ABSTRACT
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.
Subject(s)
Dermatofibrosarcoma , Child , Decision Trees , Dermatofibrosarcoma/genetics , Dermatofibrosarcoma/pathology , Dermatofibrosarcoma/therapy , HumansABSTRACT
BACKGROUND: Evaluation of disease severity is considered essential in the optimal management of psoriasis. OBJECTIVES: To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients' quality of life. MATERIALS AND METHODS: This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006. RESULTS: More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy. CONCLUSIONS: Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.
Subject(s)
Psoriasis/diagnosis , Psoriasis/therapy , Cross-Sectional Studies , Female , Humans , Male , Middle Aged , Retrospective Studies , Severity of Illness Index , SpainABSTRACT
BACKGROUND: Evaluation of disease severity is considered essential in the optimal management of psoriasis. OBJECTIVES: To describe the clinical characteristics and therapeutic profile of patients with moderate to severe psoriasis in Spain and to assess the impact of the disease on the patients' quality of life. MATERIALS AND METHODS: This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006. RESULTS: More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy. CONCLUSIONS: Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.
ABSTRACT
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.
Subject(s)
Neurocutaneous Syndromes/complications , Scoliosis/complications , Tooth Diseases/complications , Adolescent , Adult , Child , Female , Humans , Male , Middle AgedABSTRACT
Bullous pemphigoid is an acquired autoimmune blistering disorder extremely uncommon in children, characterized by circulating IgG antibodies to antigens of the epidermal basement membrane zone. In general, the clinical course of this condition is good and relapses are rare. The early diagnosis and treatment are fundamental. We present a 3-month-old girl with a blistering eruption on her palms and soles, and urticarial plaques on trunk, and face, 3 weeks after vaccine at two months (hepatitis B, diphtheria, tetanus, pertussis, polio, Haemophilus influenzae B, meningococcal C, pneumococcus). The clinical course worsened with vaccinations at 4 and 6 months. The control of lesions was achieved with oral deflazacort 1 mg/kg/day, with a gradual decrease until 3 months of therapy. The patient is still in remission after 8 months of follow-up. Bullous pemphigoid has been connected with some drugs and vaccinations, 1 day to 4 weeks after receiving immunization. Although the exact mechanism of induction is unclear, this case report has a visible relationship with vaccinations.
Subject(s)
Diphtheria-Tetanus-Pertussis Vaccine/adverse effects , Hepatitis B Vaccines/adverse effects , Meningococcal Vaccines/adverse effects , Pemphigoid, Bullous/chemically induced , Pneumococcal Vaccines/adverse effects , Poliovirus Vaccine, Inactivated/adverse effects , Female , Humans , Infant , Vaccines, Combined/adverse effectsSubject(s)
Hamartoma/complications , Head and Neck Neoplasms/complications , Nevus, Blue/complications , Skin Diseases/complications , Skin Neoplasms/complications , Adult , Biopsy , Carcinoma, Basal Cell/diagnosis , Dermoscopy , Diagnosis, Differential , Female , Hair Follicle/pathology , Hamartoma/diagnosis , Hamartoma/pathology , Head and Neck Neoplasms/diagnosis , Head and Neck Neoplasms/pathology , Humans , Nevus, Blue/diagnosis , Nevus, Blue/pathology , Shoulder , Skin Diseases/diagnosis , Skin Diseases/pathology , Skin Neoplasms/diagnosis , Skin Neoplasms/pathologyABSTRACT
Scalp lesions are common in psoriasis and difficult to treat. Scientific evidence on the topic is scant and fragmentary, especially with respect to long-term treatment. This consensus statement is based on a critical assessment of the results of a MEDLINE search for clinical trials of the efficacy and safety of therapies used to treat scalp psoriasis. The recommendations were developed by an expert panel using the Delphi process to reach a consensus and then ratified by the members of the Psoriasis Group of the Spanish Academy of Dermatology and Venereology. The recommended induction therapy for scalp psoriasis is either a topical corticosteroid or a topical treatment combining calcipotriol and betamethasone. The choice of an appropriate vehicle is crucial in improving effectiveness and patient adherence to treatment. The only formulations that have been studied in the long-term treatment of scalp psoriasis are a combination of calcipotriol and betamethasone in gel and calcipotriol alone in solution.
Subject(s)
Psoriasis/therapy , Scalp Dermatoses/therapy , Delphi Technique , HumansABSTRACT
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
Subject(s)
Acrodermatitis/drug therapy , Immunoglobulin G/therapeutic use , Receptors, Tumor Necrosis Factor/therapeutic use , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Etanercept , Humans , Male , Middle AgedABSTRACT
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.
Subject(s)
Psoriasis/radiotherapy , Severity of Illness Index , Skin/radiation effects , Ultraviolet Therapy/methods , Adolescent , Child , Child, Preschool , Female , Humans , Longitudinal Studies , Male , Recurrence , Remission Induction , Retrospective Studies , Skin Pigmentation , Treatment Outcome , Ultraviolet Therapy/adverse effectsABSTRACT
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.
Subject(s)
Evidence-Based Medicine , Psoriasis/drug therapy , Adalimumab , Anti-Inflammatory Agents/therapeutic use , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized , Etanercept , Immunoglobulin G/therapeutic use , Infliximab , Receptors, Tumor Necrosis Factor/therapeutic use , Severity of Illness Index , SpainABSTRACT
The treatment of psoriasis has been revolutionized by the introduction of biologic agents; these agents achieve skin clearance and long-term improvement without the risk of toxicity that has limited use of the classic systemic treatments. The role of systemic treatment in the management of psoriasis is being reviewed on the basis of a large volume of scientific evidence on the efficacy and safety of biologic agents, and new therapeutic goals and strategies are being devised for patients with moderate-to-severe psoriasis. This has led to the need to establish severity criteria that will provide the rationale for the indication of the different systemic agents currently available for the treatment of moderate-to-severe psoriasis, as well as therapeutic goals, efficacy measures, therapeutic strategies, screening protocols, and choice of treatment based on the risk-benefit ratio of the different agents. These criteria must be established through consensus by experienced dermatologists and based on available scientific evidence. The present document reflects the consensus of the Spanish Psoriasis Group on these different issues in the management of moderate-to-severe psoriasis.
Subject(s)
Psoriasis/diagnosis , Psoriasis/drug therapy , Biological Therapy , Humans , Severity of Illness IndexABSTRACT
Topical therapy continues to be one of the pillars of psoriasis management. Topical corticosteroids and vitamin D analogs are the drugs of choice during the induction phase, and vitamin D analogs continue to be drugs of choice for maintenance therapy. Tazarotene and dithranol are suitable options in patients with certain, specific characteristics. The calcineurin inhibitors can be considered to be second-line treatment for psoriasis of the face and flexures. The efficacy and safety of the fixed-dose combination of betamethasone and calcipotriol in the induction phase is greater than that of either drug alone. The combination of corticosteroids with salicylic acid achieves better results than corticosteroids in monotherapy. None of the drugs evaluated stands out over the others in all clinical situations, and their use must therefore be individualized in each patient and adjusted according to the course of the disease.
Subject(s)
Psoriasis/drug therapy , Administration, Topical , Betamethasone/administration & dosage , Calcitriol/administration & dosage , Calcitriol/analogs & derivatives , Drug Therapy, Combination , HumansSubject(s)
Lipodystrophy/diagnosis , Abdomen , Adipose Tissue/pathology , Adult , Biopsy , Diagnosis, Differential , Humans , MaleABSTRACT
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.
