P44 Rapidly progressive cutaneous ulceration in a toddler

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Abstract Introduction/Background Pyoderma gangrenosum is a rare ulcerative inflammatory cutaneous condition of unknown aetiology which is uncommon in childhood. Only 4% of reported cases occur in individuals younger than 15. The initial lesion in PG is commonly a painful hemorrhagic nodule or pustule that rapidly progresses into a large necrotic boggy ulcer that may resolve with cribriform scars. About 50% of patients with PG are associated with an underlying systemic disease, including inflammatory bowel disease, hematologic malignancies disease, and arthritis. It may occur in the context of classic syndromes like PAPA. Description/Method A 3-year-old male child was admitted to the Rheumatology ward with fever and generalized ulcers for two months. The ulcers started as multiple small pustules over the buttock, bilateral thigh, and extensor aspect of upper limbs and evolved into large ulcers within two months. Ulcers were round to oval in shape with irregular margins, undermined edges and violaceous borders. He also developed similar ulcers at intravenous cannula sites over the dorsum of hands suggesting the pathergy phenomenon. Laboratory reports suggested increased inflammatory markers. Skin biopsy showed dense dermal neutrophilic infiltration without vasculitis, suggesting pyoderma gangrenosum. Bacterial fungal and tubercular cultures from the biopsy tissue were normal. Bone marrow examination ruled out hematopoietic malignancy. Colonoscopy was normal ruling out subclinical IBD. Whole exome sequencing for auto inflammatory panel didn’t reveal any pathogenic variant. Diagnosis was considered as childhood-onset idiopathic pyoderma gangrenosum. The child was started on steroids along with cyclosporine, ulcers slowly started to heal over a period of two months and started to recur on the buttocks at sites of minor trauma. Discussion/Results The most common underlying etiologies in pediatric PG are IBD (inflammatory bowel disease), hematologic malignancies, immune deficiencies, vasculitis, PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum and acne), SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), and as well as PASH (pyoderma gangrenousm, acne and hidradenitis suppurevitiva). Idiopathic cases account for 49% of cases. Adult PG, most commonly occurs in lower limbs, but lesions in pediatric PG are multiple and disseminated without any specific location. Cyclosporine, Methotrexate, Mycophenolate mofetil, and cyclophosphamide can be used as steroid-sparing agents.TNF inhibitors and Anakinra can be used as biological agents. Key learning points/Conclusion Pediatric PG can be misdiagnosed due to its rarity and atypical presentation; a thorough clinically oriented workup is mandatory to make the correct diagnosis and choose the appropriate treatment. Treating a resistant PG can be challenging. Further growth in the field of genomic sequencing paves the way for a new diagnosis.