Inter- and intra-observer variability in the selection of therapy for infantile hemangiomas among pediatric dermatologists in Spain.

BACKGROUND: Guidelines and expert recommendations on infantile hemangiomas (IH) are aimed at increasing homogeneity in clinical decisions based on the risk of sequelae. OBJECTIVE: The objective was to analyze the inter- and intra-observer agreement among pediatric dermatologists in the choice of treatment for IH. METHODS: We performed a cross-sectional inter-rater and intra-rater agreement study within the Spanish infantile hemangioma registry. Twenty-seven pediatric dermatologists were invited to participate in a survey with 50 clinical vignettes randomly selected within the registry. Each vignette contained a picture of an infantile hemangioma with a clinical description. Raters chose therapy among observation, topical timolol, or oral propranolol. The same survey reordered was completed 1 month later to assess intra-rater agreement. Vignettes were stratified into hemangioma risk categories following the Spanish consensus on IH. The agreement was measured using kappa statistics appropriate for the type of data (Gwet's AC1 coefficient and Gwet's paired t test). RESULTS: Twenty-four dermatologists completed the survey. Vignettes represented 7.8% of the Spanish hemangioma registry. The inter-rater agreement on the treatment decision was fair (AC1  = 0.39, 95% confidence interval [CI]: 0.30-0.47). When stratified by risk category, good agreement was reached for high-risk hemangiomas (AC1  = 0.77, 95% CI: 0.51-1.00), whereas for intermediate- and low-risk categories, the agreement was only fair (AC1 0.31, 95% CI: 0.16-0.46 and AC1  = 0.38, 95% CI: 0.27-0.48, respectively). Propranolol was the main option for high-risk hemangiomas (86.4%), timolol for intermediate-risk (36.8%), and observation for low-risk ones (55.9%). The intra-rater agreement was good. The inter-rater agreement between pediatric dermatologists on the treatment of IH is only fair. Variability was most significant with intermediate- and low-risk hemangiomas.

Craniofacial hyperhidrosis: Clinical characteristics and response to treatment in a cohort of 97 patients treated with oral oxybutynin.

The term craniofacial hyperhidrosis (HH) refers to HH that affects the face and/or scalp. Few studies have focused on this specific location, and even fewer distinguish between the two areas. Our study aims are to describe the clinical characteristics of patients with craniofacial HH, specifying whether the condition affects the scalp, face or both, and to compare these cases with those recorded at other locations. As secondary objectives, we determine the effectiveness and adverse effects of oral oxybutynin (OOx), and assess patients' adherence to treatment. This prospective observational study was carried out with respect to the period 2007-2019. All patients diagnosed with HH of the scalp and/or face and who were treated with OOx at our HH unit were included in the study group. Of 292 patients treated with OOx, 97 (33.2%) had craniofacial HH. Of these, 4 (4.1%) presented HH exclusively on the scalp, 56 (57.7%) exclusively on the face and 37 (38.1%) in both locations. The patients in the latter category (compared with those with exclusively facial HH) were significantly older than the others, had a later onset of HH, a greater frequency of secondary HH, less simultaneous involvement of the classical areas of primary focal HH (the palms, soles, and armpits) and greater generalized HH and of the trunk. No significant differences were observed between the three locations (face, scalp, or both) regarding the efficacy and side effects of OOx. The patients with exclusively facial HH presented greater adherence to treatment.

Survival study of treatment adherence by patients given oral glycopyrrolate for hyperhidrosis following treatment failure with oral oxybutynin.

