Pediatric cutaneous T-cell neoplasm and mimics with gamma-delta expression: Not always aggressive.

Pediatric cutaneous T-cell lymphoma with γδ immunophenotype is extremely rare. Only a few cases of γδ T-cell neoplasm have been reported in the literature, and therefore little is known whether γδ T-cell neoplasms in children are distinct from their adult counterparts with respect to the clinicopathological presentation, behavior, and prognosis. In this study, we demonstrate three unique pediatric cutaneous T-cell neoplasm and mimics with increased γδ T cells. All cases showed an indolent clinical course.

A survey assessment of combined pediatric dermatology-rheumatology clinics.

The expertise of both dermatology and rheumatology may be beneficial when managing autoimmune conditions with cutaneous and systemic manifestations in children. This survey study was directed to pediatric dermatologists who participate in combined pediatric dermatology-rheumatology clinics; 13 sites in North America responded. The results provide information regarding clinic operations, benefits, and barriers to establishment. These findings have the potential to help institutions establish or modify combined pediatric dermatology-rheumatology clinics, although further research is needed to determine their impact.

COVID-19 associated severe mucocutaneous blistering eruptions: A case series.

Mucocutaneous eruptions are associated with numerous infectious processes and can present as erythema multiforme (EM), reactive infectious mucocutaneous eruption (RIME), Stevens Johnson syndrome (SJS), or toxic epidermal necrolysis (TEN). Limited reports have detailed the association of these eruptions with SARS-CoV-2 infection. We present a series of eight cases of severe mucocutaneous blistering eruptions associated with SARS-CoV-2 infection. A retrospective case series was performed at six tertiary medical centers from March 1, 2020 to August 1, 2022. Inclusion criteria were met with a clinical diagnosis of EM, RIME, SJS, or TEN and a positive SARS-CoV-2 test (rapid antigen or polymerase chain reaction) less than 4 weeks prior to onset of dermatologic manifestation. Data was collected at time of each patient encounter. Eight patients met criteria with six pediatric patients (<18 years of age) having a median age of 15 years and two adult patients (>18 years of age) having a median age of 36 years. Patients were found to have a clinical diagnosis of RIME in 85.7% of cases. Oral mucosal involvement was the most common clinical finding (100%), followed by ocular (50.0%), urogenital (50.0%), and skin (37.5%) involvement. Evaluation did not reveal any additional infectious triggers in four patients. Evidence of possible concurrent or previous infectious triggers were identified in four patients. This case series highlights the development of severe mucocutaneous eruptions in association with COVID-19 infection, as well as the potential contributing role of concurrent or prior infections.

Proceedings of the 10th Pediatric Dermatology Research Alliance (PeDRA) Annual Conference.

The 10th Pediatric Dermatology Research Alliance (PeDRA) Annual Conference occurred November 3-5, 2022 in Bethesda, Maryland. This conference was the first in-person PeDRA conference after 2 years of a virtual format due to COVID-19. Fittingly, given the effects of the pandemic, the conference theme was "Reimagining Community." The conference included presentations and panel sessions on finding individual and collective purpose, leveraging community in pursuit of a shared goal, and creating a community of resources in collaboration with NIH. The goal of this meeting was to connect clinicians, basic scientists, patients, patient advocates, and industry partners. The reimagined community of pediatric dermatology research is a synergistic space for all members to better understand, prevent, treat, and cure dermatologic diseases and conditions in children. This two-and-a-half-day conference with over 300 attendees featured educational seminars including a keynote address, didactic lecture and panel sessions, skill-building workshops, 13 topic-specific breakout sessions, and an interactive poster session where 108 active and finished research projects could be discussed.

Methotrexate for inflammatory skin disease in pediatric patients: Consensus treatment guidelines.

