Linear IgA bullous dermatosis of childhood: Retrospective single-center cohort.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single-center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the "cluster of jewels" sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

Adult-onset linear IgA bullous dermatosis: a retrospective single-center cohort study of 81 patients and literature review.

BACKGROUND: Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder that may be drug-induced or paraneoplastic. We aim to characterize features of LABD and determine differentiating factors among idiopathic, drug-induced, or malignancy-associated diseases. METHODS: We conducted a single-center retrospective chart review of adult patients with linear IgA bullous dermatosis at a large tertiary referral center and a literature review of adult linear IgA bullous dermatosis. RESULTS: Eighty-one patients were included in the study. Ten patients (12.3%) had comorbid malignancy and nine (11.1%) had inflammatory bowel disease. Median disease duration was significantly shorter in both drug-induced (1.2 vs. 48.8 months; P < 0.001) and malignancy-associated (1.7 vs. 48.8 months; P < 0.001) LABD compared with idiopathic LABD. Recurrent episodes occurred significantly more often in idiopathic LABD compared to those with drug-induced (76.1 vs. 11.5%; P < 0.001) or malignancy-associated disease (76.1 vs. 33.3%; P = 0.019). Time to diagnosis was significantly shorter in the drug-induced (0.2 vs. 5.4 months; P < 0.001) and malignancy-associated groups (0.7 vs. 5.4 months; P = 0.049) compared with idiopathic; similarly, time to improvement was significantly shorter in both drug-induced (0.4 vs. 3.0 months; P < 0.001) and malignancy-associated disease (1.1 vs. 3.0 months; P = 0.016). Clinical morphology was indistinguishable between groups. Limitations included retrospective data collection, data from tertiary referral centers, and limited racial and ethnic diversity. CONCLUSION: Screening for underlying malignancy, as well as for a predisposing medication or possibly inflammatory bowel disease, may be advisable in patients with LABD, particularly when it is newly diagnosed.

Guidelines of care for the management of atopic dermatitis in adults with phototherapy and systemic therapies.

BACKGROUND: For people with atopic dermatitis (AD) refractory to topical therapies, treatment with phototherapy and systemic therapies can be considered. Multiple biologic therapies and Janus kinase (JAK)inhibitors have been approved since 2014 to treat AD. These guidelines update the 2014 recommendations for management of AD with phototherapy and systemic therapies. OBJECTIVE: To provide evidence-based recommendations on the use of phototherapy and systemic therapies for AD in adults. METHODS: A multidisciplinary workgroup conducted a systematic review and applied the GRADE approach for assessing the certainty of evidence and formulating and grading recommendations. RESULTS: The workgroup developed 11 recommendations on the management of AD in adults with phototherapy and systemic agents, including biologics, oral JAK inhibitors, and other immunomodulatory medications. LIMITATIONS: Most randomized controlled trials of phototherapy and systemic therapies for AD are of short duration with subsequent extension studies, limiting comparative long-term efficacy and safety conclusions. CONCLUSIONS: We make strong recommendations for the use of dupilumab, tralokinumab, abrocitinib, baricitinib, and upadacitinib. We make conditional recommendations in favor of using phototherapy, azathioprine, cyclosporine, methotrexate, and mycophenolate, and against the use of systemic corticosteroids.

Executive summary: Guidelines of care for the management of atopic dermatitis in adults with phototherapy and systemic therapies.

BACKGROUND: The summarized guidelines update the 2014 recommendations for the management of AD with phototherapy and systemic therapies. METHODS: A multidisciplinary workgroup conducted a systematic review and applied the GRADE approach for assessing the certainty of the evidence and formulating and grading recommendations. RESULTS: The workgroup developed 11 recommendations on the management of AD in adults with phototherapy and systemic therapies, including biologics, oral Janus Kinase inhibitors, and other immunomodulatory medications. CONCLUSIONS: The evidence supported strong recommendations for the use of dupilumab, tralokinumab, abrocitinib, baricitinib, and upadacitinib and conditional recommendations in favor of using phototherapy, azathioprine, cyclosporine, methotrexate, and mycophenolate, and against the use of systemic corticosteroids.