Oral anticholinergics such as oxybutynin (OOx) and glycopyrrolate (OGly) are frequently used in the management of hyperhidrosis. Although OOx is considered currently the anticholinergic drug of first choice, OGly is a safe and effective alternative if OOx fails. The aim of this study was to identify the main variables associated with treatment adherence by patients receiving OGly, for whom previous treatment with OOx had failed. A prospective study was conducted of patients with hyperhidrosis receiving treatment with OGly in the period 2012 to 2019. Epidemiological variables, treatment details, effectiveness and adverse effects were recorded. A total of 58 patients (41 women), with a mean age of 35.9 years, were included in the study. The median follow-up period was 32 months. At 3 months, 70.7% of the patients had responded to treatment (excellent response: 75.6%), and adverse effects were reported by 70.7%. At 12 months, 53.4% had responded (excellent response: 74.2%), with adverse effects in 70.9%. The variables associated with poorer adherence were affected areas: palms of the hands, soles of the feet and armpits. The only variable associated with greater adherence was the generalized presence of hyperhidrosis. Our results provide valuable insights into the outcomes achieved when OGly is used to treat hyperhidrosis.

Adjusting oral glycopyrrolate medication for hyperhidrosis to reflect seasonal temperature variations.

The condition of most patients with hyperhidrosis (HH) is known to worsen with increased temperature. However, most prior studies of oral glycopyrrolate (OGly) for the treatment of HH have assumed a stable treatment protocol, without taking into account seasonal variations in temperature. The main aim of this study is to evaluate the outcomes derived from performing a seasonal adjustment of the dose of OGly for patients with HH. A prospective study of patients who began OGly for HH, and maintained treatment for at least 1 year, was performed. All patients had experienced treatment failure with oral oxybutynin. All were recommended to vary the dose of medication according to the time of year. Of the 35 patients included in the study, 20 (57.14%) varied the dose. Those with palmar and plantar HH had a greater propensity to do so. The patients who varied the dose according to the time of year were significantly more likely to report an "Excellent" response after 12 months of treatment. The frequency of adverse effects was 71.4% with no significant differences among the study groups. However, there was a significantly higher proportion of adverse effects other than oral xerosis in the group that did not vary the dose.

[Incontinentia pigmenti. A descriptive study of experience in two different hospitals]. / Incontinencia pigmenti. Estudio descriptivo de la experiencia en dos centros hospitalarios.

INTRODUCTION: Incontinentia pigmenti is a rare genodermatosis of inheritance linked to the Xchromosome that affects tissues derived from ectoderm. The aim of the study is to review, as completely as possible, the cases diagnosed in paediatric patients in two hospitals. MATERIAL AND METHODS: A retrospective cross-sectional study was carried out, using the clinical, analytical, radiological, and genetic data of paediatric patients diagnosed with incontinentia pigmenti from 2004 to 2018. The data collected were analysed and evaluated at a multidisciplinary level. RESULTS: A total of thirteen patients diagnosed with incontinentia pigmenti were included in the study. All of them were female. A genetic study was performed on 11 patients, which confirmed findings compatible with incontinentia pigmenti in 10 of them. Extracutaneous involvement associated with the disease was observed at neurological level (radiological findings in 6 cases, and clinical expression in 3 of them), ophthalmological level (4 cases), dental level (7 cases), and haematological level (4 cases). CONCLUSIONS: A presentation is given of the most complete study published so far of incontinentia pigmenti in Spain. In this study, the results of the disease manifestations were similar to the largest case series published internationally, which reinforces the importance of a multidisciplinary study and follow-up.

Use of topical rapamycin in the treatment of superficial lymphatic malformations.

BACKGROUND: The superficial lymphatic component of vascular malformations poses a significant treatment challenge. It is responsible for the majority of symptoms presented, and to date, there is no consensus regarding treatment. OBJECTIVE: To evaluate the effectiveness of topical rapamycin in treating superficial lymphatic malformations (LM). METHODS: A case series study was performed of patients with superficial LM, treated with topical rapamycin. The clinical characteristics of patients and the concentration and application mode of the drug were recorded. The changes in the signs and symptoms observed and associated adverse effects were noted and analyzed. RESULTS: The study population consisted of 11 patients of an average age of 10.5 years. All were treated with topical rapamycin: 6 patients with a 1% concentration, 1 with a 0.8% concentration, and 4 with a 0.4% concentration. Changes in the clinical appearance of the lesions were observed in all patients. The associated symptoms, present in 9 of 11 patients, improved in every case. The mean follow-up time was 16.1 months. LIMITATIONS: This study is retrospective, with a small sample size and considerable heterogeneity of lesions and treatment approaches. CONCLUSION: Treatment with topical rapamycin modifies the clinical appearance and alleviates symptoms of superficial LM.

