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The characteristics of epidermolysis bullosa (EB) demand higher than average provider support for transition from pediatric to adult care. We administered an online Qualtrics survey to members of the Epidermolysis Bullosa Clinical Research Consortium (EBCRC), a group of providers who care for patients with EB, in order to examine their practices and perspectives on transition of care (TOC) and identify barriers to successful implementation. Sixteen of eighteen medical centers completed the survey. Eighty-eight percent of center representatives expressed concerns about their patients transitioning/transferring from the pediatric to adult-centered care. Thirty-eight percent of providers reported having a formal TOC program in place. Our findings support the desire for formal TOC programs, the need for a team-based approach and, in particular, identification of adult providers to participate in the transition to improve this often challenging time., (© 2024 Wiley Periodicals LLC.)

Clear cell sarcoma of the kidney is a rare renal malignancy, accounting for 2%-4% of all pediatric renal tumors. In this case report, we describe a 9-year-old boy with an asymptomatic, solitary mass on the scalp, ultimately found to be metastatic clear cell sarcoma of the kidney. This report reviews indications for imaging scalp masses to facilitate making an accurate diagnosis and treatment planning., (© 2024 Wiley Periodicals LLC.)

The most common bacteria isolated from wound cultures in patients recorded in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database (EBCCOD) are Staphylococcus aureus and Pseudomonas aeruginosa. Given the prevalence of P. aeruginosa in this patient population and prior research implicating P. aeruginosa's potential role in carcinogenesis, we sought to further analyze patients with recorded wound cultures positive for Pseudomonas aeruginosa in the EBCCOD. We provide a descriptive analysis of this subset of patients and highlight potential avenues for future longitudinal studies that may have significant implications in our wound care management for patients with epidermolysis bullosa., (© 2023 Wiley Periodicals LLC.)

Pediatric teledermatology rapidly expanded with the COVID-19 pandemic, and the impacts of this expansion on patients' access to care have not yet been entirely defined. In this retrospective study of 3027 patients in an academic pediatric dermatology practice, patients who identified as having a primary language other than English were less likely to access pediatric dermatology care during the COVID lockdown. This study did not identify a significant or meaningful difference in age, geography, socioeconomic status, ethnicity, or race between patients who were offered pediatric dermatology care that was either in-person or via synchronous telehealth. These findings are overall reassuring that there were not major disparities in telehealth utilization during the COVID shelter-in-place mandate, although highlight the need for institutions to ensure systems are in place to enhance telehealth access for patients with non-English primary language., (© 2023 Wiley Periodicals LLC.)

Background/objectives: The primary objective was to assess pain catastrophizing and functional disability in pediatric patients with epidermolysis bullosa (EB) and their parents/guardians. Secondary objectives included examining relationships between pain catastrophizing, functional disability, and correlations with other factors (e.g., age, disease severity, and percent of body surface area (BSA) involved)., Methods: Patients with EB ages 8-16 and their parents/guardians who were English or Spanish speaking completed a one-time online survey. Parent measures included: demographics questionnaire, Pain Catastrophizing Scale-Parent (PCS), and Parent Functional Disability Inventory (FDI). Child measures included: PCS child and child FDI. Higher scores on both scales indicate higher levels of catastrophizing and functional disability., Results: Of 31 children, the mean age was 11.47 years and the majority (70.97%) had dystrophic EB. Mean scores were: 35.84 = PCS parent; 34.58 = PCS child; 30.87 = parent FDI; 29.77 = child FDI. Total scores for PCS parent, parent FDI, and child FDI increased significantly with disease severity and percentage of involved BSA (p < .01 for all). Total scores for PCS child increased significantly with percent of EB skin involvement (p = .04) but not disease severity. Older children reported more functional disability than their parents and younger children (p = .02)., Conclusions: Our results demonstrate significant positive correlations between negative thoughts related to pain and the experience of functional difficulties in patients with EB and their caregivers. Psychological, psychiatric, and/or behavioral interventions to help managing chronic pain may be effective for patients with EB., (© 2022 Wiley Periodicals LLC. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.)

