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Society for Publication of Acta Dermato-Venereologica Pyoderma gangrenosum (PG) is a neutrophilic dermatosis (ND) characterized by a chronic ulcerating skin condition that appears to be immune mediated. Recent diagnostic criteria have been proposed for PG as a result of a Delphi consensus exercise using the RAND Health Care/University of California at Los Angeles Appropriateness Method (1). The cause of PG remains unknown, but up to 50% of cases occur in association with multiple systemic diseases, such as inflammatory bowel disease, rheumatological condi tions, or malignant haematological disorders, in particular acute myeloid leukaemia (AML) and myelodysplastic syndrome (MDS) (2, 3). A clonal link between malignant myeloid cells and skin-infiltrating neutrophils has been described recently in ND associated with AML and MDS (4). Fanconi anaemia (FA) is a rare autosomal recessive bone marrow failure syndrome due to genetic defects of a DNA repair pathway. The major complications of FA are aplastic anaemia, AML, MDS and specific solid tumours. We describe here 2 adult patients with long-standing FA diagnoses who developed PG, revealing an acceleration of their myeloid disorder. To our knowledge only 2 similar cases have been reported previously (5, 6).

Pyoderma Gangrenosum Revealing Myeloid Activation of Fanconi Anaemia: Two Case Reports