Adjusting oral oxybutynin medication for hyperhidrosis to reflect seasonal temperature variations.

Most studies of oral oxybutynin (OOx) for the treatment of hyperhidrosis (HH) have assumed a stable treatment protocol, without taking into account adverse effects (AE) or seasonal variations in temperature. The objective is to evaluate adjusting the dose of OOx according to the time of year. Prospective study of patients who began OOx for HH between 2007 and 2017, and maintained treatment for at least 1 year, with a progressively increasing dose was performed. All patients were recommended to vary the dose of medication according to the time of year. Baseline epidemiological data, the response to treatment and AE were analyzed. About 122 patients were included (average age of 33.8 years). Up to 60.7% varied the dose. Significantly better results were obtained in the group that varied the dose. Twenty patients suspended the treatment in winter. Among them the adjusted OR was 3.04 (95% CI 1.24-7.45) for an excellent response. The frequency of AE was 74.6% with no differences among groups. Most patients are able to control their HH without requiring the same dose of OOx throughout the year. Given that the possible AE of OOx are dose-dependent, it seems reasonable to vary it according to the time of year.

Microcystic Lymphatic Malformation Successfully Treated With Topical Rapamycin.

Microcystic lymphatic malformations (MLM) are low-flow vascular malformations composed of multiple small cysts. MLM usually affect deep-lying structures, which makes their treatment even more difficult and complex. A novel and interesting treatment is rapamycin, a mammalian target of rapamycin inhibitor that when orally administrated has offered favorable results. However, until recently, topical rapamycin had not been used in the treatment of MLM. Case 1 is a girl aged 13 years with extensive MLM affecting the muscles in the right buttock. The patient had received frequent cycles of cryotherapy, but they had failed to control the associated symptoms. In the previous 12 months, the patient had reported greater discomfort, swelling, exudate, and superinfection of the affected region. Because no specific treatment has yet been approved for MLM, and as a step before the use of aggressive systemic or intralesional treatments, it was decided to initiate treatment with 1% rapamycin ointment. After 4 months of treatment, the patient presented a marked improvement, with a significant reduction of associated complications and no major side effects. Case 2 is a boy aged 5 years who underwent surgery for an intergluteal lipoblastoma at 3 weeks of life and developed a MLM on the scar 6 months afterward. The lesion showed slow growth and continuous exudation with frequent episodes of superinfection. Treatments with laser multiplex and intralesional bleomycin were performed unsuccessfully. In the previous 4 months, the patient had been treated with 1% rapamycin ointment with significant improvement and no side effects.

Initial study of transdermal oxybutynin for treating hyperhidrosis.

Oral oxybutynin for treating hyperhidrosis is effective and safe. Its side-effects are mild but frequent so we consider whether transdermal oxybutynin (considered to have a better side-effect profile) could be an alternative for treating hyperhidrosis. During 2015, a prospective study was conducted. Epidemiological variables, effectiveness (using the Hyperhidrosis Disease Severity Scale) and tolerance to transdermal oxybutynin were compiled concerning two different groups (patients previously treated or untreated with oral oxybutynin), at baseline, and at 3 and 12 months. Seven previously treated and six previously untreated patients were included. Five patients in the first group discontinued the treatment within 3 months. Of the two remaining patients, one reported ineffectiveness and the other obtained an excellent response but discontinued due to local irritation. Among the untreated patients, two showed no response and four experienced improvement (three with "partial response" and one with "excellent response"). All patients discontinued treatment within 12 months. No major adverse effects were observed. The absence of active metabolites after transdermal oxybutynin could result in less effectiveness than oral oxybutynin, although it is usually well tolerated. In conclusion, transdermal oxybutynin could have low effectiveness for the treatment of hyperhidrosis in patients following intolerance to oral oxybutynin but could provide good results in patients who have never tried systemic drugs.