Methotrexate (MTX) is a readily accessible drug, first used in 1948 and employed for a wide variety of indications since then. However, despite widespread off-label use, FDA labeling does not include approved indications for the use of MTX for many inflammatory skin diseases in pediatric patients, including morphea, psoriasis, atopic dermatitis, and alopecia areata, among others. Without published treatment guidelines, some clinicians may be hesitant to use MTX off-label, or uncomfortable prescribing MTX in this population. To address this unmet need, an expert consensus committee was convened to develop evidence- and consensus-based guidelines for use of MTX to treat pediatric inflammatory skin disease. Clinicians with experience and expertise in clinical research, drug development, and treating inflammatory skin disease in pediatric patients with MTX were recruited. Five committees were created based on major topic areas: (1) indications and contraindications, (2) dosing, (3) interactions with immunizations and medications, (4) adverse effects (potential for and management of), and (5) monitoring needs. Pertinent questions were generated and addressed by the relevant committee. The entire group participated in a modified Delphi process to establish agreement on recommendations for each question. The committee developed 46 evidence- and consensus-based recommendations, each with >70% agreement among members, across all five topics. These are presented in tables and text, along with a discussion of supporting literature, and level of evidence. These evidence- and consensus-based recommendations will support safe and effective use of MTX for the underserved population of pediatric patients who may benefit from this valuable, time-honored medication.

The national burden of inpatient dermatology in adults.

BACKGROUND: Management of inpatient skin disease represents a unique subspecialty within dermatology. OBJECTIVE: To assess the national burden of inpatient dermatology in adults. METHODS: Using the 2014 National Inpatient Sample, we performed a retrospective cohort study of adults hospitalized for dermatologic conditions. RESULTS: In 2014, there were 644,320 weighted hospitalizations principally for skin disease in adults, which cost the health care system $5.04 billion. Overall, skin disease was diagnosed in 1 in 8 hospitalized adults. Dermatologic hospitalizations were associated with a lack of medical insurance (odds ratio [OR], 2.27; 95% confidence interval [CI], 2.20-2.34), residence in a low-income community (OR, 1.10; 95% CI, 1.07-1.13), and small (OR, 1.27; 95% CI, 1.23-1.32) or rural hospitals (OR, 1.38; 95% CI, 1.32-1.44). Racial minorities were less likely to be hospitalized for skin disease than were whites (for blacks: OR, 0.77; 95% CI, 0.75-0.79; for Hispanics: OR, 0.85; 95% CI, 0.83-0.8; for Asians: OR, 0.59; 95% CI, 0.55-0.64). Only 0.47% of patients admitted for skin disease experienced in-hospital mortality; however, mortality rates were high in hospitalizations for cutaneous lymphomas (9.19%) and malignant melanoma (6.54%). LIMITATIONS: We could not assess the impact of inpatient dermatology consultations on hospitalization outcomes. CONCLUSIONS: Skin disease is highly prevalent among hospitalized patients.

Hospital readmissions among patients with skin disease: A retrospective cohort study.

BACKGROUND: Hospital readmissions represent a potential target for reducing unnecessary health care expenditures; however, readmissions following dermatology hospitalizations remain poorly characterized. OBJECTIVE: To assess the frequency and demographics of readmissions for skin disease. METHODS: We performed a retrospective cohort study of dermatology hospitalizations by using the 2014 Nationwide Readmissions Database. RESULTS: Readmissions following dermatologic hospitalizations cost the American health care system $1.05 billion in 2014. The 30-day rate of all-cause readmission following the 647,251 weighted index admissions for skin disease was 12.63%. Readmission was most common following hospitalizations for cutaneous lymphomas (39.63%), connective tissue disorders (26.28%), and cutaneous congenital abnormalities (23.86%). Predictors of readmission included public insurance with Medicaid (odds ratio [OR], 1.61; 95% confidence interval [CI], 1.53-1.70) or Medicare (OR, 1.55; 95% CI, 1.48-1.62), residence in a low-income community (OR, 1.14; 95% CI, 1.09-1.20), an increased number of chronic conditions (OR, 4.46; 95% CI, 4.15-4.79), and a large hospital (OR, 1.10; 95% CI, 1.05-1.16). Urban (OR, 0.90; 95% CI, 0.87-0.94) and rural (OR, 0.78; 95% CI, 0.73-0.82) nonteaching hospitals were protective against readmissions from skin disease. LIMITATIONS: We were unable to assess the impact of inpatient dermatology consultations on hospital readmission rates. CONCLUSIONS: There are significant health care and demographic disparities in readmissions for skin disease.