Background/objectives: We evaluated the acceptance of synchronous (live video) telehealth for pediatric dermatology., Methods: This was a prospective, single-center study of patient and dermatologist surveys paired at the encounter level for telehealth encounters with Children's Hospital Colorado Pediatric Dermatology Clinic between 21 April 2020 and 22 May 2020., Results: Dermatologists were most receptive to a telehealth encounter for isotretinoin monitoring (96.6%) and non-isotretinoin acne (89.5%). In contrast, 71.8% and 58.8% of patients surveyed were open to telehealth for isotretinoin encounters and non-isotretinoin acne encounters, respectively. There was no significant correlation between patient and dermatologist satisfaction regarding a telehealth encounter (r = 0.09, CI [-0.09, 0.26], p = .34) or between patient and dermatologist preference for telehealth encounter (r = 0.07, CI [-0.11, 0.25] p = .46). Dermatologists reported needing a photo to aid their physical examination in 38/363 (10.7%) of encounters and preferred in-person examinations when an encounter would have benefitted from laboratories, procedures, dermatoscopic examination, examination by palpation, and accurate weights in infants., Conclusions: Synchronous, live-video telehealth is an effective method of healthcare delivery in certain situations for pediatric dermatology, but it does not replace in-person encounters. Families and dermatologists have different perceptions about its acceptance., (© 2022 Wiley Periodicals LLC.)

Background/objectives: Patients with epidermolysis bullosa (EB) require care of wounds that are colonized or infected with bacteria. A subset of EB patients are at risk for squamous cell carcinoma, and bacterial-host interactions have been considered in this risk. The EB Clinical Characterization and Outcomes Database serves as a repository of information from EB patients at multiple centers in the United States and Canada. Access to this resource enabled broad-scale analysis of wound cultures., Methods: A retrospective analysis of 739 wound cultures from 158 patients from 13 centers between 2001 and 2018., Results: Of 152 patients with a positive culture, Staphylococcus aureus (SA) was recovered from 131 patients (86%), Pseudomonas aeruginosa (PA) from 56 (37%), and Streptococcus pyogenes (GAS) from 34 (22%). Sixty-eight percent of patients had cultures positive for methicillin-sensitive SA, and 47%, methicillin-resistant SA (18 patients had cultures that grew both methicillin-susceptible and methicillin-resistant SA at different points in time). Of 15 patients with SA-positive cultures with recorded mupirocin susceptibility testing, 11 had mupirocin-susceptible SA and 6 patients mupirocin-resistant SA (2 patients grew both mupirocin-susceptible and mupirocin-resistant SA). SCC was reported in 23 patients in the entire database, of whom 10 had documented wound cultures positive for SA, PA, and Proteus species in 90%, 50%, and 20% of cases, respectively., Conclusions: SA and PA were the most commonly isolated bacteria from wounds. Methicillin resistance and mupirocin resistance were reported in 47% and 40% of patients tested, respectively, highlighting the importance of ongoing antimicrobial strategies to limit antibiotic resistance., (© 2020 Wiley Periodicals LLC.)

Topical and systemic retinoids have long been used in the treatment of ichthyoses and other disorders of cornification. Due to the need for long-term use of retinoids for these disorders, often beginning in childhood, numerous clinical concerns must be considered. Systemic retinoids have known side effects involving bone and eye. Additionally, potential psychiatric and cardiovascular effects need to be considered. Contraceptive concerns, as well as the additive cardiovascular and bone effects of systemic retinoid use with hormonal contraception must also be deliberated for patients of childbearing potential. The Pediatric Dermatology Research Alliance (PeDRA) Use of Retinoids in Ichthyosis Work Group was formed to address these issues and to establish best practices regarding the use of retinoids in ichthyoses based on available evidence and expert opinion., (© 2020 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)

