Consensus statement from the AEDV Psoriasis Working Group (SWG) and Pediatric Working group (PWG) on the management of pediatric psoriasis. / Documento de consenso del Grupo Español de Psoriasis (GPS) y del grupo Español de Dermatología Pediátrica (GEDP) de la AEDV sobre el manejo de la psoriasis pediátrica.

Justification and objectives: The Spanish Academy of Dermatology and Venereology (AEDV) Psoriasis and Pediatric Working Groups (PSW and PWG) have developed a set of recommendations for the management of pediatric psoriasis based on the best available evidence and experts' opinion. Methodology: The methodology of nominal groups was followed, with help from a scoping review. A coordinator was designated, and a group of experts was selected based on their experience and knowledge on the management of psoriasis. The coordinator defined both the objectives and the key points of the document. Then, with help from a documentalist, a systematic literature review was conducted across Medline, Embase and Cochrane Library until May 2023. Systematic literature reviews, meta-analyses, and observational studies were included. National and international clinical practice guidelines and consensus documents were reviewed. With this information, the coordinator proposed preliminary recommendations that were discussed and modified in a nominal group meeting with all experts. After several review processes, which included an external review, the final document was generated. Results: Practical recommendations on the evaluation and management of patients with pediatric psoriasis are presented in association with other AEDV documents. The evaluation of the pediatric patient, the definition of the therapeutic objectives, the criteria for indication and selection of treatment are addressed. Practical issues such as therapeutic failure, response maintenance, comorbidity and risk management are also included.

Off-label Prescription in Paediatric Dermatology: A Retrospective Observational Study in a Tertiary Hospital.

Off-label prescription in paediatric patients is common, where some studies indicate that dermatological conditions are more prone to off-label treatment. This is the first study to analyse the prevalence of off-label prescription in paediatric dermatology consultation. This retrospective observational study was performed using the medical records of paediatric patients who were evaluated in a paediatric dermatological consultation in Pontevedra University Hospital, Pontevedra, Spain. Of the 468 patients reviewed, 186 prescriptions were issued and 51.10% were off-label prescription drugs. The dermatological conditions for which off-label prescription was most common were atopic dermatitis (29.0%), followed by warts (12.9%) and infantile haemangiomas (11.8%). With respect to drugs, topical tacrolimus (23.7%) was the most frequently prescribed off-label drug. The main reason for prescribing an off-label drug was for a disease not included on the label (62.4%), followed by issuing it at a lower age than authorized (55.9%). There was a significant association between a higher percentage of off-label prescription and younger age (p < 0.001), and the treatment of vitiligo, infantile haemangiomas and warts (p < 0.001). Likewise, the off-label prescription was significantly more common in the case of topical terbinafine, timolol, desloratadine and topical salicylic acid (p < 0.001). To conclude, off-label prescription is predominant in paediatric dermatology, as observed in 51.1% of our patients.

Hospitalization in Patients with Psoriasis: Impact of Biological Therapies on Temporal Evolution.

BACKGROUND: Psoriasis is an immunomediated disease mostly controlled at the outpatient level, although there is a low percentage of patients that require systemic drugs or even hospitalization for an adequate control. Biological drugs have represented a turning point in its treatment. So far, despite the growing interest in psoriasis and its management with biological therapies, there is a lack of studies focusing on their impact on hospitalization, a relevant issue to patients and to the sustainability of our healthcare system. OBJECTIVE: In this study, we aimed to describe the temporal evolution of the hospitalizations of patients with psoriasis throughout the period between eight years before the commercialization of the first biological drugs and present, and secondly, whether this market irruption was related to a decrease in the number of admissions. METHODS: Data was collected retrospectively from the Dermatology department of the Complexo Hospitalario Universitario de Ponte- vedra (CHUP) including patients of all ages with a diagnosis of psoriasis and at least one admission to the department of Dermatology along the study period. We established different time periods for comparing the average hospitalizations per 100,000 inhabitants-year and the average stay, considering that the first biologic drug marketed for the treatment of psoriasis was in 2004. RESULTS: Regression models indicated a significant change in the temporal trend of the hospitalization rate per 100,000 inhabitants-year starting in 2004. In all cases, a gradual and significant decrease in the number of admissions per 100,000 inhabitants-year and in the average hospitalization rate per psoriasis per 100,000 inhabitants-year along the study period were found. There was also a significant decrease in medical hospitalizations and medical hospitalizations excluding psoriasis throughout the study period. CONCLUSIONS: In our study population hospitalizations for psoriasis descended progressively and significantly from 2004. So far there are no extensive data on the impact of biological therapies on psoriasis hospitalization. J Drugs Dermatol. 2021;20(2):208-214. doi:10.36849/JDD.4931.

