ISOLATED PYODERMA GANGRENOSUM AND ADALIMUMAB:CASE REPORT IN PAEDIATRIC AGE

Abstract

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.