Understanding Hurley Stage III Hidradenitis Suppurativa Patients' Experiences With Pain: A Cross-Sectional Analysis.

BACKGROUND: More than 90% of patients with hidradenitis suppurativa (HS) report that pain interferes with their quality of life (QoL) and pain may have a larger impact on QoL than disease severity alone. OBJECTIVES: The purpose of this study was to understand the impact of pain on the daily lives of patients with Hurley stage III HS. METHODS: This was a single-center, prospective cross-sectional study that was conducted at Beacon Dermatology in Calgary, AB. Patients ≥ 18 years old with Hurley stage III HS in at least one area of the body were prospectively invited to participate in this study. The study consisted of survey questions on patients' demographic information, past medical histories, HS-related pain histories, and previous therapies for pain management. Additionally, patients completed a series of standardized rating scales on their pain and overall QoL. RESULTS: Of the 10 patients that participated in the study, 90% (9/10) expressed a desire for more counselling on pain management options. Many patients (8/10, 80%) reported routine use of over-the-counter pain medications and 70% (7/10) used complementary and alternative medicines (CAMs) to manage their pain. Patients' efficacy ratings of HS treatments in controlling their pain revealed that topical treatments provided minimal or no relief, while surgical interventions had the highest efficacy for reducing pain. Patients' average worst pain over the preceding 24 hrs was 6.3 +/- 2.5 (2-9) on the Numerical Rating Scale for pain and the mean Dermatology Life Quality Index score was 19.5 +/- 8.2 (5-29). CONCLUSIONS: Patients with Hurley stage III HS report high levels of daily pain and QoL impairment and many individuals use over-the-counter treatments and CAMs to manage their pain. Physicians involved in the care of HS patients should consider implementing routine counselling on pain management into their clinical practices, especially for patients with severe HS.

Human Leukocyte Antigen Gene Testing and Carbamazepine-Induced Toxic Epidermal Necrolysis: A Study of Pediatric Practice.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening drug-induced dermatologic conditions. SJS/TEN occurs in 1-10 per 10 000 patients taking carbamazepine (CBZ) (Pratt VM, McLeod HL, Rubinstein WS et al. Medical Genetics Summaries. National Center for Biotechnology Information US; 2018: 1-527). The development of SJS/TEN is associated with variable drug metabolism and presence of an at-risk HLA haplotype. HLA-B*15:02 and HLA-A*31:01 haplotypes can produce a hyperimmune response in the setting of CBZ use in patients of Asian and European descent, respectively (Schneider JA, Cohen PR. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A concise review with a comprehensive summary of therapeutic interventions emphasizing supportive measures. Adv Ther. 2017; 34:1235-1244). OBJECTIVE: The US Food and Drug Administration (FDA) and the Canadian pharmacogenomics Network for Drug Safety (CPNDS) recommend that patients with high-risk ethnic backgrounds should be genetic tested before initiating CBZ (Sukasem C, Chaichan C, Nakkrut T et al. Association between HLA-B Alleles and Carbamazepine-induced maculopapular exanthema and severe cutaneous reactions in Thai patients. Journal of Immunology Research. 2018; 1-11).We sought out to assess the awareness of this in prescribing practitioners and their standard of practice. METHODS: We created a 15-question survey and distributed to pediatric neurologists and pediatricians at the University of Alberta. We hypothesized that there was a discordance between the standard of practice and the recommendation by the FDA and CPNDS. RESULTS: The survey results indicated a lack of awareness of the at-risk ethnicities for CBZ-induced SJS/TEN. HLA gene testing was rarely done prior to initiation of CBZ in high-risk patients. In addition, there was a lack of awareness for standard of care for genetic testing in Canada and worldwide. CONCLUSIONS: Our results demonstrate an evident gap between current prescriber practices and existing FDA and CPNDS recommendations to screen for HLA genotypes. We hope that this study captures the realistic potential to improve patient outcomes.

Onset of frontal fibrosing alopecia during inhibition of Th1/17 Pathways with ustekinumab.

The mechanism underlying frontal fibrosing alopecia (FFA) is unknown, but proposed mechanisms share commonality of T cell-mediated destruction of the hair follicle bulge. IL-12 and IL-23 are key cytokines involved in CD4 T cell differentiation towards Th1 and Th17 phenotypes. We present a 62-year-old woman who developed persistent FFA while on ustekinumab for treatment of preexisting psoriasis. This case presents evidence against Th1 and Th17 pathways as essential to pathogenesis in FFA. This case also suggests that IL-12 and IL-23 inhibition is ineffective for this form of scarring alopecia.

Macular cutaneous amyloidosis treated with methyl aminolevulinate and daylight photodynamic therapy: A case report.

Primary cutaneous amyloidosis is characterized by polymerization of extracellular amyloid precursors in ß-pleated sheet conformation into larger fibrillar aggregates. Observation in models of Alzheimer's disease have noted that amyloid polymerization in the brain is blocked by reactive oxygen species. Singlet oxygen is formed in the skin during methyl aminolevulinate photodynamic therapy. Therefore, we speculate that type II photochemical reaction is responsible for the observed therapeutic activity of methyl aminolevulinate photodynamic therapy in our patient with primary cutaneous amyloidosis. Our case is the first report demonstrating the efficacy of daylight photodynamic therapy in primary cutaneous amyloidosis. Daylight photodynamic therapy may provide a convenient and cost-effective therapeutic option in primary cutaneous amyloidosis, and its efficacy should be further confirmed in prospective trials.