Chronic recurrent osteomyelitis: A diagnostic and therapeutic challenge. / Osteomielitis crónica recurrente: un reto diagnóstico y terapéutico.

INTRODUCTION: Chronic recurrent osteomyelitis (CRO) is a rare disease characterised by unifocal or multifocal aseptic inflammatory bony lesions. OBJECTIVE: To evaluate clinical features and response to treatment with non-steroidal anti-inflammatory drugs in patients with CRO. MATERIALS AND METHODS: A retrospective assessment was conducted on 5 children with OCR over a period of 8 years. They had been diagnosed based on clinical, laboratory findings, histological study of injuries, and imaging test. RESULTS: Multifocal disease was observed in 40% of cases, and unifocal in 60%. The large majority (80%) were male, with a mean age at diagnosis of 13 years (range: 11-15 years). The bones most frequently affected were the clavicle and tibia, representing 18.2%. The most common presenting symptom was pain in all patients, with fever being present in 20% of cases. Imaging studies such as CT and MRI were effective in 100%, and in all cases the biopsy reported a lymphoplasmacytic infiltrate. After a mean follow-up of 5.9 years, there was a good treatment response in 100%, with a mean recurrence at 5.25 months being observed. CONCLUSIONS: The anodyne results of most clinical studies to assess CRO, and the similarity in clinical presentation of this with many infectious or tumour diseases makes its diagnosis a real challenge. On the other hand treatment with NSAIDs can be considered a good initial therapeutic option.

Methotrexate in Moderate to Severe Psoriasis: Review of the Literature and Expert Recommendations.

Methotrexate (MTX) is the most frequently used conventional systemic drug in the treatment of psoriasis. Despite over 50years of experience in this setting, certain aspects of the use of this drug in clinical practice are still little standardized and poorly understood. For this reason, a group of 15 experts took part in a consensus development conference to achieve consensus on a series of recommendations on the use of MTX in psoriasis. The guidelines, which were developed on the basis of a systematic review of the literature, were validated by 2 rounds of voting and categorized by level of evidence and grade of recommendation. Before MTX can be used to treat moderate to severe psoriasis, the patient must be evaluated to assess the suitability of the treatment, including consideration of vaccination status and screening for tuberculosis and pregnancy. The recommended starting dose for a patient with no risk factors is 10 to 20mg/wk, the therapeutic dose for most patients is 15mg/wk, and the maximum dose is 20mg/wk. Most patients who respond to treatment will show improvement within 8weeks. Parenteral administration of MTX is desirable when there is a risk of erroroneous dosing, nonadherence, gastrointestinal intolerance, or inadequate response to the therapeutic dose taken orally. Noninvasive methods are preferred for monitoring hepatotoxicity. MTX is a good treatment option for patients with a history of cancer, but is not recommended in patients with chronic hepatitisB infection or individuals who are seropositive for human immunodeficiency virus.

Osteochondromas in fibrodysplasia ossificans progressiva: a widespread trait with a streaking but overlooked appearance when arising at femoral bone end.

Metaphyseal bony outgrowths are a well-recognized feature of fibrodysplasia ossificans progressiva (FOP) phenotype, but its genuine frequency, topographic distribution, morphological aspect, and potential implications are not fully established. To better ascertain the frequency and characteristics of osteocartilaginous exostoses in FOP disease, we conducted a cross-sectional radiological study based on all the traceable cases identified in a previous comprehensive national research. Metaphyseal exostoses were present in all the 17 cases of FOP studied. Although most often arising from the distal femoral (where metaphyseal exostoses adopt a peculiar not yet reported appearance) and proximal tibial bones, we have found that they are not restricted to these areas, but rather can be seen scattered at a variety of other skeletal sites. Using nuclear magnetic resonance imaging, we show that these exophytic outgrowths are true osteochondromas. As a whole, these results are in agreement with data coming from the literature review. Our study confirms the presence of metaphyseal osteochondromas as a very frequent trait of FOP phenotype and an outstanding feature of its anomalous skeletal developmental component. In line with recent evidences, this might imply that dysregulation of BMP signaling, in addition to promoting exuberant heterotopic ossification, could induce aberrant chondrogenesis and osteochondroma formation. Unveiling the molecular links between these physiopathological pathways could help to illuminate the mechanisms that govern bone morphogenesis.

Safety and effectiveness of ustekinumab and antitumour necrosis factor therapy in patients with psoriasis and chronic viral hepatitis B or C: a retrospective, multicentre study in a clinical setting.