Background/objectives: Epidermolysis bullosa (EB) comprises a group of inherited skin blistering diseases. There is currently no cure, and management includes skin protection and prevention of infection. To date, there has been no systematic investigation of home skin care practices among EB patients on a multicenter scale., Methods: This cross-sectional, observational study included data collected from patients with EB enrolled in the Epidermolysis Bullosa Characterization and Clinical Outcomes Database (EBCCOD) who provided answers to a patient-directed questionnaire between January 1, 2017, and December 31, 2017., Results: Of 202 respondents, 130 (64.4%) had dystrophic EB, 51 (25.2%) had EB simplex, 21 (7.4%) had junctional EB, 3 (1.5%) had Kindler syndrome, and 3 (1.5%) had an unspecified subtype. Seventy-eight patients reported cleansing in plain water only (39%). Of those who used an additive in their cleansing water, 75 (57%) added salt, 71 (54%) added bleach, 36 (27%) added vinegar, and 34 (26%) endorsed the use of an "other" additive (multiple additives possible). Reported concentrations of additives ranged widely from 0.002% sodium hypochlorite and 0.002% acetic acid solutions, which are thought to have negligible effects on microbes, to 0.09% sodium hypochlorite and 0.156% acetic acid, concentrations shown to be cytotoxic. One hundred eighty-eight patients answered questions regarding topical product use (93%). Of those, 131 reported topical antimicrobial use (70%). Mupirocin and bacitracin were the most commonly reported topical antibiotics (59, 58 [31.4%, 30.9%], respectively)., Conclusions: These findings highlight the variety of skin care routines and frequent use of topical antimicrobials among EB patients and have potential implications for antibiotic resistance. The reported range of bleach and vinegar additives to cleansing water, including cytotoxic concentrations, emphasizes the need for clear and optimized skin cleansing recommendations., (© 2020 Wiley Periodicals, Inc.)

Background/objectives: There are no reliably effective, well-tolerated topical agents for the treatment of hyperhidrosis. We sought to evaluate the efficacy and tolerability of oxybutynin 3% gel in adolescents and young adults with primary focal hyperhidrosis., Methods: Patients with severe axillary hyperhidrosis were treated with topical oxybutynin 3% gel for 4 weeks. Response to treatment was assessed by calculating change in Hyperhidrosis Disease Severity Score from baseline to weeks 1 and 4. Change in health-related quality of life was assessed using the Children's Dermatology Life Quality Index or the Dermatology Life Quality Index. Adverse effects were evaluated using patient diaries, investigator global review, and physical examination., Results: Of 10 patients aged 13-24 enrolled, seven completed the study. Of those who completed the study, four (57.1%) reported reduction in axillary Hyperhidrosis Disease Severity Score at week 1 and all seven (100%) at week 4. Six patients (85.7%) reported reduction in Children's Dermatology Life Quality Index or Dermatology Life Quality Index score. Anticholinergic adverse effects were infrequent. The majority of treatment-related adverse events were mild to moderate in severity. One patient experienced a severe adverse event., Conclusion: Oxybutynin 3% gel reduced hyperhidrosis severity and improved health-related quality of life in this small pilot study. Safety and efficacy should be further evaluated in a large, prospective, placebo-controlled study., (© 2018 Wiley Periodicals, Inc.)

Background/objectives: Pediatricians manage skin conditions such as atopic dermatitis (AD) but report that their dermatologic training is inadequate. Online modules may enhance medical education when sufficient didactic or clinical teaching experiences are lacking. We assessed whether an online module about AD improved pediatric residents' knowledge and changed their clinical management of AD., Methods: Target and control cohorts of pediatric residents from two institutions were recruited. Target subjects took a 30-question test about AD early in their residency, reviewed the online module, and repeated the test 6 months and 1 year later. The control subjects, who had 1 year of clinical experience but had not reviewed the online module, also took the test. The mean percentage of correct answers was calculated and compared using two-sided, two-sample independent t tests and repeated-measures analysis of variance. For a subset of participants, clinical documentation from AD encounters was reviewed and 13 practice behaviors were compared using the Fisher exact test., Results: Twenty-five subjects in the target cohort and 29 subjects in the control cohort completed the study. The target cohort improved from 18.0 ± 3.2 to 23.4 ± 3.4 correctly answered questions over 1 year (P < .001). This final value was greater than that of the control cohort (20.7 ± 4.5; P = .01). Meaningful differences in practice behaviors were not seen., Conclusion: Pediatric residents who reviewed an online module about AD demonstrated statistically significant improvement in disease-specific knowledge over time and had statistically significantly higher scores than controls. Online dermatology education may effectively supplement traditional clinical teaching., (© 2017 Wiley Periodicals, Inc.)

Hereditary progressive mucinous histiocytosis is a rare, benign, skin-limited form of non-Langerhans cell histiocytosis. We report on a 5-year-old boy who presented in infancy with self-resolving dermal nodules but later developed persistent and progressive erythematous papules on the face and scalp. Histologic evaluation revealed dermal aggregates of S-100/CD1a-negative histiocytes with abundant mucin. We present this case to highlight the evolution of the lesional morphology in infancy and early childhood and to stress the importance of histology in confirming this rare disorder., (© 2015 Wiley Periodicals, Inc.)

