Phototherapy with UVB narrowband, UVA/UVBnb, and UVA1 differentially impacts serum 25-hydroxyvitamin-D3.

BACKGROUND: Ultraviolet (UV) B radiation increases serum 25-hydroxyvitamin-D3 [25(OH)D], but the influence of UVA1 and UVA/narrowband UVB (UVBnb) phototherapy on serum vitamin D is unknown. OBJECTIVE: We sought to investigate the influence of UVBnb, UVA1, and UVA/UVBnb phototherapy on serum levels of 25(OH)D and related parameters in patients with an inflammatory skin condition. METHODS: 25(OH)D, as well as calcium, parathormone, phosphate, and albumin were measured before therapy, 2 weeks after start, and after completion of the phototherapy. Diagnoses were divided in 4 groups: atopic dermatitis, psoriasis, morphea, and others. RESULTS: We surveyed 116 dermatologic patients undergoing phototherapy with UVA1 (n = 38), UVA/UVBnb (n = 30), or UVBnb (n = 48) 2 to 3 times a week for 53 to 90 days. UVBnb phototherapy increased serum 25(OH)D from 22.1 to 39.5 ng/mL after the therapy (P < .001). The lower the baseline 25(OH)D level was, the steeper the increase in 25(OH)D was upon application of UVBnb phototherapy. UVA/UVBnb therapy also increased serum 25(OH)D, from 23.9 to 50.3 ng/mL (P = .003). Conversely, in the UVA1 therapy group, 25(OH)D serum levels decreased significantly from 21.9 to 19.0 ng/mL (P < .001). LIMITATIONS: The study design was open trial without randomization. An influence of a precise skin disease cannot be excluded because of the heterogeneous diagnoses. Bias may have arisen from patient preference for treatment at our center, referral, unrecognized differences in underlying skin disease, and other factors. CONCLUSION: Phototherapy with UVBnb and UVA/UVBnb increased 25(OH)D serum level significantly. UVA1 therapy alone induced a reduction in serum 25(OH)D concentrations.

Dermatofibrosarcoma protuberans in childhood treated with slow Mohs micrographic surgery.

Dermatofibrosarcoma protuberans (DFSP) in childhood is a rare tumor with high recurrence rates. Wide local excision can result in disfiguring mutilation, whereas Mohs micrographic surgery (MMS) reduces surgical margins. MMS in children is not performed routinely, as the required infrastructures such as a histopathology lab in close proximity to the operating room is often lacking. We retrospectively reviewed children diagnosed with DFSP treated at our hospital over 2 years. We recorded surgical treatment details, including margins, duration of inpatient stay, outcome, follow-up, and molecular genetic tumor tissue analysis. Four children with a median age of 6.8 years (range 6.0-8.8 years) were identified who had a diagnostic delay of a median of 2.5 years (range 0.5-4.0 years); all underwent complete tumor excision using the slow MMS technique using vacuum-assisted closure systems between repeated excisions and before wound closure. The median maximal safety margins were 1.5 cm (range 1.0-3.0 cm). By using vacuum-assisted closure systems, no dressing changes were needed, pain was limited, and full mobility was maintained in all children. The median total time in the hospital was 11 days (range 10-14 days). No relapses occurred during a median follow-up of 25.8 months (range 11.3-32.6 months). Collagen 1A1/platelet-derived growth factor B (COL1A1/PDGFB) translocation on chromosomes 17 and 22 was detected in all three analyzable specimens. Lesions suspected of being DFSP warrant prompt histologic evaluation; interdisciplinary management is mandatory in particular for children. Micrographic surgery allows smaller surgical margins than wide excision and should be considered as the treatment of choice in children with DFSP. The interim usage of vacuum-assisted closure systems increases patient comfort. Translocations in the COL1A1/PDGFB gene imply susceptibility to targeted treatment modalities for therapy-resistant cases.

Microsporum audouinii tinea capitis in a Swiss school: assessment and management of patients and asymptomatic carriers.

We report three cases involving 7- to 8-year-old children from a Swiss school who had refractory tinea capitis due to an unusual strain of Microsporum audouinii which perforates hair in vitro. The patients showed no response to modern oral antifungal drugs like terbinafine and fluconazole. After switching to oral griseofulvin, two of the patients had a complete recovery, while the third was cured after the introduction of oral itraconazole. Given the high potential for contagion of this anthropophilic dermatophyte, all family members and three entire school classes were screened using the 'toothbrush technique'. Three family members and five class-mates were found to be asymptomatic carriers of M. audouinii and were consequently treated to avoid further transmission or reinfection of the treated patients. This is the first report of an outbreak of M. audouinii in Switzerland and underlines the importance of screening all contacts of patients with M. audouinii tinea capitis. Further, the effectiveness of griseofulvin in Microsporum tinea capitis has been corroborated, while newer antimycotic drugs like fluconazole or terbinafine failed.