Refractory acne keloidalis nuchae treated with radiotherapy.

Acne keloidalis nuchae (AKN) is difficult to treat, and the clinical response is usually poor. Few reports have been published about scarring alopecia treated with radiotherapy (RT). We report a case of AKN that had been refractory to various other treatments but which responded to RT with excellent clinical and cosmetic results.

Oral oxybutynin for the treatment of hyperhidrosis: outcomes after one-year follow-up.

BACKGROUND/OBJECTIVES: Although many treatments are available to address hyperhidrosis, the results are not always satisfactory. The aim of the study was to assess the effectiveness, optimal dosage regimen and long-term safety of oral oxybutynin in the treatment of hyperhidrosis. METHODS: A retrospective review was performed on 110 patients who underwent treatment for hyperhidrosis between February 2007 and December 2013. Their response to treatment was evaluated using the hyperhidrosis disease severity scale at baseline, 3 and 12 months. Additionally, the safety and effectiveness of different up-dosing and fixed-dose regimens were compared. RESULTS: After 3 months of treatment, 87 of the 110 patients (79%) had responded (63%), which was considered excellent. After 12 months, 63 patients (62%) continued to respond, and the response was considered excellent in 50%. Nine patients were lost to follow up between month 3 and 12. In total, 77 and 70% of the patients who responded at 3 and 12 months, respectively, reported mild adverse events. No serious adverse events were observed. Treatment adherence was significantly higher among patients following the individualised up-dosing regimen. CONCLUSION: Oral oxybutynin may be an effective and safe option for the long-term treatment of hyperhidrosis. To improve treatment adherence, oxybutynin dosing regimens should be individualised on the basis of the patient's tolerance and response.

Individualized Dosing of Oral Oxybutynin for the Treatment of Primary Focal Hyperhidrosis in Children and Teenagers.

BACKGROUND/OBJECTIVES: Oral anticholinergic drugs, such as oxybutynin, are often used in the treatment of hyperhidrosis, but few studies have focused on dosing strategies for children. The objective was to assess the effectiveness and safety of individualized dosing regimens of oral oxybutynin for treating primary focal hyperhidrosis (PFH) in children and teenagers. METHODS: A prospective study was performed including patients who initiated treatment for hyperhidrosis between November 2011 and November 2014. Response to treatment and adverse effects were evaluated using the Hyperhidrosis Disease Severity Scale at baseline and at 3 and 12 months. RESULTS: Of 16 patients included in the study, 15 (93.8%) had responded to treatment at the 3-month follow-up (62.5% with excellent response). At the 12-month follow-up, the 11 patients who continued the treatment were still responding (63.6% with excellent response). Adverse effects were reported for 68.8% of the patients at 3 months and 54.5% at 12 months, with a predominance of oropharyngeal xerosis. No serious adverse effects were observed. CONCLUSION: Dose individualization of oral oxybutynin according to clinical response and tolerance observed in each patient is a useful management strategy in children and teenagers.

Congenital lupus with multiorgan involvement: a case report and review of literature.

Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by transplacental antibodies that can damage fetal tissue and cause various findings. With the exception of congenital heart block, which can be easily recognized at birth because of neonatal cardiac monitoring during the delivery and immediately after birth, most cases of NLE are recognized within days to weeks of life, but fewer than 10 cases with findings present at birth have been reported. We report the case of a newborn with extensive cutaneous eruption at the time of birth and multisystemic involvement, including hematologic, respiratory, hepatic, and neurologic involvement.

Successful treatment of periungual warts with topical cidofovir.

