Webber Lucy, Cummins Michelle, Mann Rebecca, Shaw Lindsay, Ghinai Rosanna, Mahon Caroline
Department of Paediatric Dermatology, Bristol Children's Hospital, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
Department of Paediatric Haematology & Oncology, Bristol Children's Hospital, University Hospitals Bristol NHS Foundation Trust, Bristol, UK.
Pediatr Dermatol. 2019 Sep;36(5):725-727. doi: 10.1111/pde.13872. Epub 2019 Jul 18.
Fanconi anemia is a rare, autosomal recessive genomic instability disorder characterized by congenital limb anomalies, panmyelopathy and a high risk of malignancy, principally acute myeloid leukemia. Hematologic malignancy presenting with acute febrile neutrophilic dermatosis (Sweet syndrome), both deep and superficial forms, is well described in Fanconi anemia patients but is a rare phenomenon in otherwise healthy children. We present a case of panniculitis (presumptive subcutaneous Sweet syndrome) heralding transformation to acute myeloid leukemia in a 3-year-old boy with a severe Fanconi anemia phenotype.