Neoadjuvant intralesional methotrexate in cutaneous squamous cell carcinoma: a comparative cohort study.

BACKGROUND: Intralesional methotrexate (MTX-il) has been used as neoadjuvant therapy for keratoacanthoma but has only been utilized in a few isolated cases of cutaneous squamous cell carcinoma as neoadjuvant therapy (cSCC). OBJECTIVES: The objective of this study was to evaluate the effectiveness in clinical practice of presurgical MTX-il infiltration to reduce the size of the cSCC. Safety and the impact on subsequent reconstructive surgical techniques was also assessment. METHODS: Single, retrospective, observational study of two historical cohorts differentiated in time. Subjects included were diagnosed with infiltrating cSCC. Patients included in group-A received neoadjuvant MTX-il and patients included in group-B underwent scheduled surgery without prior infiltration. Univariate and multivariate analyses were performed. RESULTS: Group-A patients (n = 43) showed an average reduction in the tumour area of 0.52 cm(2) , while in group-B (n = 43), the area increased by 0.49 cm(2) . A multivariate linear regression analysis demonstrated that MTX-il was the only independent variable that significantly reduced the tumour size [mean 42.6% (95% CI: 31.17-54.03)]. Tumours ≥2 cm in size required significantly a lower percentage of complex reconstructions (P = 0.026). Lower lip tumours showed a higher reduction in group treated with MTX-il (P = 0.045). The only complication observed was discomfort during methotrexate infiltration (60.47%). CONCLUSIONS: Neoadjuvant MTX-il reduced the presurgical size of cSCC lesions and could simplify their subsequent surgery.

Facial Angiofibroma Severity Index (FASI): reliability assessment of a new tool developed to measure severity and responsiveness to therapy in tuberous sclerosis-associated facial angiofibroma.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by the development of multisystem hamartomatous tumours. Topical sirolimus has recently been suggested as a potential treatment for TSC-associated facial angiofibroma (FA). AIM: To validate a reproducible scale created for the assessment of clinical severity and treatment response in these patients. METHODS: We developed a new tool, the Facial Angiofibroma Severity Index (FASI) to evaluate the grade of erythema and the size and extent of FAs. In total, 30 different photographs of patients with TSC were shown to 56 dermatologists at each evaluation. Three evaluations using the same photographs but in a different random order were performed 1 week apart. Test and retest reliability and interobserver reproducibility were determined. RESULTS: There was good agreement between the investigators. Inter-rater reliability showed strong correlations (> 0.98; range 0.97-0.99) with inter-rater correlation coefficients (ICCs) for the FASI. The global estimated kappa coefficient for the degree of intra-rater agreement (test-retest) was 0.94 (range 0.91-0.97). CONCLUSIONS: The FASI is a valid and reliable tool for measuring the clinical severity of TSC-associated FAs, which can be applied in clinical practice to evaluate the response to treatment in these patients.

Current options for the treatment of facial angiofibromas.

Facial angiofibromas are hamartomatous growths that are closely associated with tuberous sclerosis complex and, in fact, they constitute one of the main diagnostic criteria for that disease. These lesions composed of blood vessels and fibrous tissue appear on the face at an early age. Since they have important physical and psychological repercussions for patients, several treatment options have been used to remove them or improve their appearance. However, the lack of treatment guidelines prevents us from developing a common protocol for patients with this condition. The present article aims to review the treatments for facial angiofibromas used to date and to propose a new evidence-based treatment protocol.

Spanish adaptation of the European guidelines for the evaluation and treatment of actinic keratosis.

Current trends in our setting indicate that the prevalence of actinic keratosis and similar diseases will increase in coming years and impose a greater burden on health care resources. A long list of clinical features must be taken into account when approaching the treatment of actinic keratosis. Until recently, therapeutic approaches focused solely on ablative procedures and the treatment of individual lesions and did not take into account areas of field cancerization. Now that the therapeutic arsenal has grown, standardized criteria are needed to guide the optimal choice of treatment for each patient. The elaboration of evidence-based consensus recommendations for the diagnosis and treatment of actinic keratosis generates knowledge that will help clinicians to deliver the highest level of care possible, standardizing decision-making processes and enhancing awareness among all the health professionals involved in the care pathway.

