Childhood pyoderma gangrenosum: diagnostic challenges and management of two cases from Libya

Abstract

Introduction: Pyoderma gangrenousm (PG) is an idiopathic Ulcerative, non-infective chronic inflammatory skin disorder of unknown etiology.The most common reported underlying diseases in pediatrics are inflammatory bowel disease, followed by hematologic disorders, vasculitis, immune deficiencies and pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome. More than half of the cases occur with no underlying disease. The most frequently treatment should be tailored according to the underlying etiology. It includes systemic steroids, corticosteroid sparing agents such as dapsone, cyclosporine, azathioprine and currently TNF-alpha inhibitors such as etenrecpt, adalimumab and infliximab is a promising treatment of refractory PG. Response to treatment is high with cure rates reaching 90%. A high index suspicion and a through workup are mandatory in the management of pediatrics PG. Objectives: To describe the clinical presentation, laboratory testes and challenges with treatment trials in two pediatric cases. Methods: clinical information was gathered from the history, which was taken from their parents after an informed consent Results: for the first time we report two pediatric cases in Libya who diagnosed with refractory pyoderma gangrenousm with no associated systemic diseases except PAPA which can't be excluded since no genetic tests available in all public hospitals in Libya, we report PG in two Libyan children of 2 year-old and 5 year-old, they presented with skin lesions beginning as a small pustule that progressed to very painful large ulcer with irregular borders. There were healed ulcers with scaring present on chest, both upper and lower extremities and abdominal wall.Laboratory testes for both cases showed an increase in white blood cell counts mainly neutrophils, anemia, increased platelets count, elevated erythrosedmintation rate, and elevated C-reactive protein.No hematological abnormalities were seen in peripheral blood smears except neutrophilic response, the red cell indices were suggestive of iron deficiency anemia.Cultures for bacteria and fungi from skin lesions, and blood cultures were repeatedly negative. Immunoglobulin assay (IgG, IgM, IgA, IgE) was normal. The antinuclear antibody, cytoplasmic antibody, and antiphospholipid antibodies, tests for hepatitis B, C and HIV were all negative. They didn't have symptoms suggest ulcerative colitis, their colonoscopy was normal. Histopathology from their skin was consistent with pyoderma gangrenousm. Both cases were moderate response to high dose of oral prednisolone 2mg\kg per day complicated, however by growth retardation and cushingnoid habitus and minimal response to corticosteroid sparing agent azathioprine. Anti TNF-alpha inhibitors etenrecpt, which has been available at hospital since March 2018, we used it in case 2 because he was inadequate response to azathioprine. Both cases showed briefly remitted on azathioprine with relapsed at least 2-3 episodes per year. The ulcer lesions healed with cribriform scaring within 6-8 weeks in each episode. Conclusion: Pyoderma gangrenousm has recently been included within the spectrum of auto-inflammatory diseases, which are characterized by recurrent episodes of sterile inflammation, without circulating autoantibodies and autoreactive, which can be diagnosed by genetic tests, which is unfortunately not available in public hospitals in Libya.We couldn't either be able to start other biological treatment because of the civil war crisis in Libya.