Systemic Therapy for Vascular Anomalies and the Emergence of Genotype-Guided Management.

Improved understanding of the genetic basis of vascular anomalies has uncovered a growing need for targeted medical therapies. This is especially important for lesions not amenable to surgical interventions or interventional radiologic techniques. Recent studies and case reports have documented the effective use of tailored medical therapies in several distinct types of vascular anomalies. Sirolimus, mitogen-activated protein kinase inhibitors, and phosphoinositide 3-kinase inhibitors have emerged as potential therapies. Although this remains a growing field with significant knowledge gaps, a more optimistic outlook for patients with previously devastating impact on function and quality of life seems now within reach.

Successful management of an arteriovenous malformation with trametinib in a patient with capillary-malformation arteriovenous malformation syndrome and cardiac compromise.

Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is an autosomal dominant condition characterized by multifocal, noncontiguous pink patches on the skin that often have a surrounding pale halo. In some cases, an association with a fast flow, arteriovenous malformation (AVM) can be identified. Here, we describe a case report of a 16-year-old woman with CM-AVM syndrome and significant cardiac compromise successfully treated with trametinib, a mitogen-activated protein kinase (MEK) inhibitor.

Quantitative measurement of skin surface oiliness and shine using differential polarized images.

Excess amounts of skin surface oil can lead to adverse psychological consequences. Grease-spot photometry-based techniques measure sebum production rate. However, besides being tedious, these measurements are influenced by contact area, applied pressure, and time of application. Image analysis of polarized images has the potential to provide objective, quantitative information of skin oiliness. This study was designed to set up an imaging device for capturing and enhancing the changes in skin surface oiliness and to clinically and quantitatively, (via image analysis), evaluate varying levels of skin surface oiliness. Mineral oil was used to simulate skin surface oil. 40.5 µL of the mineral oil was applied within a two inch square area of interest on facial skin in twelve steps, from 1 to 40.5 µL, at 40% increments. The results indicate a strong correlation between the quantitative skin surface oiliness measurements and the clinical assessments. This sensitive technique has the potential to be utilized in future studies to evaluate product efficacies in reducing skin oiliness.

Trichloroacetic acid model to accurately capture the efficacy of treatments for postinflammatory hyperpigmentation.

Postinflammatory hyperpigmentation (PIH) occurs following cutaneous injury and is common following resolution of acne especially in patients with skin of color. The objective of this study was to further validate a trichloroacetic acid (TCA)-induced PIH model and compare it to acne-induced PIH using topical bakuchiol, a botanical extract that has been shown to have antimicrobial, anti-inflammatory, antioxidant, and antiacne properties. A prospective, non-randomized clinical trial was conducted on subjects with skin phototypes IV-VI with a history of acne-induced PIH. Subjects applied bakuchiol or vehicle cream twice daily to 2 acne-induced and 2 TCA-induced PIH lesions for 28 days with a third lesion serving as a control in each group. Degree of improvement was defined as the change in the Investigator Global Assessment (IGA) score over 28 days of treatment. Twenty subjects (6 males, 14 females) completed the study. For TCA-induced PIH sites, there was a statistically significant (p < 0.05) degree of improvement with bakuchiol treatment (- 0.50 ± 0.18) compared to vehicle (0.05 ± 0.15) and control (- 0.06 ± 0.17). For acne-induced PIH, there was a greater degree of improvement for bakuchiol (- 1.06 ± 0.23) when compared to vehicle (- 0.56 ± 0.16) and control (- 0.69 ± 0.18); however, statistical significance was not reached (p > 0.05). TCA-induced PIH sites were uniform in size and pigment intensity thereby allowing better comparison among sites. This emphasizes the relevance of using this model for PIH which may help reduce the barriers in clinical trials and help improve access to treatments for patients who suffer from PIH. The results suggest that topical bakuchiol may decrease the severity of PIH.

Emerging medical treatments for hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic inflammatory disease affecting intertriginous skin areas, and it is characterized by recurrent painful episodes of inflammatory drainage. Although the pathophysiology of HS is not fully understood, recent research points to an imbalance of cytokines as a contributing factor to the associated symptoms of purulent drainage and sinus tract formation. HS lesions are often characterized by a superimposed pathogenic/commensal bacterial infection that can improve with targeted antibiotic therapy. New medical treatments have emerged in recent years, many of which specifically work against a variety of proinflammatory mediators associated with HS. These newer, specified treatment options, in conjunction with surgery and lasers, are thought to provide positive outcomes and an overall improvement in quality of life in patients with HS.