Inpatient burden of pediatric dermatology in the United States.

BACKGROUND/OBJECTIVES: It is known that inpatient care accounts for a significant portion of health care expenditures, but the national burden of inpatient pediatric dermatology is poorly characterized. We sought to assess risk factors, conditions, and financial costs associated with pediatric hospitalizations for skin disease. METHODS: We performed a cross-sectional study of pediatric dermatology hospitalizations using the 2012 Kids' Inpatient Database, which samples 80% of non-birth-related pediatric admissions from 44 states to generate national estimates. The demographic characteristics of children admitted for dermatologic and nondermatologic conditions were compared, and the financial costs of these admissions were analyzed. RESULTS: In 2012, there were 74 229 (95% confidence interval (CI) = 68 620-79 978) pediatric dermatology hospitalizations, accounting for 4.2% of all pediatric admissions and $379.8 million (95% CI = $341.3-418.4 million) in health care costs. Bacterial infections (n = 59 115, 95% CI = 54 669-63 561), viral diseases (n = 3812, 95% CI = 3457-4167), and noncancerous skin growths (n = 2931, 95% CI = 2318-3545) were the most common conditions requiring hospitalization. The highest mean cost per hospitalization was for admissions for cutaneous lymphomas ($58 294, 95% CI = $31 694-84 893), congenital skin abnormalities ($24 186, 95% CI = $16 645-31 728), and ulcers ($17 064, 95% CI = $14 683-19 446). Pediatric dermatology hospitalizations were most strongly associated with living in a low-income community (odds ratio (OR) = 1.22, 95% CI = 1.16-1.29) and the South (OR = 1.32, 95% CI = 1.19-1.46) and being uninsured (OR = 1.35, 95% CI = 1.26-1.45) or having Medicaid insurance (OR = 1.17, 95% CI = 1.13-1.22). CONCLUSION: Skin disease is a common cause of hospitalizations in children, and there are disparities in these admissions that could reflect inadequate access to outpatient pediatric dermatologists.

Thyroid dermopathy and acropachy in pediatric patients.

The extrathyroid manifestations of Graves disease (GD) include thyroid orbitopathy, dermopathy, and acropachy. Thyroid dermopathy (TD), also known as pretibial myxedema, classically presents as nonpitting edema or plaquelike lesions on the pretibial region, while thyroid acropachy (TA) is seen in cases of severe TD, characterized by soft tissue swelling and clubbing of fingers and toes, as well as a periosteal reaction of the bones of the hands and feet. Both TD and TA are rare manifestations of thyroid disease and uncommonly reported in pediatric patients. Our aim was to increase awareness of dermatological manifestations associated with pediatric GD and review the literature of pediatric thyroid dermopathy as well as report a case of acropachy in a child.

Segmental storiform collagenomas: Expanding the spectrum of PTEN hamartoma tumor syndrome in children.

A 4-year-old girl with autism spectrum disorder and congenital heart disease presented to dermatology clinic for evaluation of skin growths present since infancy. Physical examination was significant for macrocephaly and agminated skin-colored to pink papulonodules in a segmental distribution on the right lower back and buttocks, biopsy of which showed storiform collagenomas (sclerotic fibromas). Genetic testing revealed a pathogenic missense mutation in the PTEN gene, and a diagnosis of PTEN hamartoma tumor syndrome was made. The segmental nature of her storiform collagenomas is unique, to our knowledge, and may be explained by a postzygotic second-hit PTEN mutation, contributing to the growing spectrum of clinical findings associated with PTEN hamartoma tumor syndrome.

Erythema ab igne in patients with sickle cell disease.