Association between bullous pemphigoid and neurologic diseases: a case-control study.

INTRODUCTION: In the past 10 years, bullous pemphigoid has been associated with other comorbidities and neurologic and psychiatric conditions in particular. Case series, small case-control studies, and large population-based studies in different Asian populations, mainland Europe, and the United Kingdom have confirmed this association. However, no data are available for the Spanish population. MATERIAL AND METHODS: This was an observational, retrospective, case-control study with 1:2 matching. Fifty-four patients with bullous pemphigoid were selected. We compared the percentage of patients in each group with concurrent neurologic conditions, ischemic heart disease, diabetes, chronic obstructive pulmonary disease, and solid tumors using univariate logistic regression. An association model was constructed with conditional multiple logistic regression. RESULTS: The case group had a significantly higher percentage of patients with cerebrovascular accident and/or transient ischemic attack (odds ratio [OR], 3.06; 95% CI, 1.19-7.87], dementia (OR, 5.52; 95% CI, 2.19-13.93), and Parkinson disease (OR, 5; 95% CI, 1.57-15.94). A significantly higher percentage of cases had neurologic conditions (OR, 6.34; 95% CI, 2.89-13.91). Dementia and Parkinson disease were independently associated with bullous pemphigoid in the multivariate analysis. CONCLUSIONS: Patients with bullous pemphigoid have a higher frequency of neurologic conditions.

An Internet-based programme to promote adequate UV exposure behaviour in adolescents in Spain.

BACKGROUND: The exponential rise in the incidence of cutaneous cancers underscores the need to promote primary prevention. Adolescents are highly familiar with the use of Internet, and new technologies and Internet systems have proven especially useful to educate this age group in other health problems. To date, there have been no reports of Internet-based primary prevention campaigns for adolescents against skin cancer. OBJECTIVES: To determine the knowledge and behaviour of a Spanish adolescent population in relation to sun exposure through an Internet-based system and to describe the use of an Internet-based school intervention programme to improve the sun exposure knowledge and behaviour in adolescents. METHODS: The cross-sectional study included 2170 school pupils aged between 12 and 16 years from public secondary schools. Pupils in the participating centres completed the survey via the website, which contained a total of 35 questions and three information blocks. For the intervention programme, a sub-sample of 12 secondary schools (total of 1290 pupils) was randomly selected. RESULTS: About 56.7% of the female pupils and 52.6% of the male pupils reported having suffered from sunburn in the previous summer. Sunburn risk increased with the frequency of sun exposure between 12 and 6 pm (OR of 3.59), null knowledge of the negative effects of sun exposure (OR of 2.102), the use of sun protection cream (OR of 0.695 for non-use) and physical sun protection measures (OR of 2.21 for 0-1 physical measures). A significant reduction in self-reported sunburns was observed in the quasi-experimental group in comparison with controls after adjusting for sex and inland or coastal location of the centre (OR = 0.45, 95% CI = 0.23-0.87, P = 0.018). Adjusted OR showed that there was a significant improvement in the use of physical measures, use of sun cream, frequency of sun cream application every 2 h and use of protection on cloudy days. CONCLUSIONS: This is the first study to demonstrate that a programme entirely conducted via Internet significantly reduces by half self-reported sunburn risk in an adolescent population achieving very high satisfaction rates.

Atopic dermatitis: update and proposed management algorithm.

Atopic dermatitis is a chronic inflammatory disease that affects 20% of children and almost 3% of adults and is associated with considerable impairment of quality of life for both patients and their families. While the condition resolves spontaneously after puberty in over 75% of cases, it can persist into adulthood. Furthermore, in young children severe forms can have serious health consequences and affect social development. There are no appropriate guidelines on how to handle cases that do not respond to routine treatment. In this article, we review the current treatments for moderate to severe atopic dermatitis, describe our experience with this disease, and propose a management algorithm.