Greater efficacy of SPF 100+ sunscreen compared with SPF 50+ in sunburn prevention during 5 consecutive days of sunlight exposure: A randomized, double-blind clinical trial.

BACKGROUND: Beach vacations are high-risk settings for overexposure to ultraviolet radiation. OBJECTIVE: To compare the sunburn protective efficacy of SPF 50+ and SPF 100+ sunscreens under actual use at the beach. METHODS: A prospective, randomized, double-blind, single-center, split-body/face study of 55 healthy individuals. Each participant applied both sunscreens to randomized sides of the face/body for up to 5 consecutive days. Blinded clinical evaluation of erythema by a single grader and objective instrumental assessments, colorimetry, and diffuse reflectance spectroscopy were performed the morning after each sun exposure. RESULTS: After 5 days, 31 (56%) participants had more sunburn on the SPF 50+ side compared to 4 (7%) on the SPF 100+ side. Overall, mean erythema intensity showed statistically significantly less erythema on the SPF 100+ side compared with the SPF 50+ side. The first observation of sunburn exclusively on the SPF 50+ side occurred after 1 day of sun exposure, whereas that for SPF 100+ occurred after 3 days of sun exposure. LIMITATIONS: Only initial sunscreen application was monitored, only 1 participant with skin phototype I was recruited, and participants were recruited from a local beach area. CONCLUSION: SPF 100+ was significantly more effective in protecting against ultraviolet radiation-induced erythema and sunburn than SPF 50+ in actual use in a beach vacation setting.

Oral Polypodium Leucotomos Extract and Its Impact on Visible Light-Induced Pigmentation in Human Subjects

BACKGROUND: Visible light (VL) has multiple effects on the skin that currently available sunscreens do not protect against. Polypodium leucotomos extract (PLE) has properties that may offer protection against VL. OBJECTIVES: To determine the effectiveness of PLE in preventing VL-induced effects. METHODS: Twenty-two subjects with Fitzpatrick skin phototype IV-VI were enrolled. On day 0, subjects were irradiated with VL. Clinical Investigator's Global Assessment (IGA) scoring and spectroscopic evaluations were performed immediately, 24 hours, and 7 days after irradiation. Subjects then received a 28-day supply of PLE (480 mg daily). Irradiation and evaluation were repeated. Three 4-mm punch biopsies were obtained for immunohistochemistry analysis: one from normal unirradiated skin and the other two twenty-four hours after irradiation, pre- and post-PLE, from sites irradiated with highest dose of VL. RESULTS: All subjects had immediate pigment darkening, persistent pigment darkening, and delayed tanning both pre- and post-PLE. For the highest VL dose (480 J/cm²) spectroscopic assessments demonstrated a statistically significant decrease in persistent pigment darkening and delayed tanning post-PLE. In addition, there was a significant decrease in cyclooxygenase-2, and a trend towards decreases in the markers for cellular damage post-PLE. While there was a trend towards lower IGA scores post-PLE, statistical significance was not reached possibly due to lack of sensitivity of the visual IGA scoring system in detecting small changes. CONCLUSIONS: Spectroscopic data and immunohistochemistry indicate an effect of PLE on visible light induced effects. As such, PLE may be used as an adjuvant to traditional means of photoprotection to protect against the effects of VL. Clinical trial registration number: NCT02904798. J Drugs Dermatol. 2019;18(12):1198-1203.

Spectral characteristics of visible light-induced pigmentation and visible light protection factor.

Solar radiation is a major contributor to the development of skin cancer. Recent studies have shown that visible light (VL), a major portion of solar spectrum, induces biologic effects on the skin. Ultraviolet filters in currently available broad-spectrum sunscreens do not offer protection against VL. This study was designed to identify the spectral characteristics of the skin responses induced by VL, which can be utilized for time efficient in vivo VL testing. Thirty-one subjects were irradiated with a light source emitting visible light with less than 0.5% long wavelength UVA1 (VL + UVA1, 370-700 nm), and 41 subjects were irradiated with pure visible light (pure VL, 400-700 nm). Assessments including clinical photography, investigator's global assessment of pigmentation and erythema, and diffuse reflectance spectroscopy (DRS) performed immediately and seven days after irradiation. Clinical and spectroscopic data showed that VL + UVA1 spectral output induced significantly darker and persistent skin responses as compared to those induced by pure VL. Spectroscopic signatures of skin responses induced by both radiation sources were identified. The signatures were found to be specific to the radiation source and time of collection. A method to evaluate VL protection factor, using quantitative information from the spectral signatures obtained, was proposed.

