Recurrent lipoatrophic panniculitis of children.

BACKGROUND: Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by immunohistochemical stains. OBJECTIVE: To depict the profile of children with recurrent idiopathic panniculitis. METHODS: Study of clinical, histopathological and immunohistochemical features in five cases with recurrent idiopathic panniculitis. RESULTS: Five children with repeated attacks of painful subcutaneous nodules in association with fever, malaise and abdominal pain or arthralgia, with subsequent lipoatrophy were reviewed. In two patients, extensive involvement led to loss of the cutaneous fatty tissue. Laboratory abnormalities included increased acute phase reactants, leukocytosis with mild neutrophilia, microcytic anaemia and elevated liver enzymes. Histopathology showed lobar panniculitis without vasculitis and with a mixed infiltrate, composed of neutrophils, mononuclear cells, lymphocytes, macrophages and myeloid cells. Neutrophils and myeloid cells were more prominent in early lesions, whereas macrophages predominated in late stages, leading to lipophagia and lipoatrophy. Immunohistochemistry showed positive staining for myeloperoxidase around the necrotic adipocytes in early stages and CD68/PGM1 macrophages in late stages. Intense STAT1 staining was observed in the inflammatory infiltrate. All patients improved with methotrexate and corticosteroids. CONCLUSION: We present five cases of lobar panniculitis and lipoatrophy in childhood. The clinico-pathologic presentation shares features with other autoinflammatory diseases.

[Recommendations for the use of methotrexate in patients with juvenile idiopathic arthritis]. / Recomendaciones para el uso de metotrexato en pacientes con artritis idiopática juvenil.

OBJECTIVES: To develop a consensus document of recommendations for the use of methotrexate (MTX) in patients with juvenile idiopathic arthritis (JIA). MATERIAL AND METHOD: A group of eleven experts proposed several clinical questions on the use of MTX in patients with JIA. A systematic review was conducted and the evidence and recommendations for each question were extracted. The results were discussed and validated by the experts in a work session to establish the final recommendations. RESULTS: MTX is recommended as the first drug for inducing remission in JIA, and its indication should be made according to the clinical category of the patient. Prior to treatment, it is recommended to perform a complete blood count, including white cells, levels of liver enzymes, serum creatinine, and other analytical parameters according to specific risk factors. Treatment should be initiated with a dose of 10-15 mg/m(2)/week. In cases of uveitis or polyarthritis, an initial dose of 15 mg/m(2)/week should be considered. For a better bioavailability and tolerability, it is preferable to administer MTX parenterally if the dose is ≥15 mg/m(2)/week. It is necessary to periodically perform an analytical monitoring of the patient and to assess possible alterations in liver enzymes to make changes if necessary. Combinations with biological agents may be necessary, as well as the concomitant addition of folic or folinic acid. CONCLUSIONS: This document describes the main recommendations for the appropriate use of MTX in JIA patients, according to scientific evidence and clinical experience.

Subdural empyema with extension to vertebral canal secondary to salmonellosis in a patient with systemic lupus erythematosus.

CASE REPORT: A case of supratentorial subdural empyema extending to the superior subdural cervical space in a 14-year-old patient with systemic lupus erythematosus is presented. The presumed etiology of the empyema was an intestinal nontyphoidal salmonella infection. DISCUSSION: We review the neurological and neurosurgical complications in systemic lupus erythematosus.

Primary solitary Echinococcosis in cervical spine. Postsurgical successful outcome after long-term albendazole treatment.

STUDY DESIGN: A case report of a young man with isolated cervical hydatidosis treated postoperatively with sustained cyclical albendazole therapy for 9 years of follow-up. OBJECTIVES: To communicate the efficacy and safety of prolonged albendazole treatment in the postoperative management of spinal hydatid disease, and recommend therapeutic regimes for preventing its recurrence. SUMMARY AND BACKGROUND DATA: Bone involvement in hydatid disease is uncommon and the cervical region of the spine is rarely affected. Surgical excision remains the treatment of choice but high rates of postoperative recurrence have highlighted the importance of adjuvant anthelmintic therapy. The selection of the drug(s) and the duration of the medical treatment is still controversial. METHODS: The patient described herein presented with isolated bone lesions, in an unusual cervical location, and without coincidental visceral involvement. Therefore, diagnosis was delayed and surgical debridement was carried out without any preoperative anthelmintic therapy. To prevent late recurrences, therapy with intermittent courses of albendazole has been maintained for nine years and is still ongoing. Response and toxicity related to therapy has been closely monitored by clinical, biochemical and radiological follow up. RESULTS: After surgery the patient has remained asymptomatic without sequelae or evidence of relapses. No clinically relevant side effects has been observed. CONCLUSION: Prolonged albendazole treatment appears to be safe and effective in the prevention of late recurrences after spine hydatidosis surgery. Long-term chemotherapeutic schedules should be considered after surgical excision of spine or bone lesions.