Discrepancies in hidradenitis suppurativa lesion characterization by providers and patients.

BACKGROUND: The hidradenitis suppurativa (HS) clinical response (HiSCR) has come under scrutiny as several HS clinical trials failed to meet primary endpoints with high placebo responses. This may be due to limitations of the tool and raters' ability to accurately characterize and count lesions, rather than lack of efficacy of the studied drug. Due to HS lesion complexity and potential differences in rater training, it was hypothesized that there would be discrepancies in how providers characterize and count lesions for HS clinical trials. OBJECTIVE: To evaluate how HS providers and patients name and count HS lesions and to identify discrepancies among providers to initiate the development of consensus-driven guidance for HS rater training. METHODS: An online survey was distributed to the members of HIdradenitis SuppuraTiva cORe outcomes set International Collaboration (HISTORIC). Respondents were asked to classify lesion images composed of multiple and different morphology types and answer questions regarding inclusion of associated dermatological conditions. RESULTS: Forty-seven HISTORIC members responded (29 providers; 18 patients). There was variability in how respondents classified HS lesions. Of 12 questions containing images, four had ≥50% of respondents choosing the same answer. With an image of a lesion composed of different morphologies, 45% of providers counted it as a single lesion and 45% counted it as multiple distinct lesions. With an image of multiple interconnected draining tunnels, 7% of providers classified it as a single draining tunnel while 79% categorized it as multiple draining tunnels with the number estimated by visual inspection. There was also variability in deciding whether lesions occurring in associated conditions should be considered separately or included in HS lesion counts. Patient responses were also variable. CONCLUSIONS: The result of the current study reaffirms the gap in how providers characterize and count HS lesions for clinical trials and the need to develop consensus-driven rater training related to HS outcome measures.

Systematic review of photodynamic therapy for the treatment of hidradenitis suppurativa.

OBJECTIVE: To perform a systematic review of available literature regarding the use of 5-aminolevulinic acid (ALA) and ALA derivative photodynamic therapy (PDT) in the treatment of hidradenitis suppurativa (HS) and provide recommendations on its use. METHODS: A systematic review was performed of all published studies up to September 1, 2019 from nine databases, including PubMed, that evaluated PDT in the treatment of HS. For each study, quality of evidence and risk of bias was evaluated. Recommendations from the body of evidence were created based on Strength of Recommendation and Taxonomy (SORT) criteria. RESULTS: Eighteen studies met inclusion criteria. The majority of studies had a high risk of bias. Blue light PDT with 20% ALA and red light PDT with 16% methyl aminolevulinate (MAL) demonstrated some benefit based on a small number of poor-quality studies with a high risk of bias (Grade C, level III evidence). The most promising results were for 1%-5% ALA with intralesional diode, with good to complete response in 78%-94% of anatomic sites treated (Grade B, level II evidence). LIMITATIONS: The majority of studies contained high levels of bias, with significant heterogeneity between studies. Conclusions are limited by small samples sizes, lack of randomized controlled trials, and differing protocols. CONCLUSION: Further studies are needed to determine the clinical efficacy of 20% ALA with blue light and MAL with red light. Intralesional diode PDT shows the most promise and warrants further investigation in larger, randomized controlled trials.

Development and validation of IHS4-55, an IHS4 dichotomous outcome to assess treatment effect for hidradenitis suppurativa.

BACKGROUND: Validated, inclusive and easy-to-use outcomes for hidradenitis suppurativa are essential both in the clinical trial setting and clinical practice. The continuous IHS4 is a validated tool that dynamically assesses nodules/abscesses/draining tunnels and classifies disease severity as mild/moderate/severe. However, dichotomous outcomes are often required for clinical trials reporting. OBJECTIVE: To develop and validate a dichotomous outcome based on IHS4 that can be used in clinical trial settings and day-to-day clinical practice. METHODS: De-identified data from the PIONEER-I and -II studies were accessed through Vivli. Potential IHS4 thresholds were analysed using baseline to Week 12 data from adalimumab- and placebo-treated hidradenitis suppurativa patients in the PIONEER-I trial. The final threshold was chosen based on its ability to discriminate between patients treated with adalimumab or placebo and its association with reduction in inflammatory lesions. The final threshold was validated using data from baseline to Week 12 from adalimumab- and placebo-treated hidradenitis suppurativa patients in both the PIONEER-II and the combined PIONEER-I and -II studies. RESULTS: The best performing cut-off for the IHS4 was a 55% reduction of the IHS4 score (IHS4-55). Patients who achieved the IHS4-55 had an odd's ratio of 2.00 [95%-CI 1.26-3.18, p = 0.003], 2.79 (95%-CI 1.76-4.43, p < 0.001) and 2.16 (95%-CI 1.43-3.29, p < 0.001) for being treated with adalimumab rather than placebo in PIONEER-I, PIONEER-II and the combined dataset, respectively. Additionally, the achievement of the IHS4-55 was associated with a significant reduction in inflammatory nodules, abscesses and draining tunnels in all analysed datasets. CONCLUSIONS: IHS4-55, a novel dichotomous IHS4 version, based on a 55% reduction of the total score was developed. The IHS4-55 performs similarly to the HiSCR in discriminating between adalimumab- and placebo-treated hidradenitis suppurativa patients and shows significant associations with reductions in lesion counts. Moreover, the IHS4-55 addresses some of the HiSCR drawbacks by dynamically including draining tunnels in a validated manner. By allowing the analysis of hidradenitis suppurativa patients with an abscess and nodule count below 3 but many draining tunnels, this outcome measure will improve inclusivity in clinical trials.