Erythema ab igne (EAI) is an asymptomatic dermatosis caused by prolonged exposure to localized heat. Affected areas have net-like hyperpigmentation that may resemble more serious conditions such as livedo racemosa or vasculitis. We report three cases of EAI in pediatric sickle cell disease (SCD) patients who were initially suspected of having a more severe, life-threatening disorder before Dermatology was consulted. Clinicians caring for pediatric SCD patients who regularly use heating pads/devices for pain relief should consider EAI in the differential diagnosis of large areas of net-like hyperpigmentation. This paper aims to increase recognition of EAI and patient education on safe practices while using heating pads.

Genetic basis for vascular anomalies.

The fundamental genetics of many isolated vascular anomalies and syndromes associated with vascular anomalies have been elucidated. The rate of discovery continues to increase, expanding our understanding of the underlying interconnected molecular pathways. This review summarizes genetic and clinical information on the following diagnoses: capillary malformation, venous malformation, lymphatic malformation, arteriovenous malformation, PIK3CA-related overgrowth spectrum (PROS), Proteus syndrome, SOLAMEN syndrome, Sturge-Weber syndrome, phakomatosis pigmentovascularis, congenital hemangioma, verrucous venous malformation, cutaneomucosal venous malformation, blue rubber bleb nevus syndrome, capillary malformation-arteriovenous malformation syndrome, Parkes-Weber syndrome, and Maffucci syndrome.

A Pigmented Subcutaneous Nodule on the Scalp.

A 52-year-old man presented to his primary doctor with a slow-growing cystic lesion on his occipital scalp. His primary care doctor diagnosed the lesion as a pilar cyst and recommended observation because the lesion was asymptomatic at that time. The patient had no significant medical or surgical history. There was no family history of skin cancer or other malignancies.

A Souvenir From France: Acrodermatitis Chronica Atrophicans Presenting in the United States.

A 70-year-old man was referred by his rheumatologist to our dermatology clinic for evaluation of dermatitis on his right arm that appeared 3 months earlier. The skin lesion was asymptomatic and the patient denied current systemic symptoms, including fever, chills, and joint pain; however, 10 months prior to this presentation he experienced arthritis in the left knee. At that time, Borrelia serology revealed positive IgG (6.07; <0.8 negative, 0.8 to 0.99 borderline, ≥1 positive) and negative IgM titers. The patient had not received treatment for Lyme disease in the past. He was referred to rheumatology for evaluation of possible Lyme disease but did not follow up until 10 months later. The arthritis has since resolved. He travels frequently to France and recalls multiple tick bites during these trips.

Outcomes of biopsies and excisions of dysplastic acral nevi: a study of 187 lesions.

BACKGROUND: Despite their frequency in clinical practice, controversy exists regarding the significance and management of dysplastic nevi (DN). Although the perception of DN as precursors to melanoma is questionable, excisions of biopsy-proven DN are commonplace in clinical practice. The management of dysplastic acral nevi is of interest given the challenge of surgery at acral sites. OBJECTIVE: To determine the outcomes of biopsies of clinically atypical acral nevi and excisions of histologically dysplastic acral nevi (HDN). MATERIALS AND METHODS: Retrospective review of consecutive patients at a private dermatology practice who had a biopsy of an atypical acral nevus from December 2004 to July 2012. RESULTS: One hundred eighty-seven atypical acral nevi were biopsied from 168 patients (77 (41%) HDN, 108 (58%) common nevi). Based on initial histology, 30 (39%) HDN were recommended for excision and eight (10%) for clinical observation. Twenty-seven of the 77 HDN were excised; 23 (85%) revealed scar only, and four (15%) revealed residual DN not involving the margin. CONCLUSION: Routine excision of biopsy-proven dysplastic acral nevi may not be necessary.

An adolescent boy with urticaria to water: review of current treatments for aquagenic urticaria.

We report the case of an adolescent boy with aquagenic urticaria unresponsive to oral antihistamine therapy. We successfully treated his condition by topical application of a petrolatum-containing cream as a protective coating. To our knowledge, this is the first report showing the use of topical therapy alone to treat aquagenic urticaria in a child. Based on the effectiveness, safety profile, and ease of use, clinicians may wish to consider this regimen as a first-line therapy.