Multimorbidity in Psoriasis as a Risk Factor for Psoriatic Arthritis: A Population-Based Study.

OBJECTIVES: To examine multimorbidity in psoriasis and its association with the development of PsA. METHODS: A retrospective cohort study was performed using the Rochester Epidemiology Project. Population-based incidence (2000-2009) and prevalence (Jan 1, 2010) cohorts of psoriasis were identified by manual chart review. A cohort of individuals without psoriasis (comparators) were identified (1:1 matched on age, sex, and county). Morbidities were defined using ≥2 Clinical Classification Software codes ≥30 days apart within prior five years. PsA was defined using ClASsification of Psoriatic ARthritis (CASPAR) criteria. χ2 and rank-sum tests were used to compare morbidities, and age-, sex-, and race-adjusted Cox models to examine the association of baseline morbidities in psoriasis with development of PsA. RESULTS: Among 817 incident psoriasis patients, the mean age was 45.2 years with 52.0% females, and 82.0% moderate/severe psoriasis. No multimorbidity differences were found between incident psoriasis patients and comparators. However, in the 1,088 prevalent psoriasis patients, multimorbidity was significantly more common compared with 1,086 comparators (OR : 1.35 and OR : 1.48 for ≥2 and ≥5 morbidities, respectively). Over a median 13.3-year follow-up, 23 patients (cumulative incidence: 2.9% by 15 years) developed PsA. Multimorbidity (≥2 morbidities) was associated with a 3-fold higher risk of developing PsA. CONCLUSION: Multimorbidity was more common in the prevalent but not incident cohort of psoriasis compared with the general population, suggesting patients with psoriasis may experience accelerated development of multimorbidity. Moreover, multimorbidity at psoriasis onset significantly increased the risk of developing PsA, highlighting the importance of monitoring multimorbid psoriasis patients for the development of PsA.

Guidelines of care for the management of acne vulgaris.

BACKGROUND: Acne vulgaris commonly affects adults, adolescents, and preadolescents aged 9 years or older. OBJECTIVE: The objective of this study was to provide evidence-based recommendations for the management of acne. METHODS: A work group conducted a systematic review and applied the Grading of Recommendations, Assessment, Development, and Evaluation approach for assessing the certainty of evidence and formulating and grading recommendations. RESULTS: This guideline presents 18 evidence-based recommendations and 5 good practice statements. Strong recommendations are made for benzoyl peroxide, topical retinoids, topical antibiotics, and oral doxycycline. Oral isotretinoin is strongly recommended for acne that is severe, causing psychosocial burden or scarring, or failing standard oral or topical therapy. Conditional recommendations are made for topical clascoterone, salicylic acid, and azelaic acid, as well as for oral minocycline, sarecycline, combined oral contraceptive pills, and spironolactone. Combining topical therapies with multiple mechanisms of action, limiting systemic antibiotic use, combining systemic antibiotics with topical therapies, and adding intralesional corticosteroid injections for larger acne lesions are recommended as good practice statements. LIMITATIONS: Analysis is based on the best available evidence at the time of the systematic review. CONCLUSIONS: These guidelines provide evidence-based recommendations for the management of acne vulgaris.

Prevalence and spectrum of infectious and inflammatory dermatologic conditions occurring in pediatric heart transplant patients on a predominantly mTOR-based immune suppressive regimen: A retrospective chart review.