A case series of hydroxychloroquine exacerbating the dermatomyositis rash.

Hydroxychloroquine (HCQ) is an antimalarial agent that is commonly used in the management of rheumatic skin disease. Few reports exist documenting exacerbation of dermatomyositis (DM) related to HCQ. Herein, we describe three adult patients with worsening DM cutaneous disease after starting HCQ and resolution or improvement with cessation. The time to exacerbation ranged from two weeks to nine months after the initiation of HCQ 400mg/day. Two of the three patients had antibodies to transcription intermediary factor 1γ (TIF1γ) and the other had antibodies to anti-nuclear matrix protein 2 (NXP2). After discontinuation of HCQ, the time to improvement or resolution of cutaneous symptoms ranged from six weeks to six months. Hydroxychloroquine may be associated with worsening cutaneous features in DM. In patients who are not improving despite escalation of immunosuppressive medications, or are worsening, we recommend a trial of discontinuing HCQ.

A national survey comparing practice patterns and residency training satisfaction for categorical dermatology versus combined internal medicine and dermatology trained physicians.

Combined internal medicine and dermatology (med-derm) training programs were created to advance complex medical dermatology and inpatient dermatology care. A prior study demonstrated that compared to categorical dermatology residents, med-derm residents had less program satisfaction, yet indicated a stronger desire to pursue careers in academia. No follow-up data on practice patterns after training has been reported. We aimed to characterize differences in residency program satisfaction and practice patterns between physicians trained in categorical dermatology compared to med-derm residency programs. We surveyed physicians who graduated from combined med-derm programs along with their counterparts, from six institutions, that either currently or historically had a combined med-derm training, from 2008-2017. Fifty-five percent of med-derm and forty-one percent of categorical-trained physicians responded. The practice patterns between the two groups were similar. A quarter of med-derm physicians continued to provide general internal medicine services. Categorical trained physicians were significantly more satisfied with their training (P=0.03) and performed more excisions on the head/neck (P=0.02). The combined graduates had significantly greater confidence in multidisciplinary care (P=0.003), prescribed more biologic (P<0.001) and non-biologic immunosuppressive agents (P=0.002), and volunteered more for the underserved patients in their communities (P=0.04). Although few differences in overall practice patterns between categorical and med-derm trained graduates were appreciated, med-derm graduates seem more comfortable with multidisciplinary care and may care for more medically complex patients requiring immunosuppression.

Factors associated with treatment satisfaction in patients with hidradenitis suppurativa: results from the Global VOICE project.