Multifocal granular cell tumors in a 10-year-old boy.

Multifocal granular cell tumor is a poorly understood entity. Its importance lies in its association with several genetic disorders and the challenging distinction between multifocal and metastatic granular cell tumor. Herein, we report an unusual case of nonmalignant, multifocal, granular cell tumors affecting the skin, including the scrotum, in a 10-year-old boy.

Multiple Pilomatricomas: A Retrospective Study and Literature Review.

Pilomatricoma is usually a single lesion. However, some patients present with more than one, a fact that has been reported in association with several systemic diseases. The aim of this analysis is to determine the prevalence of multiple pilomatricomas (MP) in our institution as well as to describe their clinical features and associated comorbidities. This is a retrospective analysis reviewing patients with a histological diagnosis of pilomatricoma registered in the database of the Pathology Department of our institution, from January 2005 to March 2017. One hundred ninety-five patients with histological criteria for pilomatricoma were registered. Fourteen (0.7%) of those patients suffered from MP. Among them, the most relevant findings were: 9 (64%) patients were women and 5 (36%) were men. The median age at diagnosis was 16 years. Seven (50%) had 2 pilomatricomas and 7 (50%) had more than 2. Two (14%) suffered from Steinert myotonic dystrophy (SMD). Regarding family background, 3 of them had relatives with pilomatricomas, one of them with pancreatic cancer, and another one with SMD. A total of 36 pilomatricomas were excised from those 14 patients with the following characteristics: The mean lesion size was 12 mm. The majority of the lesions (53%) were located on the head and neck. There was no recurrence or malignant transformation in any patient. Articles reporting on MP are scarce. We communicate 2 new familial MP without associated comorbidities. We would like to highlight that 2 of our patients with MP also developed SMD, an association previously reported. Thus, a thorough physical examination and inquiring about family history are of utmost importance for an early diagnosis of possible associated diseases.

Topical carbocysteine: A new option for the treatment of ichthyosis.

N-acetylcysteine in combination with urea is effective for the treatment of congenital ichthyosis. Although it is well tolerated, its foul smell may compromise treatment adherence. Carbocysteine is a similar molecule without that bad odor. Thus, we have tried a new formula with carbocysteine for the treatment of 4 patients with ichthyosis, with positive results.

Genotype-phenotype correlation in type 1 neurofibromatosis: pMet992del mutation and milder disease.

A few genotype-phenotype correlations have been described in type 1 neurofibromatosis. One deletion, p.Met992del, seems to be responsible for a mild form of the condition, in which there is absence of externally visible neurofibromas. We report a mother and a son with this mutation.

The Cw6 and late-cornified envelope genotype plays a significant role in anti-tumor necrosis factor response among psoriatic patients.

Our aim was to determine whether the HLA-Cw6 and late-cornified envelope (LCE) deletion polymorphisms were related to disease improvement among psoriasis patients treated with anti-tumor necrosis factor (TNF) antibodies. The study included a total of 116 patients. Positive response (68%) was defined as a reduction of at least 75% of the Psoriasis Area and Severity Index (PASI) after 24 weeks of starting the anti-TNF therapy. We found a trend toward a better response among Cw6-positive patients. The frequency of patients who did not reach the PASI75 was higher among the LCE-DD patients (P=0.028; odds ratio=2.45, 95% confidence interval=1.09-5.52). Patients who were Cw6-positive and LCE-I carriers (ID/II) were significantly more likely to reach PASI75 than those who were Cw6-negative and LCE-DD (P=0.034; odds ratio=3.14, 95% confidence interval=1.07-9.24). In conclusion, we found an interaction between the HLA-Cw6 and LCE genotypes on disease improvement among psoriatic patients treated with anti-TNFs.