INTRODUCTION: Pediatric heart transplant patients are routinely followed in dermatology clinics due to elevated risk of cutaneous malignancy. However, transplant patients may experience other, non-cancer-related dermatologic conditions including skin infections, inflammatory diseases, and drug eruptions that can cause significant medical and psychosocial comorbidity. METHODS: A retrospective chart review of all pediatric heart transplant patients at Mayo Clinic Children's Center in Rochester, MN, was performed to determine the prevalence and spectrum of non-cancer dermatologic conditions. Statistical analysis was conducted to look for associations between episodes of rejection and skin condition development. RESULTS: Of the 65 patients who received heart transplants under the age of 18 and were followed at Mayo Clinic, 69% (N = 45) were diagnosed with at least one skin condition between transplant and the time of most recent follow-up. Sixty-two percent (N = 40) of patients were diagnosed with an inflammatory skin condition (most commonly acne and atopic dermatitis), 45% (N = 29) with an infectious skin condition (most commonly warts and dermatophyte infection), and 32% (N = 21) with a drug eruption (most commonly unspecified rash and urticaria). No association was found between presence of skin disease and number of rejection episodes. CONCLUSIONS: Non-cancer dermatologic conditions are prevalent within pediatric heart transplant recipients and may directly impact their medical needs and quality of life. Dermatologist involvement in the care of post-transplant pediatric patients is important, not only for cancer screening but also for diagnosis and treatment of common infectious and inflammatory skin conditions.

Pathogenesis-directed treatment of linear porokeratosis with topical cholesterol-lovastatin.

A 2-year-old boy presented with an extensive, asymptomatic, photosensitive eruption refractory to topical steroids and tretinoin; examination and biopsies were consistent with generalized linear porokeratosis involving the face, limbs, and trunk. Treatment with topical cholesterol-lovastatin was initiated, and it successfully improved early erythematous lesions. Whole exome sequencing that targeted mevalonate pathway genes crucial in cholesterol synthesis later revealed a pathogenic, paternally inherited, porokeratosis-associated MVD, c.70+5 G>A, mutation. Topical cholesterol-lovastatin is a safe and effective empiric treatment for porokeratosis when used in the early, erythematous phase, and its success is likely mediated through its role in targeting mevalonate pathway mutations.

Complex cutaneous leishmaniasis in a pediatric patient.

Cutaneous leishmaniasis (CL), a parasitic infection caused by Leishmania protozoa and transmitted by sandfly bites, can be classified into Old World and New World subtypes. We report a case of a 2-year-old female who developed complex CL after travel to Panama. Ultimately, successful treatment required two rounds of liposomal amphotericin B. We report this case for its challenging clinical course and management.

Neonatal Sweet syndrome with associated rectovestibular fistula and review of the literature.

An otherwise healthy 4-week-old term female of Japanese heritage presented with a 1-week history of asymptomatic progressive, generalized skin lesions. The lesion morphology, distribution, and dermatopathology result was consistent with Sweet syndrome. The patient was found to have a congenital type H rectovestibular fistula. This case highlights the rare association of rectovestibular fistula in neonatal Sweet syndrome which has only been described in neonates of Japanese heritage.

Dermatologic manifestations in pediatric patients with inflammatory bowel disease.

BACKGROUND: Despite studies of dermatologic manifestations in adults with inflammatory bowel disease (IBD), little is known about the prevalence of IBD-associated skin lesions and their correlation with IBD severity in children. We aimed to address these knowledge gaps in our single-center cohort of children with IBD. METHODS: Retrospective chart review of 528 children and adolescents (≤18 years old) with IBD and seen at Mayo Clinic (Rochester, MN) between 1999 and 2017 was conducted. The Chi-Square/Fischer's exact test (with p ≤ .05 to signify statistical significance) was applied to compare categorical outcomes between Crohn's disease (CD) and ulcerative colitis (UC) patients. RESULTS: In total, 425 IBD patients (64.9% CD, 53% males) and ≥1 dermatologic diagnosis were included. Presence of ≥1 cutaneous infection was recorded in 42.8% of participants. Acne was the most common non-infectious dermatologic condition (30.8%), followed by eczema (15.8%) and perianal skin tags (14.6%). Angular cheilitis (p = .024), keratosis pilaris (KP, p = .003), and perianal skin complications (i.e., skin tags, fistula, and abscesses; all p < .001) were more frequently diagnosed among children with CD, while fungal skin infections (p = .017) were more frequently diagnosed in UC patients. Severity of IBD correlated with higher prevalence of perianal fistula (p = .003), perianal abscess (p = .041), psoriasis (p < .001), and pyoderma gangrenosum (PG, p = .003). CONCLUSIONS: Both IBD-specific and IBD-nonspecific dermatologic conditions are very prevalent in childhood IBD, the most common being infectious. Children with CD are more likely to experience angular cheilitis, KP, and perianal skin findings than those with UC. Perianal disease, psoriasis, and PG are associated with more severe IBD.