BACKGROUND: Nearly half of patients with hidradenitis suppurativa (HS) report dissatisfaction with their treatment. However, factors related to treatment satisfaction have not been explored. OBJECTIVES: To measure associations between treatment satisfaction and clinical and treatment-related characteristics among patients with HS. METHODS: Treatment satisfaction was evaluated utilizing data from a cross-sectional global survey of patients with HS recruited from 27 institutions, mainly HS referral centres, in 14 different countries from October 2017 to July 2018. The primary outcome was patients' self-reported overall satisfaction with their current treatments for HS, rated on a five-point scale from 'very dissatisfied' to 'very satisfied'. RESULTS: The final analysis cohort comprised 1418 patients with HS, most of whom were European (55%, 780 of 1418) or North American (38%, 542 of 1418), and female (85%, 1210 of 1418). Overall, 45% (640 of 1418) of participants were either dissatisfied or very dissatisfied with their current medical treatment. In adjusted analysis, patients primarily treated by a dermatologist for HS had 1·99 [95% confidence interval (CI) 1·62-2·44, P < 0·001] times the odds of being satisfied with current treatment than participants not primarily treated by a dermatologist. Treatment with biologics was associated with higher satisfaction [odds ratio (OR) 2·36, 95% CI 1·74-3·19, P < 0·001] relative to treatment with nonbiologic systemic medications. Factors associated with lower treatment satisfaction included smoking (OR 0·78, 95% CI 0·62-0·99; active vs. never), depression (OR 0·69, 95% CI 0·54-0·87), increasing number of comorbidities (OR 0·88 per comorbidity, 95% CI 0·81-0·96) and increasing flare frequency. CONCLUSIONS: There are several factors that appear to positively influence satisfaction with treatment among patients with HS, including treatment by a dermatologist and treatment with a biologic medication. Factors that appear to lower treatment satisfaction include active smoking, depression, accumulation of comorbid conditions and increasing flare frequency. Awareness of these factors may support partnered decision making with the goal of improving treatment outcomes. What is already known about this topic? Nearly half of patients with hidradenitis suppurativa report dissatisfaction with their treatments. What does this study add? Satisfaction with treatment is increased by receiving care from a dermatologist and treatment with biologics. Satisfaction with treatment is decreased by tobacco smoking, accumulation of comorbid conditions including depression, and higher flare frequency. What are the clinical implications of this work? Awareness of the identified factors associated with poor treatment satisfaction may support partnered decision making and improve treatment outcomes.

Comorbidity screening in hidradenitis suppurativa: Evidence-based recommendations from the US and Canadian Hidradenitis Suppurativa Foundations.

BACKGROUND: Hidradenitis suppurativa (HS) is associated with comorbidities that contribute to poor health, impaired life quality, and mortality risk. OBJECTIVE: To provide evidence-based screening recommendations for comorbidities linked to HS. METHODS: Systematic reviews were performed to summarize evidence on the prevalence and incidence of 30 comorbidities in patients with HS relative to the general population. The screening recommendation for each comorbidity was informed by the consistency and quality of existing studies, disease prevalence, and magnitude of association, as well as benefits, harms, and feasibility of screening. The level of evidence and strength of corresponding screening recommendation were graded by using the Strength of Recommendation Taxonomy (SORT) criteria. RESULTS: Screening is recommended for the following comorbidities: acne, dissecting cellulitis of the scalp, pilonidal disease, pyoderma gangrenosum, depression, generalized anxiety disorder, suicide, smoking, substance use disorder, polycystic ovary syndrome, obesity, dyslipidemia, diabetes mellitus, metabolic syndrome, hypertension, cardiovascular disease, inflammatory bowel disease, spondyloarthritis, and sexual dysfunction. It is also recommended to screen patients with Down syndrome for HS. The decision to screen for specific comorbidities may vary with patient risk factors. The role of the dermatologist in screening varies according to comorbidity. LIMITATIONS: Screening recommendations represent one component of a comprehensive care strategy. CONCLUSIONS: Dermatologists should support screening efforts to identify comorbid conditions in HS.

The Use of the Video, "Dear 16-Year-Old Me," as a Melanoma Education Tool in Ambulatory Dermatology.

BACKGROUND: Skin cancer continues to be the most common cancer in the United States. The rise of social media platforms and internet use offers an opportunity to present health information through video-based education. The video "Dear 16-Year-OldMe," addresses the risks associated with tanning and sun exposure, the importance of practicing sun protection, and shares stories from melanoma survivors. OBJECTIVE: To evaluate the video "Dear 16-Year-Old Me," as a patient education tool in dermatology clinics and to investigate whether viewing a short educational video can change knowledge about skin cancer risks and intention to improve skin cancer prevention behavior. PATIENTS AND METHODS/MATERIALS AND METHODS/METHODS: English-speaking clinic patients between the ages of 14 to 45 years old were recruited. Exclusion criteria include both a personal or family history of skin cancer, dysplastic nevi, or other medical comorbidities. Forty-five participants agreed to participate; 38 were eligible for analysis. RESULTS: Comparison of prevideo and postvideo responses demonstrated a statistically significant reduction in participants reporting they were likely to tan outdoors (p-value = .001). A significant increase was observed in the reported likelihood to have a professional skin examination (p-value < .001) or self-examination (p-value < .001) in the future. CONCLUSION: and Relevance: Viewing "Dear 16-Year-Old Me," resulted in reported participant changes in intention to tan outdoors and participate in skin surveillance. Although these are encouraging results, future studies with a comparison group are needed to elucidate whether these results correspond to changes in behavior. In the age of viral videos and readily accessible health information via the internet, continued investigation of video media on patient health behaviors should be pursued.

Evaluating patients' unmet needs in hidradenitis suppurativa: Results from the Global Survey Of Impact and Healthcare Needs (VOICE) Project.

