Dermatofibrosarcoma protuberans in childhood.

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age. The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence. This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options.

Dermatofibrosarcoma Protuberans in Childhood. / Dermatofibrosarcoma protuberans en la infancia.

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age. The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence. This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options.

[Etanercept. Other indications]. / Etanercept. Otras indicaciones.

The use of biological treatments in the field of Dermatology has been recent and priority focus has been given to the treatment of psoriasis. Etanercept has demonstrated its efficacy and safety in this field although it is true that it has been used in many different diseases other than psoriasis with variable results due to its action mechanism and safety profile. We present a case of Hallopeau's Acrodermatitis treated with etanercept with good results. Given the characteristics of chronicity, progressiveness and, in general, poor response to conventional treatments, the use of etanercept is of help in the therapeutic possibilities of Hallopeau's Acrodermatitis. In addition, other possible indications of etanercept in conditions other than Psoriasis are reviewed

Paediatric psoriasis--narrowband UVB treatment.

UNLABELLED: Narrowband UV-B is a safe and efficacious option for the treatment of adult psoriasis. However, the use of this therapy has been limited in children due to its long-term carcinogenic potential. It has proven to be an adequate alternative in patients whose condition is refractory to topical treatment. AIMS: To evaluate the efficacy and short-term safety of narrowband UV-B in the treatment of paediatric psoriasis, and to compare our results with those obtained in other studies on paediatric psoriasis. MATERIALS AND METHODS: Over a period of 2 years and 4 months, we administered narrowband UV-B to 20 children diagnosed with psoriasis that was refractory to topical therapy. The therapeutic response was measured using the Psoriasis Area and Severity Index (PASI). RESULTS: Between August 2005 and December 2007, 20 children received narrowband UV-B. Their median age was 13 years (range, 5-17 years), and the median initial PASI score was 8.25 (2.7-22.2). A median of 28 (10-59) sessions was required to achieve clearance, reaching almost complete or total remission (median final PASI) in all but two patients. Six patients required a new therapeutic course because of relapse, and the mean duration of remission was 8 months (4-18). No patients experienced severe adverse events during therapy, and only one discontinued treatment, for unrelated reasons. DISCUSSION AND CONCLUSION: Narrowband UV-B for the treatment of paediatric psoriasis has received little attention in the literature. This treatment has been limited in children because of its potential long-term carcinogenic effects, and most information has been extrapolated from adults. Nevertheless, narrowband UV-B phototherapy is an effective and well-tolerated therapeutic alternative in paediatric patients with severe psoriasis.

[Spanish evidence-based guidelines on the treatment of moderate-to-severe psoriasis with biologic agents]. / Directrices españolas basadas en la evidencia para el tratamiento de la psoriasis moderada a grave con agentes biológicos.

Psoriasis vulgaris is an inflammatory skin disease that is generally chronic and that affects between 1 % and 2 % of the population in industrialized Western countries. It is associated with a marked decline in quality of life. A wide range of treatments are currently available, although surveys conducted before the advent of biologic agents reflected a strong degree of dissatisfaction with the treatments then available. Extensive scientific evidence has been gathered on the safety of biologic agents, and this has led to a review of the role of systemic treatment in general and has allowed new therapeutic goals and strategies to be contemplated in patients with moderate-to-severe psoriasis. In this new situation, there is a need for Spanish guidelines on the treatment of moderate-to-severe psoriasis with biologic agents, drafted by consensus among specialists and ratified by the Spanish Psoriasis Group of the Spanish Academy of Dermatology and Venereology (AEDV). These guidelines should be evidence-based with regard to the pharmacologic characteristics, mechanism of action, administration route and regimen, efficacy, contraindications, adverse effects, and cost estimates of biologic agents approved for the treatment of moderate-to severe psoriasis in Spain.

Rosacea-like demodicosis in an HIV-positive child.

A second case of rosacea-like demodicosis in an HIV-positive child was seen at our center. No such cases have previously been published. The present case is a 2-year-old boy, the son of an HIV-positive mother, who responded well to oral erythromycin and topical metronidazole. The frequency of rosacea-like eruptions in HIV-negative children is very low. However, the incidence of these eruptions in HIV-positive children may have been underestimated. The pathogenic role of Demodex mites is discussed as well as the possible mechanisms for an exaggerated reaction.