An update on long-term outcomes of pediatric follicular mucinosis over a 20-year period: a retrospective cohort study.

BACKGROUND: Follicular mucinosis (FM) is a rare disease characterized by mucin accumulation in the follicular unit. FM's etiology is still widely debated since its first description in 1957. Follicular mucinosis is usually reported to be benign in children, although reports of malignant transformation, most commonly mycosis fungoides, exist. The present project aims to demonstrate that children with a diagnosis of follicular mucinosis have positive long-term outcomes and do not develop mycosis fungoides. MATERIALS AND METHODS: This is a retrospective cohort study where patients with a diagnosis of follicular mucinosis ages 22 years and below were identified. Data surrounding the patient's diagnosis of FM, differential diagnosis, treatments, and long-term outcomes were collected. Patients who were lost to follow-up were contacted by phone for an update on the status of their skin and overall health. RESULTS: Out of 14 patients with follow-up information, none developed subsequent mycosis fungoides or other hematologic malignancies. CONCLUSION: Pediatric patients with follicular mucinosis will likely present with limited disease and not experience malignant transformation.

Guidelines of care for the management of atopic dermatitis in adults with topical therapies.

BACKGROUND: New evidence has emerged since the 2014 guidelines that further informs the management of atopic dermatitis (AD) with topical therapies. These guidelines update the 2014 recommendations for management of AD with topical therapies. OBJECTIVE: To provide evidence-based recommendations related to management of AD in adults using topical treatments. METHODS: A multidisciplinary workgroup conducted a systematic review and applied the GRADE (Grading of Recommendations, Assessment, Development, and Evaluations) approach for assessing the certainty of evidence and formulating and grading recommendations. RESULTS: The workgroup developed 12 recommendations on the management of AD in adults with topical therapies, including nonprescription agents and prescription topical corticosteroids (TCS), calcineurin inhibitors (TCIs), Janus kinase (JAK) inhibitors, phosphodiesterase-4 inhibitors (PDE-4), antimicrobials, and antihistamines. LIMITATIONS: The pragmatic decision to limit the literature review to English-language randomized trials may have excluded data published in other languages and relevant long-term follow-up data. CONCLUSIONS: Strong recommendations are made for the use of moisturizers, TCIs, TCS, and topical PDE-4 and JAK inhibitors. Conditional recommendations are made for the use of bathing and wet wrap therapy and against the use of topical antimicrobials, antiseptics, and antihistamines.

Executive summary: American Academy of Dermatology guidelines of care for the management of atopic dermatitis in adults with topical therapies.

These guidelines update the 2014 recommendations for management of atopic dermatitis in adults with topical therapies. A multidisciplinary workgroup employed best practices for guideline development, including a systematic review of the evidence and application of the Grading of Recommendations, Assessment, Development, and Evaluation approach for assessing the certainty of the evidence and formulating and grading recommendations. The evidence on atopic dermatitis treatment supported strong recommendations for the use of nonprescription moisturizers, topical calcineurin inhibitors, topical corticosteroids, and topical PDE-4 and JAK inhibitors. Conditional recommendations are made for the use of bathing and wet wrap therapy and against the use of topical antimicrobials, antiseptics, and antihistamines.

American Academy of Dermatology Guidelines: Awareness of comorbidities associated with atopic dermatitis in adults.