Sustained clinical effectiveness and favorable safety profile of topical sirolimus for tuberous sclerosis - associated facial angiofibroma.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder characterized by the development of multisystem hamartomatous tumours. Facial angiofibroma appears in up to 80% of patients and has a considerable psychological impact. Various invasive procedures have been used, although they show limited effectiveness and potential adverse effects. OBJECTIVES: To evaluate the sustained clinical benefits and safety profile of topical sirolimus applied to treat facial angiofibromas. METHODS: This study was a non-blinded, uncontrolled case-series comprising 10 patients with TSC-associated facial angiofibroma that was treated with 0.4% sirolimus ointment 3 times a week for 9 months. Patients were clinically evaluated at baseline and at 6, 12, 24 and 36 weeks. Plasma levels of sirolimus were determined. RESULTS: A sustained improvement was observed in erythema and in the size and extension of the lesions as early as the first weeks of treatment. Sirolimus plasma levels remained below detection limits (0.3 ng/mL) in all cases. The formula was well-tolerated with no local or systemic adverse effects. CONCLUSIONS: Topical sirolimus seems to be an effective and safe medical alternative to surgery or laser-based treatments in patients with TSC-associated facial angiofibromas.

[Treatment of acne in daily clinical practice: an opinion poll among Spanish dermatologists]. / Tratamiento del acné en la práctica clínica habitual: encuesta de opinión entre los dermatólogos españoles.

BACKGROUND AND OBJECTIVES: the aim of this study was to determine the approaches used to treat acne in clinical practice by Spanish dermatologists. A secondary objective of the study was to compare the practices of Spanish dermatologists with the Acne Global Alliance treatment algorithm and develop a Spanish treatment algorithm for acne. PATIENTS AND METHODS: a multicenter, cross-sectional survey was undertaken among 872 Spanish dermatologists. The study comprised 4 randomly distributed questionnaires. The first 3 contained the following case descriptions: severe acne associated with masculinization (case 1), mild comedonal acne (case 2), and moderate papulopustular acne (case 3). The fourth questionnaire contained 5 photographs of different types of acne and an algorithm containing various treatment options, from which dermatologists were asked to choose the most appropriate. RESULTS: for case 1, 55% of dermatologists chose oral antiandrogens/contraceptive drugs plus topical retinoids or topical benzoyl peroxide/antibiotics. In case 2, 62% chose topical retinoids and, in case 3, 68% chose systemic antibiotics plus benzoyl peroxide. Combination therapy was considered the treatment of choice in all types of acne, with topical retinoids as the initial treatment option for mild and moderate forms and the preferred option for maintenance therapy. CONCLUSIONS: in general, Spanish dermatologists treat acne in accordance with the Acne Global Alliance treatment algorithm. The varying opinions reported in response to open questions, however, confirm the need to unify criteria for the treatment of acne.

[Comorbidities in psoriasis]. / Comorbilidades en psoriasis.

Although in most majority the psoriasis presents/displays a clinical cutaneous today fundamentally we know that it can be associated to other diseases extracutaneous, as much you will articular, digestive, metabolic, cardiovascular and even psychic. We reviewed in this article the atiopathogenics bases and the risks that the psoriáasics patients must to suffer these comorbidities.

Etanercept: efficacy and safety.