Periungual warts represent a treatment challenge because of its high recurrence rate and recalcitrance. These are benign lesions produced by the human papilloma virus (HPV) that often do not respond to habitual treatment. Cidofovir is a potent antiviral drug that acts inactivating viral DNA polymerase. Topical cidofovir for the treatment of HPV-related cutaneous and mucous lesions is becoming increasingly common. Our aim was to assess the efficacy and safety of cidofovir cream for the treatment of viral periungual warts. We undertook a retrospective observational study of patients with periungual warts who received treatment with topical cidofovir between January 2010 and December 2013 at the Dermatology Service of the Hospital Costa del Sol, Marbella, Spain. Data were recorded about the rate of treatment response, the adverse effects and recurrences, as well as the characteristics of the patient cohort. We identified 41 patients who had received some previous treatment. The concentration of cidofovir was 3% in all cases, usually applied twice a day (in 37 of the 41 cases). A greater or lesser response was noted in 35 cases. There were six recurrences in the follow-up period. Topical cidofovir seems to be a useful alternative for the therapeutic management of recalcitrant periungual common warts that fail to respond to usual treatment. Our experience with the use of this antiviral agent has been satisfactory, although in our opinion, it should be reserved for specific cases as its economical cost represents an important limitation.

Topical cidofovir for plantar warts.

Plantar warts are a common reason for dermatological consultations and their treatment can occasionally be a challenge. Plantar warts are benign lesions produced by the human papillomavirus (HPV) that often fail to respond to habitual treatment. Cidofovir is a potent antiviral drug that acts competitively, inhibiting viral DNA polymerase. Our aim was to assess the efficacy and safety of cidofovir cream for the treatment of viral plantar warts. We undertook a retrospective observational study of patients with plantar warts who received treatment with topical cidofovir between July 2008 and July 2011 at the Dermatology Service of the Hospital Costa del Sol, Marbella, Spain. Data about the rate of treatment response, the adverse effects, and recurrences, as well as the characteristics of the patient cohort, were recorded. We identified 35 patients who had received some previous treatment. The usual concentration was 3% (in 33 of 35 cases), applied twice a day (in 31 of 35 cases). A greater or lesser response was noted in 28 cases. There were two recurrences. Topical cidofovir seems to be a useful alternative for the therapeutic management of recalcitrant plantar common warts that fail to respond to usual treatment.

[Treatment of anogenital warts with topical cidofovir]. / Tratamiento de verrugas anogenitales con cidofovir tópico.

BACKGROUND: Cidofovir is an antiviral agent which inhibits the DNA polymerase of different viruses. Its use has been described for papillomavirus infections. METHODS: Descriptive retrospective study, including patients with difficult to treat anogenital warts who were treated with cidofovir cream. RESULTS: Nine immunocompetent patients were treated, where 6 of them had a complete response and 3 of them a partial response. CONCLUSION: Cidofovir cream could be a useful therapeutic alternative, although further studies are required to establish the best dosage and its cost-effectiveness.

Pediatric tinea faciei in southern Spain: a 30-year survey.

Tinea faciei (TF) is a common clinical form of tinea in children that is frequently misdiagnosed and treated with corticosteroids. No large case series of TF focusing on children have been published. The aim of this study was to analyze the main epidemiologic, clinical, and microbiologic features of TF in children over a period of 30 years and compare these features with those of other tineas. We undertook a retrospective study of 818 cases of tinea in children at a referral hospital in southern Spain, diagnosed between 1977 and 2006, concentrating for this study on TF. Of the 73 cases of TF diagnosed, 50.7% were in girls. Most children (46.6%) were 4 to 9 years old. At the time of diagnosis, 29.2% of the cases had been treated with topical steroids. The most frequently isolated dermatophyte was Trichophyton mentagrophytes, which was isolated significantly more frequently in TF than in the other tineas. Cases of TF in children were not extremely unusual, emphasizing that TF must be considered in children with inflammatory facial eruptions. This consideration and the more-frequent use of mycologic tests can help achieve the correct diagnosis, when present.

Giant melanoma and depression.

A 29-year-old female patient presented with a giant melanoma on the external side of the left arm and concomitant multiple visceral metastases. The patient also had major depression and had avoided a consultation despite the large size of the lesion, delaying the diagnosis and treatment of her melanoma, which as far as we know is the largest, primary cutaneous melanoma ever reported. Excision of the tumor was performed as a palliative treatment and she died 1 month later. Depression has been identified as a factor that can worsen the course of a disease, although in this case it could have been the direct consequence of the dermatologic process.