Secukinumab Therapy for Netherton Syndrome.

Importance: Netherton syndrome (NS) is a rare, severe genetic disorder of cornification with high morbidity. Treatment for NS has been notoriously difficult. Recent studies showed an upregulated helper T cell (TH) 17/interleukin 23 (IL-23) pathway in NS, suggesting the possibility of treatment strategies that target IL-17. Objective: To evaluate the clinical response of NS to treatment with the IL-17 antagonist secukinumab. Design, Setting, and Participants: This case series study reports the experience of compassionate use therapy with secukinumab in 4 patients with severe NS, including 2 children, from December 1, 2018, to December 1, 2019, with 3 patients still undergoing treatment at the time of final analysis. Data were analyzed from December 1, 2018, to December 1, 2019. Main Outcomes and Measures: Expression of IL-17 in the skin was evaluated by immunohistochemical analysis, and serum cytokine concentrations were measured using a commercially available assay. Treatment response was assessed using the Ichthyosis Area and Severity Index (IASI) total score, including measures of erythema and scaling, the Dermatology Life Quality Index (DLQI), and the 5-D itch scale. Results: In all 4 patients (age range, 9-27 years; 3 male and 1 female), immunostaining with an IL-17A antibody showed an increased number of positive cells in lesional skin. Cytokine assessment in serum samples revealed increased levels of CCL20. Treatment duration with secukinumab was 3 to 12 months at the time of this report. After 3 months of therapy, IASI scores were reduced by 44% to 88%, DLQI scores were reduced by 40% to 76%, and 5-D itch scale scores were reduced by 27% to 62%. This outcome was sustained at the 6-month follow-up. Two patients with an erythrodermic phenotype showed marked improvement of all parameters. A refractory palmoplantar eczematous eruption occurred in 2 patients, and a candidal nail infection developed in 2 patients. No severe adverse events were reported. Conclusions and Relevance: This initial case series reporting the use of anti-IL-17 therapy in NS demonstrated marked cutaneous improvement, particularly in 2 pediatric patients with erythrodermic phenotypes. Further studies are needed to evaluate the long-term benefit of this potential treatment modality.

Life-threatening or organ-impairing Henoch-Schönlein purpura: plasmapheresis may save lives and limit organ damage.

Adult-onset Henoch-Schonlein purpura (HSP) tends to become chronic-relapsing, yet rarely leads to organ impairment, e.g. due to chronic glomerulonephritis. Bed rest, compression and nonsteroidal anti-inflammatory drugs are usually sufficient to control the active phases. We report 2 cases of adult HSP with an unusually severe evolution. One patient required intensive-care treatment for hypovolemic shock caused by hemorrhagic pancolitis; the other had progressive and extremely extensive vasculitic leg ulcers. Both were refractory to common immunosuppression with systemic corticosteroids (oral and pulse) and additive steroid-sparing immunosuppressive drugs. Only after the introduction of plasmapheresis did both patients show a dramatic improvement in the disease, with rapid and almost complete healing. Plasmapheresis is a rarely used therapeutic tool in the treatment of severe HSP, but the growing literature on its highly beneficial effect underlines its potential usefulness.

Comparison of in-hospital secondary prevention for different vascular diseases.

BACKGROUND: Secondary prevention of coronary artery disease is highly effective and implemented on a large scale. However, studies testing adherence to recommended secondary prevention of other vascular diseases are rare. Our goal was to evaluate whether the kind of vascular disease influences prescription practice of secondary drug prophylaxis at hospital discharge and to which extent secondary prevention is actually complete. METHODS: A 3-month prospective observational review of the hospital discharge information of all patients hospitalized because of a vascular disease diagnosis: coronary artery disease (i.e. acute myocardial infarction [AMI] and chronic stable angina [CSA]); peripheral artery disease [PAD] and cerebrovascular disease [CVD]. The analysis was done by board registered internists with a structured form that founded on internationally accepted recommendations. RESULTS: From 271 patients 191 had coronary artery disease (105 AMI and 86 CSA), 88 PAD and 72 CVD. Global prescription rate (mean; 95% CI) of indicated secondary prophylaxis drugs was 74.1% (69.9-78.2) for AMI, 72.4% (67.2-77.5) for CSA, 74.7% (68.8-80.7) for PAD and 72.1% (66.9-77.3) for CVD. The proportion of patients who were prescribed a complete bundle of recommended medications was globally 29.5% (24.1-35.0). CONCLUSIONS: We found similar global prescription rates of secondary prevention for the different vascular diseases. However, only one third of the studied collective gets a complete set of required prophylactic drugs.