Pruritus is a common complication in patients with epidermolysis bullosa (EB). There is limited published data about the treatments that individuals with EB use for pruritus. The objective of the current study was to determine quantitatively which treatments individuals with EB have used for pruritus and to evaluate the perceived effectiveness of these treatments in pruritus relief. A questionnaire was developed to evaluate the treatments and therapies used for pruritus in patients of all ages and for all types of EB. Questions about bathing products, moisturizers, topical products, oral medications, dressings, and alternative therapies were included. A 5-point Likert scale (-2 = relieves itch a lot, -1 = relieves itch a little, 0 = no change, 1 = increases itch a little, 2 = increases itch a lot) was used to evaluate perceived effectiveness. Patients from seven North American EB centers were invited to participate. Greasy ointments (53.4%), lotions (45.2%), creams (40.4%), and oral hydroxyzine (39.0%) were the most frequently used treatments for pruritus. Treatments that were used frequently and perceived to be the most effective included creams (mean = -1.1), topical prescription corticosteroids (mean = -1.0), oils (mean = -0.9), oral hydroxyzine (mean = -0.9), topical diphenhydramine (mean = -0.9), and vaporizing rub (menthol, camphor, eucalyptus) (mean = -0.9). Systemic opioids (mean = 0.3), adherent bandages (mean = 0.3), and bleach baths (mean = 0.2) slightly increased pruritus. Randomized controlled trials of therapies will be necessary to develop evidence-based recommendations for control of pruritus in individuals with EB., Competing Interests: All authors have no conflict of interest., (© 2014 Wiley Periodicals, Inc.)

Agminated flexural melanocytic nevi in children with a history of Langerhans cell histiocytosis (LCH) are rare and thought to be coincidental or related to systemic chemotherapy. We report on an 11-year-old boy in remission from LCH, treated with only topical steroids, who presented years later with an eruption of melanocytic nevi in the bilateral inguinal and axillary regions. Rather than coincidence, we hypothesize that agminated flexural melanocytic nevi are a late sequela of LCH, possibly resulting from immune tolerance or a reaction to local inflammation., (© 2015 Wiley Periodicals, Inc.)

Epidermolysis bullosa (EB) is a genetic condition characterized by skin fragility and blistering. There is no instrument available for clinical outcome research measurements. Our aim was to develop a comprehensive instrument that is easy to use in the context of interventional studies. Item collection was accomplished using a two-step Delphi Internet survey process for practitioners and qualitative content analysis of patient and family interviews. Items were reduced based on frequency and importance using a 4-point Likert scale and were subject to consensus (>80% agreement) using the nominal group technique. Pilot data testing was performed in 21 consecutive patients attending an EB clinic. The final score, Instrument for Scoring Clinical Outcome of Research for Epidermolysis Bullosa (iscorEB), is a combined score that contains clinician items grouped in five domains (skin, mucosa, organ involvement, laboratory abnormalities, and complications and procedures; maximum score 114) and patient-derived items (pain, itch, functional limitations, sleep, mood, and effect on daily and leisurely activities; maximum score 120). Pilot testing revealed that combined (see below) and subscores were able to differentiate between EB subtypes and degrees of clinical severity (EB simplex 21.7 ± 16.5, junctional EB 28.0 ± 20.7, dystrophic EB 57.3 ± 24.6, p = 0.007; mild 17.3 ± 9.6, moderate 41.0 ± 19.4, and severe 64.5 ± 22.6, p < 0.001). There was high correlation between clinician and patient subscores (correlation coefficient = 0.79, p < 0.001). iscorEB seems to be a sensitive tool in differentiating between EB types and across the clinical spectrum of severity. Further validation studies are needed., (© 2014 Wiley Periodicals, Inc.)

Rapidly involuting congenital hemangioma (RICH) is an uncommon, often high-flow vascular tumor that presents at birth and involutes within the first year of life. It is clinically and histologically distinct from infantile hemangioma, kaposiform hemangioendothelioma, and tufted angioma, the latter two being associated with Kasabach-Merritt phenomenon. We present a female infant with RICH and profound, transient thrombocytopenia and review the extent and clinical course of thrombocytopenia in the context of congenital vascular tumors., (© 2012 Wiley Periodicals, Inc.)

