Pediatric Dermatology最新文献

Oral N-acetylcysteine (NAC) has shown efficacy for debilitating habit-driven and neuropsychiatric disorders in small, mostly adult studies. We retrospectively evaluated the therapeutic use and safety of oral NAC in 93 children from the Children's Hospital of Philadelphia. This study supports the use of oral NAC for habit-driven skin, hair, and nail abnormalities in pediatric patients.

A 5-year-old male with xeroderma pigmentosum from Honduras presented with a rapidly growing mass on the left post-auricular neck, associated with left-sided hearing loss. MRI revealed a large mass with invasion of the external auditory canal, temporal bone, and metastasis to lymph nodes. Biopsy confirmed moderately differentiated squamous cell carcinoma (SCC). Given the extensive spread, the patient was started on pembrolizumab, resulting in dramatic clinical and radiographic improvement after four rounds of therapy over 3 months, without associated side effects such as diarrhea, fever, dermatitis, or joint pain. This case demonstrates the effectiveness and tolerability of pembrolizumab in young pediatric patients with metastatic SCC, highlighting its potential as a life-saving treatment for xeroderma pigmentosum patients.

An association between confluent and reticulated papillomatosis (CARP) and obesity has been reported; however, the relationship between CARP treatment response and obesity remains unknown. A retrospective chart review was performed of 92 pediatric patients diagnosed and treated for CARP during a 7-year period at a single institution. Twenty-three non-obese patients responded to therapy at a higher rate compared to 69 obese patients (100% vs. 77.5%; p = 0.009) regardless of treatment duration, treatment type, age, or sex, based on multivariable analysis. Our results demonstrate an association between obesity and poorer treatment response necessitating prospective research to better investigate the relationship between CARP severity, weight class, and optimization of medical therapy.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of non-Hodgkin lymphoma characterized by subcutaneous nodules, indurated plaques, erythema, and cellular infiltrates in the subcutaneous fat. Biopsies show neoplastic cells expressing cytotoxic T-cell markers and displaying moderate cytologic atypia while sparing the dermis and epidermis and showing variable degrees of necrosis, hemorrhage, and inflammatory changes. We describe a pediatric case of SPTCL in a 6-year-old boy, presenting with an unusual targetoid plaque and systemic symptoms, who showed significant improvement on systemic immunosuppressants without chemotherapy. This case underscores the potential for atypical presentations of SPTCL and the efficacy of immunosuppressive therapy in achieving favorable clinical outcomes, adding to the understanding of SPTCL's clinical diversity and treatment strategies.

Background/objectives: Anaplastic large cell lymphomas (ALCLs) present unique challenges due to their clinical and genetic heterogeneity. This study investigated the clinical characteristics of children diagnosed with systemic ALCL.

Methods: Retrospective data from 14 pediatric patients diagnosed with systemic ALCL at Valme University Hospital were studied. Demographic, clinical, and treatment data were collected and statistically analyzed.

Results: The mean age at diagnosis was 8.5 years, with a male predominance (78.6%). Cutaneous presentation occurred in 35.7% of cases, with characteristic rapidly growing subcutaneous nodules. B symptoms were present in 57.1% of patients, while 100% exhibited nodal involvement. Visceral and bone marrow involvement was observed in 71.4% and 7.1% of patients, respectively. Central nervous system (CNS) involvement was absent. Anaplastic lymphoma kinase (ALK) rearrangement was positive in all cases. Anthracycline-based chemotherapy resulted in 100% 5- and 10-year overall survival rates.

Conclusions: Systemic ALCL in children often presents with advanced-stage disease, with cutaneous involvement in a significant proportion of cases. Prompt recognition of skin lesions is vital to expedite diagnosis and treatment initiation, ultimately improving patient outcomes. This study underscores the importance of vigilance and early intervention in managing pediatric ALCL.

This single-institution retrospective cohort study evaluates the risk of thromboembolic events (TE) in patients with juvenile dermatomyositis (JDM) treated with intravenous immunoglobulin (IVIg). We found no difference in the occurrence of TE in JDM patients treated with IVIg as compared to those without IVIg exposure; both cohorts had no TEs. Patients in both JDM cohorts had matched levels of other clotting risk factors, such as periods of limited mobility and hormonal contraceptive use. This research supports the growing body of evidence in the adult DM population demonstrating no significantly increased risk of TE attributable to IVIg and suggests that the occurrence of TE in JDM is low.

Malnutrition has been reported in congenital ichthyoses in several studies, but its prevalence in Indian children with congenital ichthyoses (CI) as compared to unaffected children is unknown. The objective was to assess the prevalence of malnutrition in 32 children with CI and matched healthy controls and to study the correlation between clinical severity of ichthyosis using visual ichthyosis index severity (VIIS) score with malnutrition, biochemical parameters (hemoglobin, vitamin D, protein and albumin). Malnutrition was detected in 46.8% (15/32) [wasting 40.6%; stunting 37.5%] of children with CI and 18.8% (6/32) (wasting 15.6%; stunting 12.5%) of controls (p value < 0.001) and there was positive correlation between the severity of ichthyosis and malnutrition (p value < 0.001). Children with ichthyosis are at increased risk of malnutrition, and early identification and treatment of malnutrition could improve the severity of ichthyosis and growth potential.