Idiopathic eruptive macular pigmentation in a pediatric patient and a review of the literature.

Idiopathic eruptive macular pigmentation (IEMP) is a rare, benign, self-resolving melanosis consisting of hyperpigmented macules typically on the face, trunk, and extremities that can occur in children and adolescents and often presents a diagnostic conundrum. We report a case involving an 8-year-old female whose previous clinical presentation was concerning for an atypical presentation of cutaneous mastocytosis or neurofibromatosis. The clinical and histopathologic evaluation was consistent with the diagnosis of IEMP, and no active intervention was pursued. Our accompanying literature review serves to better characterize this condition, highlight key diagnostic features, and emphasize the tendency for spontaneous resolution to avoid unnecessary treatments with limited clinical efficacy.

Bridging the gap in BASCULE syndrome: A retrospective case series of a recently described clinical entity.

BACKGROUND: Bier anemic spots, cyanosis with urticaria-like eruption (BASCULE) syndrome is a recently described entity with episodic urticarial lesions and white anemic halos on a background of erythrocyanosis, commonly affecting the lower extremities. Possible association with autonomic dysfunction remains poorly understood. Existing publications are limited, but the condition is suggested as highly underrecognized. OBJECTIVE: To further characterize clinical and epidemiologic data for BASCULE syndrome. METHODS: We performed an IRB-approved retrospective chart review on patients with BASCULE syndrome evaluated at Mayo Clinic from April 2021 to November 2022. RESULTS: A total of 17 patients were identified (13 female, 4 male). Median age of onset was 12 years (range 9-17). Lower extremities were involved in all patients (17). Most patients were symptomatic with pruritus (8) or burning pain (8); three were asymptomatic. Triggers were standing (11), hot showers or hot environments (7), or no clear trigger (4). Autonomic dysfunction was present in 10 patients. Treatment responses were observed from propranolol (3) and high-dose cetirizine (1). CONCLUSION: Novel epidemiologic data from 17 pediatric and young adult patients with BASCULE syndrome further supports an association with autonomic dysfunction and suggests a higher prevalence than previously acknowledged.

A novel rapamycin cream formulation improves facial angiofibromas associated with tuberous sclerosis complex: a double-blind randomized placebo-controlled trial.

BACKGROUND: Facial angiofibromas (FAs) are a major feature of tuberous sclerosis complex (TSC). Topical rapamycin can successfully treat FAs. A new stabilized cream formulation that protects rapamycin from oxidation has been developed in 0.5% and 1% concentrations. OBJECTIVES: To assess the efficacy and safety of a novel, stabilized topical rapamycin cream formulation. METHODS: This multicentre double-blind randomized placebo-controlled dose-response phase II/III study with a parallel design included participants aged 6-65 years with FAs of mild or moderate severity according to the Investigator's Global Assessment (IGA) scale. Participants were randomized to one of three treatment arms: topical rapamycin 0.5%, topical rapamycin 1% or placebo. Treatment was applied once daily for 26 weeks. Safety and efficacy measures were assessed at days 14, 56, 98, 140 and 182. The primary endpoint was the percentage of participants achieving IGA scores of 'clear' or 'almost clear' after 26 weeks of treatment. Secondary measures included Facial Angiofibroma Severity Index (FASI) and participant- and clinician-reported percentage-based improvement. Safety measures included the incidence of treatment-emergent adverse events and blood rapamycin concentration changes over time. RESULTS: Participants (n = 107) were randomized to receive either rapamycin 1% (n = 33), rapamycin 0.5% (n = 36) or placebo (n = 38). All treated participants were included in the final analysis. The percentage of participants with a two-grade IGA improvement was greater in the rapamycin 0.5% treatment group (11%) and rapamycin 1% group (9%) than in the placebo group (5%). However, this was not statistically significant [rapamycin 0.5%: odds ratio (OR) 1.71, 95% confidence interval (CI) 0.36-8.18 (P = 0.50); rapamycin 1%: OR 1.68, 95% CI 0.33-8.40 (P = 0.53)]. There was a statistically significant difference in the proportion of participants treated with rapamycin cream that achieved at least a one-grade improvement in IGA [rapamycin 0.5%: 56% (OR 4.73, 95% CI 1.59-14.10; P = 0.005); rapamycin 1%: 61% (OR 5.14, 95% CI 1.70-15.57; P = 0.004); placebo: 24%]. Skin adverse reactions were more common in patients following rapamycin application (64%) vs. placebo (29%). CONCLUSIONS: Both rapamycin cream formulations (0.5% and 1%) were well tolerated, and either strength could lead to clinical benefit in the treatment of FA.