Ertapenem - a potent treatment for clinical and quality of life improvement in patients with hidradenitis suppurativa.

BACKGROUND: A paucity of knowledge exists regarding the use of ertapenem in hidradenitis suppurativa. Our retrospective chart review and telephone interview aims to investigate the utility of intravenous ertapenem in severe, refractory hidradenitis suppurativa. METHODS: This retrospective chart review and telephone interview included patients with severe, refractory hidradenitis suppurativa treated with intravenous ertapenem between March 2013 and December 2016. Data were obtained from medical charts. During the telephone interview, patients were asked questions relating to satisfaction, quality of life changes, and disease state changes with ertapenem therapy. RESULTS: A total of 36 patients including 22 females and 14 males with Hurley stage II or III hidradenitis suppurativa were included. Thirty-five patients (97.2%), demonstrated improvements in hidradenitis suppurativa with ertapenem treatment. In total, 28 patients participated in our telephone interview. Twenty patients (71.4%) were very satisfied (n = 12) or satisfied (n = 8). Quality of life improved in 85.7% of patients (n = 24). CONCLUSION: Following ertapenem therapy, patients reported improvements in quality of life. This treatment appears promising as an adjunct to biologics or as a bridge to surgery in the treatment of severe, refractory hidradenitis suppurativa.

Hidradenitis suppurativa in children: The Henry Ford experience.

BACKGROUND: Although recent hidradenitis suppurativa studies have shown that early-onset disease is associated with a positive family history and more widespread disease, research in pediatric hidradenitis suppurativa is limited. METHODS: Thirty-three children diagnosed with hidradenitis suppurativa during an 18-month period were included in this institutional review board-approved, retrospective chart review. Information on demographic characteristic, family history, and timing of onset (prepubescent vs postpubescent) was extracted. The Fisher exact test, Cochran-Armitage exact trend test, and chi-square test were used to examine the association between prepubescent or postpubescent onset of hidradenitis suppurativa and sex, disease severity, and family history. RESULTS: A significantly higher percentage of patients with postpubescent onset were female (85.7%) than male (14.3%), whereas those with prepubescent onset were more likely to be male (58.3%) than female (41.7%; P = .02). Associations between disease onset and positive family history of hidradenitis suppurativa (P = .47) or higher Hurley stage of disease (P = .15) were not statistically significant. CONCLUSION: Boys are more likely to have prepubescent onset of hidradenitis suppurativa and girls to have postpubescent onset. This shift in sex distribution is unexplained, but we hypothesize that, whereas the role of ovarian hormones in the pathogenesis of HS may underlie much of adult-onset disease, it is less important in prepubescent disease.

Hidradenitis suppurativa: an update on connecting the tracts.

Hidradenitis suppurativa (HS) is a devastating disease involving abscesses, sinus tracts, and inflammation classically affecting the axilla, groin, and/or anogenital region. Although the disease pathogenesis is not fully understood, recent advances suggest that HS pathology runs much deeper than the cutaneous manifestations. It is now believed that HS is a systemic inflammatory disease that gives rise to the characteristic cutaneous manifestations. This disease is problematic for both patients and physicians to manage because of a variety of diagnostic and management difficulties. This article seeks to provide updates on the current understanding of HS to increase awareness and improve management.

A retrospective review of light- and laser-based management of hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a chronic skin disease that is difficult to manage and often refractory to medical management. Light and laser therapies have been utilized in the field of dermatology to treat a variety of skin conditions, but more recently were studied in the management of HS. Usually performed as outpatient procedures with a low risk of complications, they offer several advantages to traditional cold knife surgical procedures. We reviewed the various light- and laser-based treatments studied in HS and compared their efficacy. Outcome measures were not standardized, which made comparison difficult. Studies performed were few in number and often limited by small patient samples. Larger, randomized controlled trials that compare these treatment modalities to medical and surgical management are needed. Despite their limitations, light- and laser-based therapies are promising treatment options for patients with refractory HS.