Congenital malalignment of the great toenail. Report of two cases.

Congenital malalignment of the great toenail is characterized by lateral deviation of nail plates, which are not parallel to the distal phalanx. We report two cases of congenital malalignment of the great toenail in two otherwise healthy children. Because this entity is probably underdiagnosed, a high index of suspicion is required to prevent from diagnostic errors and unnecessary treatments.

Dermatology Self-Medication in Nursing Students and Professionals: A Multicentre Study.

Current evidence shows that the prevalence of self-medication in healthcare professionals and their students is troublingly high despite them knowing the risks involved. There is limited research on self-medication in dermatology, and there are even fewer studies on this practice among nurses and nursing students, despite the potential mucocutaneous health problems that may affect them. The aims of our study were to examine the prevalence of self-medication mainly in the field of dermatology among nurses and nursing students as well as to explore if age or years of professional/academic practice influenced such behaviour. This multicentre cross-sectional study was conducted in 2021. In total, 120 nurses from the University Hospital of Pontevedra and 303 nursing students from the Universities of Vigo and Santiago de Compostela (N-W Spain) participated in this study (n = 423). An ad hoc questionnaire was used to evaluate self-medication decision-making. Self-medication for dermatological diseases was reported by 58.39% (n = 247) of participants. Among our respondents, 44.44% of nurses and 42.68% of students would recommend treatment for skin diseases to a third party. We found a higher prevalence of medication without prescription in nurses than in students (p < 0.001). More experience (p = 0.01) and older age (p < 0.001) were associated with more self-medication in the case of nurses and students, respectively. The prevalence of self-medication and treatment recommendation to a third party are cause for concern. Identifying these situations and associated factors may help to implement evidence-based strategies and education.

Genital primary herpes simplex infection in a 5-month-old infant.

Genital primary herpes simplex infection is very uncommon in infants. Asymptomatic and oral primary infections are the most frequent presentations in this age group. We report a case of genital primary herpetic infection in a 5-month-old male, exclusively breastfed, whose mother suffered from active labial herpes.

NFKBIZ and CW6 in Adalimumab Response Among Psoriasis Patients: Genetic Association and Alternative Transcript Analysis.

BACKGROUND: Nuclear factor (NF)-κB is an essential mediator of the tumor necrosis factor (TNF) pathway, and has been implicated in psoriasis. NFKBIZ is a nuclear inhibitor of NF-κB with a prominent role in the pathogenesis of psoriasis. The genetic variation at the NFKBIZ gene has been associated with the risk of developing psoriasis, and could also contribute to defining the response to anti-TNF biological drugs. OBJECTIVES: The objectives of this study were to determine the association of a common NFKBIZ insertion/deletion (indel) polymorphism (rs3217713) with the response to adalimumab and determine the differences in the relative expression of a NFKBIZ alternative transcript in patients with a positive versus negative response. METHODS: We genotyped a common NFKBIZ polymorphism in 169 psoriasis patients treated with adalimumab classified as responders (n = 120) and non-responders (n = 49), according to whether they had a 75% reduction in the Psoriasis Area and Severity Index score (PASI75) at week 24. The Cw6 polymorphism was also determined and allele and genotype frequencies were compared between the groups. We also determined the rate of the expression of a NFKBIZ transcript lacking exon 10 relative to the normal transcript in 60 patients (27 non-responders). In addition, because the intron indel could affect RNA splicing, we investigated whether the level of the alternative transcript was related to the intronic genotype. RESULTS: The NFKBIZ polymorphism was associated with adalimumab response, with carriers of the deletion allele significantly more frequent among responders (odds ratio = 2.76, 95% confidence interval 1.19-6.43; p = 0.015). The presence of the HLA-CW6 allele was also associated with a positive response in our cohort (p = 0.018). The alternative transcript was amplified in all the samples. We found higher but non-significant values of normal to alternative transcript in responders as well as in NFKBIZ insertion homozygotes. CONCLUSION: Our study supported a significant effect of a common NFKBIZ polymorphism on the response to adalimumab. This result could help to optimize the prescription of this anti-TNF, but requires confirmation in other cohorts.