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.

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.

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.

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.

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.

Temporary localized hypertrichosis after henna pseudotattoo.

We report the case of a 5-year-old boy who had a temporary dragon-shaped henna pseudotattoo, reinforced 4 days later. Two weeks later, as the pseudotattoo began to disappear, hypertrichosis developed in the area corresponding to the previous psuedotattoo. Skin biopsy showed an increase in vellus hair follicles, with slight peripheral fibrosis. After 4 months, the hypertrichosis resolved spontaneously.

[Spindle-cell carcinoma in situ treated with 5% imiquimod]. / Carcinoma espinocelular in situ tratado con imiquimod al 5 %

We present the case of a 70-year-old woman with no personal history of interest who presented to her physician with a plaque in the right infraumbilical region whose largest diameter measured nine centimeters. The clinical and histological diagnosis was Bowen's disease. She was treated with 5 % imiquimod cream, once a day, four times a week, for eight weeks, with an excellent response. One year after treatment, no new lesions have appeared in the treated area.