Percutaneous MR Imaging-Guided Laser Ablation for the Treatment of Symptomatic Cervicofacial Vascular Malformations.

PURPOSE: To evaluate the safety and effectiveness of percutaneous magnetic resonance (MR) imaging-guided laser ablation for the treatment of symptomatic soft tissue vascular malformations (VMs) in the face and neck. MATERIALS AND METHODS: An institutional review board-approved retrospective review was undertaken of all consecutive patients who underwent MR imaging-guided and monitored laser ablation for treatment of symptomatic, cervicofacial soft tissue VM. Preablation and postablation MR imaging findings were independently reviewed. Preablation and postablation VM sizes were documented. Preablation T2 signal characteristics and enhancement patterns as well as postablation change in both signal and enhancement were semiquantitatively assessed. Changes in VM size were compared using a paired t test. RESULTS: Thirteen patients (women, 9; age, 14.5-69.5 years) with 13 VMs were treated for moderate-to-severe pain (n = 4), swelling/mass effect (n = 8), or predominantly cosmesis (n = 1) with 22 total ablation sessions. The baseline maximum VM diameter was 5.7 cm ± 4.2. At baseline, all VMs (100%) demonstrated variable T2-weighted signal hyperintensity and enhancement. For painful VM, the baseline pain score was 8 ± 1. Clinical follow-up was available for 10 patients. Of patients with available follow-up, 3 (100%) treated for moderate-to-severe pain and 7 (100%) treated for swelling/mass effect reported subjective complete or partial symptomatic relief. The patient treated predominantly for cosmetic reasons was lost to follow-up. Two patients (15.4%) experienced minor adverse events by the Society of Interventional Radiology standards. There were no major adverse events. CONCLUSIONS: MR imaging-guided and monitored percutaneous laser ablation is safe and effective for the treatment of symptomatic, cervicofacial VMs.

Response to sirolimus in capillary lymphatic venous malformations and associated syndromes: Impact on symptomatology, quality of life, and radiographic response.

BACKGROUND: Capillary lymphatic venous malformations (CLVM) and associated syndromes, including Klippel-Trenaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformation, epidermal nevi, skeletal, and spinal syndrome (CLOVES), are underrecognized disorders associated with high morbidity from chronic pain, recurrent infections, bleeding, and clotting complications. The rarity of these disorders and heterogeneity of clinical presentations make large-scale randomized clinical drug trials challenging. Identification of PIK3CA (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha [gene]) mutations in CLVM has made targeted medications, such as sirolimus, attractive treatment options. The aim of this study was to investigate the safety and efficacy of sirolimus therapy in CLVM. PROCEDURE: A combined prospective and retrospective cohort of pediatric and young adult patients with CLVM treated with sirolimus was evaluated for disease response, including symptom improvement, quality of life (QOL), and radiologic response. Sirolimus dosing regimens and toxicities were also assessed. RESULTS: Twenty-nine patients with CLVM, including KTS and CLOVES, were included. Ninety-three percent of patients reported improved QOL, and 86% had improvement in at least one symptom. Most significantly, improvement was noted in 100% of patients with bleeding and 89% with thrombotic complications with corresponding decreases in mean D-dimer (p = .008) and increases in mean fibrinogen (p = .016). No patients had progressive disease on sirolimus. Most common side effects included neutropenia, lymphopenia, infection, and aphthous ulcers/stomatitis. No toxicities were life-threatening, and none required long-term discontinuation of sirolimus. CONCLUSION: Sirolimus appears to be effective at reducing complications and improving QOL in patients with CLVM and associated syndromes. In this patient cohort, sirolimus was well tolerated and resulted in few treatment-related toxicities.