BACKGROUND: Studies found associations between atopic dermatitis (AD) and various comorbidities. OBJECTIVE: To appraise evidence of the association between AD and comorbidities among adults. METHODS: Our multidisciplinary work group conducted a systematic review of the association between AD and selected comorbidities. We applied the Grading of Recommendations, Assessment, Development, and Evaluation for prognosis approach for assessing the certainty of the evidence, providing statements of association based on the available evidence. RESULTS: Analysis of the evidence resulted in 32 statements. Clear evidence of the association of AD in adults and select allergic, atopic, immune-mediated mental health and bone health conditions and skin infections was identified. There is some evidence supporting an association between AD and substance use, attention deficit hyperactivity disorder, and elements of metabolic syndrome. Evidence suggests a small association with various cardiovascular conditions. The association between AD in adults and autism spectrum disorders, myocardial infarction, stroke, and metabolic syndrome is inconclusive. LIMITATIONS: This analysis is based on the best available evidence at the time it was conducted. This guideline does not make recommendations for screening or management of comorbidities in adults with AD. CONCLUSIONS: Clinicians should be aware of comorbidities associated with AD. Further research is needed to determine whether screening or management of comorbidities is beneficial for adults with AD.

A 28-year single institution experience with primary skin malignancies in the pediatric population.

The aim of this study is to report our institution's experience with pediatric skin malignancies. A single institution retrospective review of pediatric patients with a primary skin malignancy from 1992 to 2020 was performed. Demographics, tumor characteristics and treatment outcomes were reviewed. Ninety-nine patients with 109 primary malignant skin lesions were reviewed. The most common lesion was malignant melanoma [MM] (n = 50, 45.9%). Compared to non-melanoma skin cancer (NMSC), MM were more likely to present on trunk or extremities (p=.01, OR = 3.2), and be misdiagnosed (p=.03, OR = 2.7). NMSC were more common in the head and neck region (p=.01, OR = 3.2), and were associated with a personal history of skin cancer (p=.0005, OR = 17.1) or a known risk factor (p=.04, OR = 2.5). Patients with MM were 12.4-times more likely to develop metastatic disease compared to NMSC (p<.0001). Increased Breslow's thickness also increased the odds of developing metastatic disease (p=.03, OR = 1.6 per 1-mm increase). Interval time between lesion recognition and diagnostic biopsy or surgical treatment did not impact overall survival. Malignant melanoma was the most common malignancy in our cohort, followed by basal cell carcinoma. Malignant melanoma was the most likely tumor to be misdiagnosed and/or metastasize. Treatment delays did not impact risk of metastasis, recurrence or survival rate, though some patients succumbed to disease. These results may be attributed to small sample size or the biology of melanoma in pediatric patients. Awareness of skin malignancies in the pediatric population is imperative to providers and the public, with low threshold for specialty consultation and excision when warranted.

Trends in utilization of non-first-line topical acne medications among children, adolescents, and adults in the United States, 2012-2016.

BACKGROUND/OBJECTIVE: Current knowledge about usage of effective, but non-first-line topical acne medications in the United States is limited. We aimed to investigate utilization patterns and temporal trends for such acne medications in the US ambulatory care. METHODS: Pediatric (≤18 years old) and adult (>18 years old) data from the 2012 to 2016 (inclusive) cycles of the US National Ambulatory Medical Care Survey were extracted. Utilization patterns of six non-first-line topical acne medications (ie, azelaic acid, salicylic acid, glycolic acid, sulfur, resorcinol, and zinc) were compared and followed over time. RESULTS: Data from 218 410 US office-based sampled visits during 2012-2016 were included in the analysis. Across all acne visits (n = 1542), salicylic acid (1.58%), azelaic acid (1.22%), and glycolic acid (0.52%) were the most frequently used agents, while zinc and resorcinol were not used. Sulfur (0.52%) and salicylic acid (0.33%) were the only medications used in preadolescents, and none of these medications were used in the neonatal or infantile group. Temporal trends for using at least one of these medications were insignificant among both pediatric and adult age groups (P = .825 and .136, respectively). CONCLUSIONS: Salicylic acid and azelaic acid are the most frequently used of the studied second-line medications to treat acne, although the use of these and the other non-first-line topical medications overall is uncommon, especially among younger groups of US pediatric patients.

