ISOLATED PYODERMA GANGRENOSUM AND ADALIMUMAB:CASE REPORT IN PAEDIATRIC AGE

Introduction: Pyoderma Gangrenosum (PG) is a sterile neutrophilic disorder, rarely described in children and adolescents, and frequently it is known as secondary to other chronic inflammatory diseases. However, epidemiological, clinical and therapeutic data on paediatric PG are numerically limited and no randomized controlled trials have been published. Associated diseases in paediatric cases are inflammatory bowel diseases, vasculitis, immune deficiencies, PAPA Syndrome. The treatment with systemic steroids and cyclosporine is well documented in the literature as the first-line treatment. In nonresponders, other treatment lines are indicated, as: corticosteroids and mycophenolate mofetil, mycophenolate mofetil and cyclosporine, tacrolimus, dapsone, infliximab, plasmapheresis. In case reports adalimumab has been used as the treatment for patients who did not respond to other drugs. Objectives: We evaluated the efficacy of adalimumab in PG nonresponder to steroids, steroids plus cyclosporine, dapsone plus cyclosporine. Methods: We describe the clinical course of a 16-years-old female affected by PG at the left leg since age 13. Autoimmune tests were negative, as abdominal US with evaluation of the thickness of bowel wall, tTG, AGA, IgA, IgG, IgM, IgE, C3, C4, transaminases, ocular study with slit lamp were negative. She showed HLA DQ8, as the mother affected by Celiac disease. Two biopsies studies confirmed the diagnosis of PG, and the treatment with steroids and antibiotics was started. However, the disease gone on and cyclosporine was associated to steroids. In a further line of treatment, she was treated with dapsone plus cyclosporine. PG extended to the right leg and worsened with acute lesions. She was treated with topic steroids and antibiotics, with a further extension of the lesions. We proposed to start adalimumab (40 mg s.c. /14 days) and the tapering of topic steroids. Results: Skin injuries progressively improved, and after 3 doses of adalimumab PG is limited to the atrophic lesions in the zones of previous ulcerative lesions. Conclusion: Adalimumab is a safe and promising treatment in isolated paediatric PG and can reduce the use of steroids or other immune suppressive drugs. We highlight the role of accurate diagnostic procedures to primary exclude associated diseases. Informed consent to publish had been obtained from the parents

Treatment of severe psoriasis in children: Recommendations of an Italian expert group

This article provides comprehensive recommendations for the systemic treatment of severe pediatric psoriasis based on evidence obtained from a systematic review of the literature and the consensus opinion of expert dermatologists and pediatricians. For each systemic treatment, the grade of recommendation (A, B, C) based on the treatment\ue2\u80\u99s approval by the European Medicines Agency for childhood psoriasis and the experts\ue2\u80\u99 opinions is discussed. The grade of recommendation for narrow-band-ultraviolet B phototherapy, cyclosporine, and retinoids is C, while that for methotrexate is C/B. The use of adalimumab, etanercept, and ustekinumab has a grade A recommendation. No conventional systemic treatments are approved for pediatric psoriasis. Adalimumab is approved by the European Medicines Agency as a first-line treatment for severe chronic plaque psoriasis in children (\ue2\u89\ua5 4 years old) and adolescents. Etanercept and ustekinumab are approved as second-line therapy in children \ue2\u89\ua56 and \ue2\u89\ua5 12 years, respectively. Conclusion: A treatment algorithm as well as practical tools (i.e., tabular summaries of differential diagnoses, treatment mechanism of actions, dosing regimens, control parameters) are provided to assist in therapeutic reasoning and decision-making for individual patients. These treatment recommendations are endorsed by major Italian Pediatric and Dermatology Societies

Additional file 1: of Topical corticosteroid phobia in parents of pediatric patients with atopic dermatitis: a multicentre survey

Univariate and multivariate analysis results (dependent variable: TCS fear). (DOCX 22 kb