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

Abstract

Background: 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 etanercept, 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.Objective: To describe the clinical presentation, laboratory tests 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. Result: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 tests for both cases showed an increase in white blood cell counts mainly neutrophils, anaemia, increased platelets count, elevated erythrocyte sedimentation rate, and elevated C-reactive protein. No haematological abnormalities were seen in peripheral blood smears except neutrophilic response, the red cell indices were suggestive of iron deficiency anaemia. 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 cushingoid habitus and minimal response to corticosteroid sparing agent azathioprine. Anti TNF-alpha inhibitors etanercept, 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...

A rare case study of pyoderma gangrenosum with dilated cardiomyopathy and multiple cerebral infarct in malnourished children

“…However, the diagnosis is often delayed because it excludes other differential diagnoses with limited definitive signs and symptoms, as reported in this study. Pain control, local wound care, topical and systemic drug therapy were required as modalities of PG treatment [ 4 , 8 , 10 , 16 ]. Systemic drug therapy is indicated to treat more extensive lesions with high-dose corticosteroids (prednisone 1–2 mg/kg/d) because of the underlying inflammatory process and rapidity of response in 2–3 days [ 3 ].…”

“…Pyoderma gangrenosum (PG) is an extremely rare case of chronic non-infectious dermatoses characterized by painful and necrotic ulceration with undermined border. Based on previous reports and retrospective case series, the global incidence of PG was approximately 3–10 cases per million population per year [ [1] , [2] , [3] , [4] ]. However, most investigations reported adult cases because the peak of the incidence was between 20 and 50 years of age, while 3–4 % were recorded among children.…”