Use of spironolactone to treat acne in adolescent females.

BACKGROUND/OBJECTIVES: Studies assessing the utility of spironolactone for treating acne in adolescent females are lacking. Thus, we sought to examine spironolactone's role in treating this patient population. METHODS: A retrospective review was performed to determine the efficacy of spironolactone treatment in adolescent females seen at Mayo Clinic in Rochester, Minnesota, from 2007 to 2017. RESULTS: In a cohort of 80 pediatric patients with a median age of 19 years (range, 14-20 years), 64 patients (80%) experienced improvement of acne on treatment with spironolactone (median dose, 100 mg daily) with a favorable side effect profile. Approximately a quarter of patients (22.5%) had a complete response; more than half (58.8%) had a complete response or a partial response greater than 50%. Initial and maximal responses were observed at a median of 3 months and 5 months, respectively. Patients received treatment with spironolactone for a median duration of 7 months (range, 3-45 months) with limited side effects. CONCLUSIONS: Spironolactone demonstrated efficacy in treating acne in adolescent females and is a safe long-term alternative to systemic antibiotics in these patients.

Joint AAD-NPF Guidelines of care for the management and treatment of psoriasis with topical therapy and alternative medicine modalities for psoriasis severity measures.

Psoriasis is a chronic, inflammatory, multisystem disease that affects up to 3.2% of the United States population. This guideline addresses important clinical questions that arise in psoriasis management and care and provides recommendations based on the available evidence. The treatment of psoriasis with topical agents and with alternative medicine will be reviewed, emphasizing treatment recommendations and the role of dermatologists in monitoring and educating patients regarding benefits as well as risks that may be associated. This guideline will also address the severity assessment methods of psoriasis in adults.

Drug reaction with eosinophilia and systemic symptoms (DRESS) in the pediatric population: A systematic review of the literature.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a drug-induced hypersensitivity reaction that can have fatal complications. Although substantial data exist regarding DRESS in adults, to our knowledge, a systematic review of available literature has not been performed in children. OBJECTIVE: To review available data on DRESS in the pediatric population. METHODS: A systematic literature review was performed for pediatric (aged <18 years) patients with DRESS. RESULTS: We included 82 articles with 148 patients; of these, 97.9% experienced a skin rash, and the liver was the second most common organ involved (84.5%). Among 143 patients for which a treatment regimen was reported, 85.3% were treated with systemic steroids. Intravenous immunoglobulin alone failed to improve symptoms in 5 patients who were initially misdiagnosed, whereas those treated with intravenous immunoglobulin and steroids (2.7%) showed rapid clinical improvement. The mortality rate was low (3.0%). Complications included multiorgan failure and acute respiratory distress syndrome. LIMITATIONS: Limitations included limited availability of data for statistical analysis. CONCLUSION: Pediatric DRESS commonly involves the liver. With treatment, the prognosis is commonly good, but serious complications may occur. Corticosteroids, possibly in conjunction with intravenous immunoglobulin in severe cases, may serve as an effective, valuable treatment of pediatric DRESS.

Joint American Academy of Dermatology-National Psoriasis Foundation guidelines of care for the management of psoriasis with systemic nonbiologic therapies.

Psoriasis is a chronic inflammatory disease involving multiple organ systems and affecting approximately 2% of the world's population. In this guideline, we focus the discussion on systemic, nonbiologic medications for the treatment of this disease. We provide detailed discussion of efficacy and safety for the most commonly used medications, including methotrexate, cyclosporine, and acitretin, and provide recommendations to assist prescribers in initiating and managing patients on these treatments. Additionally, we discuss newer therapies, including tofacitinib and apremilast, and briefly touch on a number of other medications, including fumaric acid esters (used outside the United States) and therapies that are no longer widely used for the treatment of psoriasis (ie, hydroxyurea, leflunomide, mycophenolate mofetil, thioguanine, and tacrolimus).