Pediatric Dermatology最新文献

We present two pediatric patients who exhibited an unusual clinical presentation of cutaneous acute graft-versus-host disease (GVHD), characterized by livedo-like appearance. Such manifestations of cutaneous acute GVHD have not been previously documented.

Janus kinase inhibitors (JAKi) are drugs that block tyrosine kinases responsible for transducing cytokine signals. The first JAKi was approved by the US Food and Drug Administration (FDA) in 2011 to treat rheumatoid arthritis in adults. A pediatric indication was not approved until 8 years later, for acute graft-versus-host disease. Since then, topical and oral formulations have gained FDA approval for pediatric patients with dermatologic diseases. While increasing evidence supports the safety of these medications in adults, data are limited in children. We sought to determine whether JAKi adverse events (AEs) as reported in clinical trials and via postapproval pharmacovigilance services are comparable in adult and pediatric patients. Pharmacovigilance data were extracted from the FDA's Adverse Event Reporting System and the Canada Vigilance Adverse Reaction Online Database for baricitinib, upadacitinib, abrocitinib, ruxolitinib, and tofacitinib. The pooled data were analyzed to detect the most common AEs for specific JAKi and for the drug class. We assessed 399,649 AEs from 133,216 adults and 2883 AEs from 955 patients under 18 years old and identified slightly different AE profiles for the two age groups. Both populations had increased risk for infections and gastrointestinal AEs. However, pediatric patients reported a higher proportion of blood and lymphatic disorders, while reports of nervous system and musculoskeletal/connective tissue disorders were more common in adults. The spectrum of AEs extracted from pharmacovigilance reports was similar to clinical trials. The JAKi AE profiles we observed may prove helpful in counseling patients and their parents before starting therapy and for monitoring once patients are on therapy.

Rosacea diagnosis and treatment in children are challenging, due to its rarity and to the lack of approved pharmacological agents for its treatment in this age group. In this case series, response to treatment with once daily applications of ivermectin (IVM) 1% cream for 12 weeks in 10 children affected by mild to severe inflammatory rosacea was evaluated clinically by Investigator Erythema Severity Assessment (IESA), Investigator Global Assessment of Severity (IGA-S), Investigator Global Assessment score of Global Efficacy (IGA-GE), and instrumentally by dermoscopy and Erythema-Directed Digital Photography (EDDP). Clinical improvement was achieved at the end of treatment compared to baseline (IESA: 2.3 vs. 0.5; IGA-S: 2.1 vs. 0.3) and confirmed by IGA-GE (0 = 55%, 1 = 33%, 3 = 11%) and instrumental monitoring (EDDP: 2.7 vs. 0.6). Once daily IVM application may be an effective therapeutic option for children with rosacea.

Background and objectives: Dermoid cysts are congenital anomalies that contain ectodermal elements such as teeth, bone, and nerves. The purpose of this study was to identify trends in the characteristics of dermoid cysts of the auricle and the demographics of the patient population they affect.

Methods: A PubMed and EMBASE search for English-language publications that included cases of dermoid cyst(s) of the auricle from database inception to October 2022 was performed.

Results: A total of 38 cases of dermoid cysts of the auricle were identified from 20 publications. The age, sex, and Fitzpatrick skin type of the patients, locations of the dermoid cysts, associated anomalies, treatment methods, and postoperative outcomes were examined. Patients' ages ranged from 0 to 63 years, with 27.6% (8/29) in the 0-17 age range. 56.8% (21/37) were reported as male. Dermoid cysts were frequently present on the postauricular surface (18/24, 75.0%) and on light skin (Fitzpatrick types I-III; 18/22, 81.8%). Unilateral dermoid cysts were more commonly reported on the right ear (17/22, 77.3%); only one patient had bilateral cysts. Associated anomalies included microtia, prominent ears, foot polysyndactyly, hemifacial microsomia, and cryptotia. All auricular dermoid cysts were managed with complete surgical excision, and no postoperative complications or recurrences were reported.

Conclusions: Dermoid cysts of the auricle are reported more frequently in individuals with lighter skin; however, this is likely due to reporting bias. Complete surgical excision can be used to treat patients successfully, with a very low risk of complications and no need for preoperative imaging.

A 15-year-old boy presented with a yellowish, asymptomatic nodule on the left forearm, which developed in the last year. A diagnosis of juvenile xanthogranuloma was confirmed with histopathology. Line-field confocal optical coherence tomography (LC-OCT), a new skin imaging technique, was performed prior to surgery, showing a strong correspondence with histopathological sections. As juvenile xanthogranuloma is a benign disorder of the pediatric age, LC-OCT may represent an alternative to surgery for achieving a fast diagnosis in uncertain cases.

Junctional epidermolysis bullosa (JEB) is a rare genodermatosis characterized by fragility of the skin and mucous membranes due to alterations in the dermal epidermal junction. This condition manifests as mechanically induced bullous lesions that heal with hypertrophic granulation tissue and/or atrophic scars. Here, we report two brothers carrying a homozygous LAMB3 missense variant, p.Gly254Asp, which affects the N-terminal end of the laminin-332 (LM332) β3 chain, previously described in another JEB family sharing a common ethnic origin and LAMB3 haplotype with the siblings reported here. Moreover, all affected patients with p.Gly254Asp mutation from both families exhibits a distinct phenotype consisting of a few localized long-standing skin lesions characterized by excessive granulation tissue formation or keloid scars, without new blistering, and associated with amelogenesis imperfecta. Our patients also showed nail dystrophy, expanding the phenotypic spectrum and confirming the peculiar role of the N-terminal end of the β3 chain in regulating the granulation tissue response associated with the wound healing process.

We report five cases of spider angiomas in children treated topically with timolol 0.5% (ophthalmic solution or gel-forming solution), for 6 months. Parents and patients were advised to apply one drop twice daily. Four of them showed either partial (2) or complete response (2) and only one patient had no clinical change. No adverse effects were reported. This pilot case series shows that topical timolol may prove useful as a noninvasive, available and well-tolerated treatment option for spider angiomas in children who seek a treatment alternative to pulsed dye laser.

Congenital insensitivity to pain with anhidrosis (CIPA), also known as hereditary sensory and autonomic neuropathy (HSAN) type IV, is an extremely rare autosomal recessive congenital condition characterized by the loss of sensation to pain and absence of sweating with one case report linking this with harlequin color change. To explore the relationship further, we developed a survey using the Research Electronic Data Capture software and distributed it to families and others with close relationships and knowledge of patients with CIPA. Our results indicate that harlequin color change, characterized by unilateral flushing of the face and/or body, was significant and noted by all respondents as being present early in the condition appearing around the same time as first symptoms and around or before diagnosis of CIPA. Future clinicians should be aware of this dermatological phenomenon and its potential association with CIPA patients, especially during early disease manifestation where diagnosis can be difficult.