BACKGROUND: A needs assessment for patients with hidradenitis suppurativa (HS) will support advancements in multidisciplinary care, treatment, research, advocacy, and philanthropy. OBJECTIVE: To evaluate unmet needs from the perspective of HS patients. METHODS: Prospective multinational survey of patients between October 2017 and July 2018. RESULTS: Before receiving a formal HS diagnosis, 63.7% (n = 827) of patients visited a physician ≥5 times. Mean delay in diagnosis was 10.2 ± 8.9 years. Patients experienced flare daily, weekly, or monthly in 23.0%, 29.8%, and 31.1%, respectively. Most (61.4% [n = 798]) rated recent HS-related pain as moderate or higher, and 4.5% described recent pain to be the worst possible. Access to dermatology was rated as difficult by 37.0% (n = 481). Patients reported visiting the emergency department and hospital ≥5 times for symptoms in 18.3% and 12.5%, respectively. An extreme impact on life was reported by 43.3% (n = 563), and 14.5% were disabled due to disease. Patients reported a high frequency of comorbidities, most commonly mood disorders. Patients were dissatisfied with medical or procedural treatments in 45.9% and 34.6%, respectively. LIMITATIONS: Data were self-reported. Patients with more severe disease may have been selected. CONCLUSION: HS patients have identified several critical unmet needs that will require stakeholder collaboration to meaningfully address.

Programmed cell death protein 1 inhibitor-induced recalcitrant mixed small and medium vessel vasculitis.

Pembrolizumab, a programmed cell death protein 1 (PD1) inhibitor, has been known to be associated with several adverse reactions, including immune related adverse events. In less than one percent of patients, PD1 inhibitors have been linked to the development of connective tissue disease. Patients with previously known connective tissue disease are hypothesized to be at increased risk of flares in as many as 40% of cases. A 70-year-old man with a past medical history significant for rheumatoid arthritis in remission and stage IV lung adenocarcinoma presented to the dermatology clinic after one cycle of nivolumab and eight cycles of pembrolizumab exhibiting worsening, painful bilateral lower extremity ulcers for approximately one month. On the lower legs, three large black retiform eschars and bullous purpuric plaques were observed. Vasculitis is a rare complication of PD1 inhibitor therapy, with the majority of cases reported in literature either medium vessel or large vessel vasculitis. Only glucocorticoids have proven effective for PD1-induced vasculitis and these patients generally require multi-specialty management.

A perplexing case of superficial granulomatous pyoderma with sporotrichoid-like distribution.

Superficial granulomatous pyoderma (SGP) is a rare pyoderma gangrenosum (PG) variant that differs from classic PG in that the ulcers tend to be more superficial, lack a rapidly advancing border, and are not typically associated with an underlying systemic disease. The ulcers are most commonly painless and located on the trunk, with a clean granulating base. They generally do not show undermining but may have a vegetative border. Lesions usually respond well to either topical or intralesional corticosteroids with complete healing. The classic histopathologic finding is a "three-layer granuloma" in the superficial dermis consisting of central neutrophilic inflammation and necrosis, a surrounding layer of histiocytes and multinucleated giant cells, and an outer most layer of plasma cells and eosinophils. Herein, we present a unique case of SGP with sporotrichoid-like distribution on the lower extremity.

Acquired partial lipodystrophy treated with poly-L-lactic acid and hyaluronic acid fillers: a case report.

Acquired partial lipodystrophy (APL), also known as Barraquer-Simons syndrome, is a rare disorder characterized by progressive fat loss in the upper body. Use of poly-L-lactic acid and hyaluronic acid (HA) fillers for the treatment of APL is neither approved by the Food and Drug Administration nor described in the literature. Herein, we describe a case of APL that achieved significant improvement in facial volume following treatment with combination poly-L-lactic acid and HA fillers.

Dermoscopy findings of a one mm lentigo maligna.

A 66-year-old man presented with a grayish 1mm macule on the left cheek. Dermatoscopic examination revealed annular-granular structures partially surrounding a single follicular ostium. Histopathologic examination demonstrated atypical, confluent single melanocytes at the basal layer with nesting, crowding, and upward migration descending along the hair follicle, consistent with the diagnosis of lentigo maligna (LM). This case presents evidence in support of the Schiffner progression model for LMs, starting with asymmetric pigmented follicular openings composed of annular-granular structures, which later coalesce into gray to dark black/brown streaks, and then form pseudo-networks or rhomboidal structures. The finding of annular-granular structures partially surrounding a single hair follicle may be the earliest recognizable dermatoscopic feature of an LM and may help clinicians diagnose facial LMs earlier.