BACKGROUND: Both the safety and efficacy of biologic therapy may be affected in the presence of highly prevalent chronic viral hepatitis. OBJECTIVE: To evaluate the safety and effectiveness of ustekinumab and antitumour necrosis factor therapy in patients with psoriasis and concomitant chronic viral hepatitis. METHODS: This was a retrospective, multicentre study. Twenty-five patients with psoriasis and concurrent hepatitis C virus (HCV) (20 patients) or hepatitis B virus (HBV) (five patients) infection who had received at least one biologic agent (etanercept, 21 treatments; adalimumab, four; ustekinumab, four; infliximab, two) were included. Clinical, imaging and laboratory data were recorded. RESULTS: In the case of HCV infection, the majority of the patients did not exhibit increases in their viral load or serum liver tests. Aspartate aminotransferase, alanine aminotransferase and gamma glutamyl transpeptidase were doubled from the baseline measurement in only one patient treated with etanercept. Two other cases exhibited viral load increases during the follow-up period. In total, 18 of the 26 treatments achieved a 75% improvement in their Psoriasis Area and Severity Index (PASI 75) score during the follow-up period. Two patients treated with etanercept were diagnosed with hepatocellular carcinoma. In the case of HBV infection, all of the patients were being treated with antiviral therapy, and none presented significant variations in viral load or serum liver enzymes. All patients achieved a PASI 75 during follow-up. CONCLUSIONS: Biologic therapy was effective and safe for the majority of our patients with HCV and HBV infection, although there may be a risk of reactivation or aggravation. We describe the first cases to receive ustekinumab. The use of biologics should be limited to those cases in which the risk-benefit ratio is justified.

[Early reconstruction of congenital pseudoarthrosis of the tibia with free vascularized fibular grafts]. / Reconstrucción precoz de pseudoartrosis congénita de tibia con injerto libre vascularizado de peroné.

Congenital pseudoarthrosis of the tibia (CPT) is an uncommon disease presenting a tibial nonunion since birth and frequently associated to neurofibromatosis type 1. Surgical management by wide excision of the pseudoarthrosis and sustitution of the defect with vascularized bone in an early stage has proven to be the most effective technique in terms of bone consolidation. We present a clinical case of a 22-month-old patient with CPT treated successfully by reconstruction with a free vascularized fibula graft with an excellent functional result.

[Early detection of anal intraepithelial neoplasia in high-risk patients]. / Detección precoz de la neoplasia intraepitelial anal en pacientes de alto riesgo.

The incidence of anal squamous cell carcinoma has increased alarmingly, particularly in high-risk groups such as men who have sex with men and immunosuppressed patients. Infection with an oncogenic strain of the human papillomavirus in the anal canal or perianal skin leads to anal intraepithelial neoplasias (AIN), progressive dysplastic intraepithelial lesions that are the precursors of anal squamous cell carcinoma. AIN can be diagnosed through cytological screening and biopsy guided by high-resolution anoscopy and can be treated using a range of procedures in an effort to prevent progression to invasive anal carcinoma. Given the recent advances in the understanding of this disease, and the increasing calls from experts for the establishment of screening programs to identify AIN, we review current knowledge on the condition, its diagnosis, and treatment from the point of view of dermatology.

[Adverse reactions during biological therapy for psoriasis: results of a survey of the Spanish Psoriasis Group]. / Efectos adversos observados durante la terapia biológica en la psoriasis. Resultados de una encuesta al Grupo Español de Psoriasis.

BACKGROUND: Biologic therapies have been a major breakthrough in the treatment of psoriasis because they are more selective and have a better short-term and medium-term safety profile. There are reliable data to support both the efficacy and the safety of these drugs. However, it is always useful to report the clinical experience of dermatologists who are experts in the use of biologic agents to treat psoriasis, particularly with regard to their safety. MATERIAL AND METHODS: We present the results of a survey administered to the members of Spanish Psoriasis Group and based on a series of questions referring to the clinical safety of these agents. A total of 988 patients treated with efalizumab, infliximab, etanercept, and adalimumab were reported by 15 members of the group. RESULTS: There was a particularly high proportion of reactions (34%) to infliximab infusions. Blood test abnormalities were detected in 13.25% of patients and infections in 12.24%, with one case of pulmonary tuberculosis. Attention is drawn to the adverse effects profile of efalizumab: de novo arthritis in 5.8% and rebound in 20.9% of patients. CONCLUSION: The safety data provided by our study should be taken into account in view of the large number of patients recruited by dermatologists experienced in the use of this type of therapy.

