P44 Rapidly progressive cutaneous ulceration in a toddler

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