Epidermolysis bullosa (EB) is a group of rare, inherited, blistering diseases that typically present in infancy. EB is not curable, and treatment is entirely supportive. There is a paucity of standardized recommendations to guide management. To assess the current state of EB care, an original online survey was conducted targeting attending physicians experienced with the care of EB. Members of the Society for Pediatric Dermatology residing in the United States and Canada served as the source pool. Parameters assessed included clinic visits, availability of subspecialists, and performance of surveillance studies. Fifty-six completed surveys were analyzed. Most providers saw between 1 and 10 individuals with EB per year in a general dermatology clinic. For each EB type there was considerable variation in the frequency of clinic visits, availability and use of specialists, and use of laboratory and imaging studies. Some agreement was observed in the frequency of follow-up for infants with more severe EB types, as well as for the components of a history, physical, and routine laboratory studies. These findings describe variations in the current state of EB care that pediatric dermatologists provide. The development and acceptance of evidence-based guidelines and outcome measures may lead to greater uniformity in EB care., (© 2014 Wiley Periodicals, Inc.)

The natural history of inherited epidermolysis bullosa (EB) varies significantly across subtypes. When confronted with an infant suspected to have EB, rapidly determining the type and subtype is critical in counselling families accurately about the infant's diagnosis and prognosis. Although transmission electron microscopy (TEM) has been considered the criterion standard for EB diagnosis, immunofluorescence microscopy (IFM) using monoclonal antibodies (mAbs) to EB-specific basement membrane zone proteins has several advantages, but few studies have evaluated the diagnostic utility of IFM. We sought to evaluate the clinical utility of IFM using an expanded panel of EB-specific mAbs. This was a retrospective review of pathology reports from infants younger < 1 year old with suspected EB primarily analyzed with IFM by the Stanford Dermatopathology service. Seventy-seven cases were identified for analysis, of which 20 were suboptimal for IFM analysis. Fifty-five cases were diagnosed with EB and classified as follows: EB simplex (n = 5), junctional EB (n = 31), dystrophic EB (n = 19). TEM was available in 36 of 55 cases (65%). IFM with an expanded panel of EB-specific mAbs should be considered the first-line diagnostic test to evaluate infants with clinically suspected EB., (© 2013 Wiley Periodicals, Inc.)

Subcutaneous fat necrosis of the newborn is a form of panniculitis that most often occurs in full-term infants with predisposing risk factors. Three neonates with hypoxic ischemic encephalopathy were treated with therapeutic hypothermia and developed extensive subcutaneous fat necrosis. All three infants developed extensive subcutaneous fat necrosis, involving the back, scalp, and arms. Mild, asymptomatic hypercalcemia was noted in one infant in the weeks following the subcutaneous fat necrosis. Hypothermia as a risk factor for subcutaneous fat necrosis is reviewed. Clinicians should be aware of subcutaneous fat necrosis as a possible risk factor and complication associated with asphyxiated newborns who may undergo therapeutic hypothermia. Future studies for therapeutic hypothermia should evaluate neonates for the development of subcutaneous fat necrosis., (© 2011 Wiley Periodicals, Inc.)

Accurately measuring the size of infantile hemangiomas is critical for objectively quantifying their growth, involution, and treatment response. Volumetric measurements are particularly appropriate because hemangiomas tend to mark out their territory early on and then grow and involute volumetrically. To our knowledge, only two publications have proposed bedside methods for measuring hemangioma volume. In these two publications, the authors modeled hemangiomas as perfect spheres or half-spheres, but many hemangiomas more closely approximate ellipsoids, with each of their axes in three dimensions having different, rather than equal, lengths. We present an alternative bedside method for calculating hemangioma volume, using a formula derived with calculus requiring only three bedside measurements using a disposable paper tape measure., (© 2011 Wiley Periodicals, Inc.)

We report four cases of diffuse infantile hepatic hemangioma, a rare but potentially life-threatening subset of hepatic hemangiomas. All patients demonstrated distinctive dome-shaped red-purple cutaneous hemangiomas. Two patients responded to steroids and propranolol (one in combination with vincristine), and two responded to steroids and vincristine. After a systematic literature review, we identified 26 previously reported cases of diffuse infantile hepatic hemangioma. Diffuse infantile hepatic hemangioma had a mortality rate of 17% and a >70% incidence of hypothyroidism, often severe (n = 30). More than one-third of patients developed heart failure (high output in half the cases). Based on our experience, early aggressive medical management, as well as thyroid replacement when indicated, should be initiated early in the course of diffuse infantile hepatic hemangioma pending evaluation for liver transplant., (© 2011 Wiley Periodicals, Inc.)

