper decontamination. OBJECTIVE: To determine the status on the usage and sterilization methods employed by dermatology staff and residents affiliated with an academic training centre in Ontario, Canada, with the overall goal of improving patient safety. METHODS: An anonymous qualitative survey was conducted and analyzed. RESULTS: The survey had a response rate of 71% (n=34 of 48); 11 of 34 physicians reported using contact cryoprobes in their practice to treat viral infections, benign tumours, and malignant tumours. Out of the 11 using contact cryoprobes, 3 (27%) employed any type of cleaning method between patients. CONCLUSIONS: Current use of contact cryotherapy probes could be improved for safety considerations. Patient safety may be enhanced by the incorporation of routine autoclaving or other recommended decontamination methods of contact cryotherapy probes between patients.

Acute rhabdomyolysis as a complication of interferon treatment for stage IIIc melanoma.

BACKGROUND: Interferon alfa-2b is a treatment option for select patients with stage 3 melanoma. OBJECTIVE: To highlight the potential complication of acute rhabdomyolysis from interferon alfa-2b in this patient population. METHODS: We present a case of a 63-year-old female with stage IIIc melanoma who developed acute rhabdomyolysis after completing 2 of 4 weeks of induction with high-dose interferon alfa-2b. She recovered after hydration with intravenous fluids and discontinuation of interferon alfa-2b. RESULTS: Review of the literature revealed eight cases of reported rhabdomyolysis triggered by interferon use, including four cases of melanoma. CONCLUSION: In patients treated with interferon, especially at high doses, screening for creatine kinase and myoglobinuria should be performed if the patient develops symptoms suggestive of acute rhabdomyolysis.

The prophylactic use of C1 inhibitor in hereditary angioedema patients undergoing invasive surgical procedures: a retrospective study.

BACKGROUND: Hereditary Angioedema (HAE) is a rare autosomal dominant condition characterized by episodic angioedema, which may be triggered by invasive procedures and surgery. C1 inhibitor (C1 INH) was approved in the United States and Canada in 2009 and 2010, respectively, for the treatment of acute attacks. Most recently in April 2013, it was approved in Europe for short-term prophylaxis (STP), prior to medical, dental, or surgical procedures, to prevent HAE attacks in both children and adults. Currently, C1 INH is not approved in Canada or the United States for STP of HAE attacks. Our objective was to demonstrate the effectiveness of C1 INH as a short-term prophylactic treatment for patients with Type I HAE undergoing invasive surgical procedures. METHODS: A retrospective chart review between 1997-2013 was performed at one Canadian Tertiary Care Allergy and Asthma Clinic affiliated with The Ottawa Hospital, in Ottawa, Canada. The standard dose of C1 INH for STP was 10 or 20 U/kg. RESULTS: In all 24 procedures, there were no post-procedure HAE attacks after short-term prophylactic administration of C1 INH. CONCLUSIONS: In this retrospective chart review at one tertiary care Allergy and Clinical Immunology Clinic, short-term prophylactic use of C1 INH was found to be effective at preventing post-procedure HAE attacks, in patients diagnosed with Type I HAE.

Allergic contact dermatitis from dimethyl fumarate after contact with a Chinese sofa.

BACKGROUND: Dimethyl fumarate has been successfully used in the treatment of psoriasis in the past. Despite its clinical use, cutaneous contact with this molecule may cause contact dermatitis. OBJECTIVE: We report a case in which skin exposure to a synthetic fabric sofa containing dimethyl fumarate made by a Chinese furniture manufacturer resulted in a severe, pruritic, papulovesicular, eczematous dermatitis to the sites of contact with the sofa. METHODS: The patient was patch-tested with serial dilutions of dimethyl fumarate. RESULTS: Patch testing results revealed strong positive reactions to dimethyl fumarate at different concentrations. CONCLUSION: Dimethyl fumarate is a potent contact sensitizer and is commonly found in sachets inside furniture and footwear boxes.

A 10-year review of p-phenylenediamine allergy and related para-amino compounds at the Ottawa Patch Test Clinic.

BACKGROUND: p-Phenylenediamine (PPD) is an important allergen; 5.0% of patients tested positive to PPD when patch-tested, according to the North American Contact Dermatitis Group. Hair dyes are the main source of exposure. OBJECTIVE: To assess the significance of PPD allergy at the Ottawa Patch Test Clinic. METHODS: We assessed the epidemiology of PPD allergies and determined the cross-reactivity with other para-amino compounds. Charts of patients visiting the Ottawa Patch Test Clinic between May 1997 and July 2009 were reviewed. RESULTS: One hundred thirty-four patients were found to have a contact allergy to PPD; 75.4% were female, 24.6% were male, 13.4% were hairdressers, 18.7% had a history of atopy, 90.3% were sensitized by hair dye, 2.2% were sensitized by henna tattoos, and 7.5% were sensitized by other sources. Positive patch-test reactions to textile dyes were seen in 24.6%, 7.5% reacted to benzocaine, 6.0% reacted to sulfa drugs, 1.5% reacted to isopropyl-para-phenylenediamine, and 1.5% reacted to para-aminobenzoic acid. CONCLUSIONS: PPD is an important source of allergic contact allergy. Our results show a significant relationship of PPD with other related para-amino compounds.

An automated system for delivery of an unstable transcription factor to hematopoietic stem cell cultures.

An automated delivery system for cell culture applications would permit studying more complex culture strategies and simplify measures taken to expose cells to unstable molecules. We are interested in understanding how intracellular TAT-HOXB4 protein concentration affects hematopoietic stem cell (HSC) fate; however, current manual dosing strategies of this unstable protein are labor intensive and produce wide concentration ranges which may not promote optimal growth. In this study we describe a programmable automated delivery system that was designed to integrate into a clinically relevant, single-use, closed-system bioprocess and facilitate transcription factor delivery studies. The development of a reporter cell assay allowed for kinetic studies to determine the intracellular (1.4 +/- 0.2 h) and extracellular (3.7 +/- 1.8 h and 78 +/- 27 h at 37 degrees C and 4 degrees C, respectively) half-lives of TAT-HOXB4 activity. These kinetic parameters were incorporated into a mathematical model, which was used to predict the dynamic intracellular concentration of TAT-HOXB4 and optimize the delivery of the protein. The automated system was validated for primary cell culture using human peripheral blood patient samples. Significant expansion of human primitive progenitor cells was obtained upon addition of TAT-HOXB4 without user intervention. The delivery system is thus capable of being used as a clinically relevant tool for the exploration and optimization of temporally sensitive stem cell culture systems.