Manifestation of anxiety and depression among pediatric patients with psoriasis: A review.

Psoriasis is a chronic, inflammatory skin disorder with manifestations extending beyond visual presentation to encompass psychological burden. Though the association between psoriasis and mental health disorders in adults is widely accepted, there is inadequate research to generalize these findings to pediatric populations. In this review of the literature, we discuss the prevalence of anxiety and depression among pediatric patients with psoriasis in hopes of raising awareness of these associations and advocating for psychological screening and intervention in dermatology practice.

Acne incidence in preadolescents and association with increased body mass index: A population-based retrospective cohort study of 643 cases with age- and sex-matched community controls.

BACKGROUND/OBJECTIVES: Little is known about acne incidence in preadolescents and its potential association with body mass index (BMI). Our study aims to determine acne incidence in preadolescents and its association with BMI. METHODS: A population-based retrospective cohort study identified 7- to ≤12 year-olds with an initial acne diagnosis during 2010-2018, and incidence was calculated. Two age- and sex-matched controls without acne were randomly selected per case, and BMI was recorded. RESULTS: A total of 643 acne patients were identified. Annual age- and sex-adjusted incidence rate was 58.0 per 10,000 person-years, higher in females vs. males (89.2 vs. 28.2 per 10,000 person-years, p < .001), and increased with age (4.3, 24.4, and 144.3 per 10,000 person-years among 7-8, 9-10, and 11-12 year-olds, respectively, p < .001). Systemic medication use was associated with increasing BMI (odds ratio = 1.43 per 5 kg/m2 increase in BMI, 95% CI 1.07-1.92, p = .015). Median BMI percentile was higher among acne cases vs. controls (75.0 vs. 65.0, p < .001), as was the proportion with BMI ≥95th percentile (16.7% vs. 12.2%, p = .01). CONCLUSION: Acne incidence is higher in preadolescent girls than boys and increases with age. Preadolescents with acne are more likely to be obese than those without acne. Those with higher BMIs are more likely to be given systemic treatment.

The impact of childhood psoriasis on the quality of life of parents and caregivers.

We used the Psoriasis Caregiver Impact Scale to explore the quality of life (QoL) of parents/caregivers of children with psoriasis. We found that the QoL of parents of children with psoriasis is negatively affected in numerous domains including family and social life, emotional health, work, activities, and finances.

Infantile perianal pyramidal protrusion: A retrospective review of 27 patients.

BACKGROUND: Infantile perianal pyramidal protrusion (IPPP) is a rare benign skin condition that is typically seen in infant girls. This condition is often mistaken for other skin lesions such as acrochordons, condyloma acuminatum, and sequelae of sexual abuse. Many clinicians are unfamiliar with IPPP which can lead to aggressive workup and treatment. The purpose of this study was to elucidate demographic data, clinical features, diagnostic workup, and management of IPPP in a large cohort of patients seen at an academic medical center. METHODS: A retrospective review of patients diagnosed with IPPP at Mayo Clinic, Rochester, MN was conducted. Demographic data, examination findings, diagnostic workup, and treatments were noted. RESULTS: Twenty-seven patients with IPPP, 24 of which were girls and 3 were boys from the ages of 1 day to 4 years (mean 10.8 months, median 8.6 months) were identified. Symptoms were reported in 63% (17/27) of patients. Four patients received treatment with topical corticosteroid or local anesthetic. No patient underwent skin biopsy. CONCLUSION: IPPP is commonly observed in healthy prepubertal girls. Constipation is a common symptom. Awareness of this entity may prevent overtreatment and unnecessary evaluation.

Pediatric lichen planus: A single-center retrospective review of 26 patients with follow up.