Terra firma-forme dermatosis (TFFD) is an idiopathic condition characterized by asymptomatic, dirtlike, thinly papillomatous, hyperpigmented plaques which cannot be removed with routine cleansing, but clear with firm wiping with 70% isopropyl or ethyl alcohol. Commonly involved sites include the neck, trunk, and umbilicus. Most reports have described adolescents or young adults with a history of normal hygiene and vigorous but unsuccessful attempts at cleansing the affected areas. We describe a 4-month-old girl with TFFD, who is the youngest patient thus far reported with this condition., (© 2011 Wiley Periodicals, Inc.)

Recent discussions regarding the burden of skin disease and patient-centered medicine highlight the profound effects skin disease can have on individuals, their families, and society as a whole. Local support groups, often connected to national patient advocacy groups, can be an invaluable resource for patients, and offer physicians the opportunity to learn more about patients' disease experiences while providing adjunctive therapy for conditions such as alopecia areata and vitiligo, for which medical options are often limited. We created a support group for children with alopecia areata and their parents as a model for other diseases such as vitiligo and epidermolysis bullosa. Herein we outline the steps involved in establishing a support group, including the many resources available for patient support, steps in the recruitment of patients, topics for discussion and goals for the group, and the logistics of running a meeting. Creating this family support group was a relatively straightforward and rewarding experience for us, and we hope that other pediatric dermatologists can utilize this model for their patients.

Our objective was to describe the efficacy of darbepoetin alfa and ferric gluconate complex in the treatment of the anemia associated with recessive dystrophic epidermolysis bullosa. To accomplish this aim, we retrospectively reviewed a series of patients with this disease treated in a single institution with darbepoetin alfa and parenteral iron for anemia. Four patients with recessive dystrophic epidermolysis bullosa were treated for a mean length of treatment of 14.5 months (4-18 months). Three patients received parenteral iron in the form of ferric gluconate complex and one received iron dextran. The mean pretreatment hemoglobin was 6.8 g/dL (4.9-9.6 g/dL). All four had improvements in their hemoglobin levels and energy levels with a mean increase in hemoglobin level of 2.8 g/dL (p = 0.003). We found darbepoetin alfa and ferric gluconate complex to be effective in the treatment of anemia associated with recessive dystrophic epidermolysis bullosa and to have distinct advantages over previously described formulations, and we concluded that they should be considered in the supportive care of this disease. We recommend the development of a standardized protocol for the evaluation and management of recessive dystrophic epidermolysis bullosa-associated anemia.

Immune-mediated blistering diseases are rare in childhood, with the subset of IgA pemphigus being extremely uncommon. A child with a bullous disorder of the oral mucosa, skin, and colon is reported because of the unusual nature of the clinical and laboratory findings. Immunofluorescence studies demonstrated both intercellular epidermal and basement membrane zone deposition of IgA and IgG antibodies, as well as intercellular colonic deposition of IgA antibodies. IgA and IgG ELISA tests were positive for desmoglein 3 as the intercellular epidermal antigen. The generation of multiple antibodies against several potential antigenic targets suggests epitope spreading may play a role in this disease. We believe this represents a novel variant of IgA pemphigus.

Childhood dermatitis herpetiformis (DH) is an immunobullous disease associated with gluten-sensitive enteropathy. This disease is rare in children and is typically characterized by intensely pruritic vesicles on the extensor surfaces. Definitive diagnosis of DH depends on the direct immunofluorescence finding of granular or fibrillar IgA deposits along the basement membrane zone of biopsied perilesional skin. We report an 11-year-old boy with an unusual presentation of DH characterized by a 7-month history of chronic urticaria-like skin lesions. He had evanescent, largely asymptomatic, urticarial wheals on his trunk, face, and extremities that were unresponsive to conventional therapy for urticaria. Skin biopsy specimen findings were consistent with DH and direct immunofluorescence of perilesional skin was diagnostic. The patient had no symptoms of gluten-sensitive enteropathy at the time of diagnosis, and his skin lesions rapidly cleared with dapsone therapy. This patient serves to highlight an unusual presentation of childhood DH and the need to consider this diagnosis when evaluating chronic urticarial lesions in children.