Successful treatment of three cases of primary extramammary Paget's disease of the vulva with Imiquimod--proposal of a therapeutic schedule.

BACKGROUND/AIM: Extramammary Paget's disease (EMPD) is an intraepidermal adenocarcinoma of apocrine gland-bearing skin. Although surgery remains the mainstay of treatment, loss of tissue function and high recurrence rates have been reported. Recently, topical Imiquimod has been shown as a safe and effective treatment option for extramammary Paget's disease. METHODS: Three patients diagnosed of EMPD of the vulva were treated with a daily application of 5% Imiquimod cream for three weeks, followed by an every other day application for an additional three weeks. RESULTS: Complete clinical and histological remission of the disease was achieved in the three patients. Mild irritation and tenderness were observed as the only side effects. CONCLUSION: Based on our results, we consider that Imiquimod cream is a safe and effective therapeutic option for the treatment of vulvar EMPD. These promising results warrant further studies to determine the real efficacy and safety of Imiquimod 5% cream for the treatment of this uncommon disease.

Postmenopausal frontal fibrosing alopecia.

Recently a new entity, postmenopausal frontal fibrosing alopecia, was added to the established subtypes of scarring alopecias affecting postmenopausal women. This condition is characterized by a progressive frontal hairline recession associated with scarring. We studied the clinical and histopathologic features in four women with this disorder. Of note, a history of bilateral oophorectomy in two of them appears to be a new association. All four cases had frontoparietal recession of the hairline and two of them also had loss of their eyebrows. None of our four patients had any mucous membrane or other skin lesions. Histological examination showed perifollicular fibrosis and lymphocytic inflammation around the isthmus and infundibular areas of the follicles. No effective treatments have emerged for this type of postmenopausal alopecia, but progression of the hair loss and scarring appears to be self-limiting. We believe that this condition is a distinct clinicopathological variant of lichen planopilaris.

Successful treatment of aphthous ulcerations in AIDS patients using topical granulocyte-macrophage colony-stimulating factor.

Oral recurrent aphthous ulceration (RAU) is a well-recognized complication in patients infected with human immunodeficiency virus. RAU can be progressive and destructive, causing dysphagia and secondary malnutrition. The aetiology of RAU remains unknown, and its response to available treatments is often unsatisfactory. We describe three patients with advanced AIDS who suffered from extensive RAU which failed to respond to several treatments, including topical viscous lidocaine and topical and systemic glucocorticoids. Owing to difficulties in using thalidomide (two patients had neurological conditions which precluded thalidomide use), all three patients were treated with an oral solution containing recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF, 400 microg in 5% glucose 200 mL). From the first application, all three patients showed significant improvement of their lesions and amelioration of pain, and they were completely cured in a few days. No adverse effects were recorded. The patients did not show relapses of RAU over a prolonged follow-up. Controlled trials are warranted in order to establish the role of GM-CSF as a valid, alternative option for aphthous ulcerations of the mouth in AIDS patients in whom corticosteroids or thalidomide are not suitable.

Anterior endoscopic approach to the thoracolumbar spine.

STUDY DESIGN: A technique for anterior endoscopic exposure of the thoracolumbar spine is described. OBJECTIVE: To develop an endoscopic approach to the thoracolumbar spine with fewer complications than are encountered in the conventional approach. SUMMARY OF BACKGROUND DATA: The efficacy of the anterior endoscopic approach to the thoracic spine has been shown. The thoracolumbar hinge is usually approached by open surgery. METHODS: The study was performed in three stages. The first phase was carried out on experimental animals (pigs). Once the bases were established, the approach was studied in fresh cadavers. Finally, it was performed on three patients with thoracolumbar scoliosis who required discectomy and anterior release. RESULTS: After a series of attempts on the animals, it was found that the most effective method for exposure of the thoracolumbar spine was the initial thoracoscopic approach followed by the retroperitoneal endoscopic approach. This was confirmed in cadavers. When applied to the clinical situation, there were no significant complications in any of the patients treated with this method, and postoperative pain and discomfort were minimal. CONCLUSIONS: The anterior endoscopic approach to the lumbar spine involves minor trauma, results in rapid recovery and less pain, and produces good results aesthetically.