BACKGROUND/OBJECTIVES: Pediatric lichen planus (LP) is rare with variable prevalence and atypical presentations compared to adults. Data on LP are lacking for the pediatric population in the United States. We present demographics, presentations, and treatments for a pediatric LP cohort. METHODS: We reviewed 26 patients diagnosed with LP at 20 years or younger. Treatment responses were defined as no response, partial response, and complete response. RESULTS: Demographics included 54% females and median diagnosis age of 16 years (range 6-20). Most patients presented with cutaneous LP (65%), with fewer having associated oral (23%), nail (7.7%), or genital (3.8%) involvement. Some had cutaneous-only LP (38%) or strictly mucosal LP (oral-only 19% and genital-only 15%). LP lesions were pruritic (50%), painful (19%), and/or asymptomatic (35%). Complete/partial responses occurred with medium-potency topical corticosteroids in cutaneous (n = 7; 64%), oral (n = 3; 75%), and genital LP (n = 3; 100%), with high/ultra-high potency topical corticosteroids in oral LP (n = 6; 86%), and with topical calcineurin inhibitors in genital LP (n = 2; 100%). Side effects were clobetasol-related oral candidiasis and biopsy-related penile depressed scar. Most patients with available follow-up achieved remission (n = 17; 81%). CONCLUSIONS: Pediatric LP usually presents in adolescence with cutaneous involvement and is symptomatic. However, patients frequently can have oral, genital, or nail lesions or may be asymptomatic, so they need thorough examinations and follow-up. Long-term remission is common due to treatment or natural disease course. Medium-potency corticosteroids are recommended for cutaneous, oral, and genital LP. Various other local and systemic therapies exist with successful treatment responses.

Tumor necrosis factor-α inhibitor-induced morphea and psoriasiform dermatitis in a pediatric patient with Crohn's disease.

Tumor necrosis factor-alpha inhibitor therapy for inflammatory bowel disease may be associated with paradoxical cutaneous adverse events, most commonly psoriasiform eruptions. We present the case of a pediatric female patient with Crohn's disease who developed multiple concurrent cutaneous eruptions while on infliximab treatment, including morphea, psoriasiform dermatitis, and genital lichen sclerosus. Although refractory to skin-directed treatments, all three conditions resolved upon discontinuation of infliximab, supporting their development as a paradoxical reaction to infliximab therapy.

Socioeconomic status, race, and preadolescent acne: A population-based retrospective cohort analysis in a mixed rural-urban community of the United States (Olmsted County, Minnesota).

BACKGROUND/OBJECTIVE: There is limited information about the potential relationship of socioeconomic status (SES) with acne in preadolescents. Our objective was to assess the possible relationship between SES and preadolescent acne. METHODS: A population-based retrospective cohort study was conducted to identify Olmsted County, Minnesota, residents with an initial acne diagnosis between 7 and ≤12 years old during 2010 to 2018 using the Rochester Epidemiology Project. For each acne case, we randomly selected 2 sex- and age-matched controls without an acne diagnosis from the county. Individual HOUsing-based SocioEconomic Status index (HOUSES) derived from real property data was used to evaluate SES, represented as four quartiles with higher quartile representing higher SES. RESULTS: A total of 604 patients met the criteria. HOUSES distribution significantly differed between cases and controls (p = .001); a higher proportion of acne cases were in quartile 4 (42.2% vs. 32.7%), indicating higher SES. Race and ethnicity did not significantly differ between cases and controls. Among cases and controls, 74.5% and 72.3% were White, respectively. Study limitations include its retrospective design, only patients who visited a physician were included, and Olmsted County residents are largely non-Hispanic White. CONCLUSION: Preadolescents diagnosed with acne have a higher SES than those without diagnosed acne, highlighting a potential disparity in access to care and appropriate diagnosis.

An investigation of vascular anomalies centers' transition of care practices.

This study aims to examine transition of care (TOC) practices of multidisciplinary vascular anomalies centers (VACs). Thirty-seven of 71 VAC leaders to whom the survey was sent completed the questionnaire. TOC and transfer practices varied with only 16% of VACs having TOC programs. The most frequently cited barriers to developing a TOC program were lack of resources and difficulty finding expert adult providers.