OBJECTIVE: To evaluate the efficacy and safety of etanercept in the treatment of patients with moderate to severe plaque psoriasis. METHODS: An observational, longitudinal, and retrospective study involving two groups of dose of treatment with etanercept (50 vs. 100 mg/week). The selected patients presented moderate to severe plaque psoriasis, and they had received treatment with the mentioned drug. A total of 58 patients were included in the study. The efficacy of the drug was evaluated by measuring the psoriasis area and severity index (PASI), body surface area (BSA) and physician's global assessment (PGA) in weeks 8, 16, 24, 32, 40 and 48. RESULTS: A statistically significant improvement was observed in the PASI, BSA and PGA indexes after 24 and 48 weeks of therapy. As for PASI, and after 48 weeks of treatment, PASI 50, 75 and 90 were 100.0%, 92.3% and 69.2%, respectively. In our series, etanercept 50 mg/week reached the same results after 48 weeks as etanercept 100 mg/week, though the initial response was faster in the last group. The PASI, BSA and PGA indexes diminished significantly with the treatment, though without statistically significant differences between both groups. As for the safety, etanercept was well tolerated, and no serious adverse events were recorded. There were no cases of tuberculosis or opportunistic infections. CONCLUSIONS: Our study confirms the efficacy and safety outcomes of the clinical trials of etanercept in psoriasis with both doses of treatment. As for the safety, etanercept was well tolerated, and all the recorded adverse events coincided with the known potential side-effects of treatment.

[Efficacy of short- and long-term efalizumab]. / Eficacia de efalizumab a corto y largo plazo.

T cells play an important role in the immune system and in the inflammatory response that determines the development and maintenance of psoriasis plaques. Better understanding of the pathophysiology of this disease has led to the development of specific biological treatments aimed at patients with extensive psoriasis. Traditionally, psoriasis has been treated with drugs which, in spite of their efficacy, have a toxicity associated to their long-term use. Thus, they cannot be used safely, comfortably or efficiently in many patients. Efalizumab, a biological agent specifically and selectively directed towards blocking the key steps in the pathogenesis of psoriasis, has been shown to be effective and safe in the short and long term in the treatment of psoriasis in more than 15 phase I, II and III clinical trials. In this article, the results of efficacy at 12 weeks, 6 months and three years are reviewed. Efalizumab arises as an important addition to the dermatological pharmacopoeia for the long-term treatment of psoriasis.

Granulomatous slack skin disease in a child: the outcome.

Granulomatous slack skin syndrome is a rare clinical and pathologic disorder. Only 42 patients have been reported, one of whom we described in 1997--the only child so far reported. We now describe the evolution of this patient and the transformation of the disease into a peripheral T-cell lymphoma, and the complications resulting in the child's death.

[New clinical aspects of dermatomycosis.]. / Nuevos aspectos clínicos de las dermatomicosis.

Usually the clinical diagnostic of tinea is easy for the dermatologist, but occasionally, mostly as a result of an unsuitable treatment, the cutaneous lesions can show up atypical forms. In this article tinea capitis in the elderly, tinea follicularis et granulomatosa of the legs, tinea faciei and tinea incognito are discussed.

[Epidermal nevus syndrome (of Solomon, Fretzin and Dewald)]. / Syndrome du naevus épidermique (de Solomon, Fretzin et Dewald).

Epidermal naevus syndrome is an entity which has been well established by Solomon, Fretzin and Dewald since 1968, presenting cutaneous, visceral, ocular, osseous and neurological malformations. We report a case with multiple osseous manifestations affecting the entire skeleton, but mainly the skull (osteolysis with absence of frontal bone), the vertebral column (scoliosis with triple deviation), the upper right limb (demineralisation and multiple pathological fractures) and lower members (fractures of the right and left femur) with enlargement of the entire ventricular system and well marked scissures, signs of an cortico-subcortical atrophy shown by scanogram.

[Alagille's syndrome. Clinical and histo-pathologic study]. / Le syndrome D'alagille. Etude clinique et histo-pathologique.

Alagille 's syndrome, described in 1970, associates a chronic intrahepatic cholestasis (hypoplasia of the interlobulary biliary ducts), vertebral and visceral malformations, retarded physical, mental and sexual development and neonatal jaundice. A series of cutaneous manifestations are reviewed in their clinical, histological and ultrastructural aspects. Its relation with the zinc deficiency syndrome is discussed.