Pyoderma gangrenosum and sickle cell vaso-occlusive crisis: fortuitous association or causal relationship ?: a case report

Abstract

Abstract Background The association between pyoderma gangrenosum (PG) and painful vaso-occlusive crisis (VOC) in sickle cell disease (SCD) patients is a rare clinical entity, rarely reported in the literature (it is the 3rd published case). It illustrates a critical therapeutic dilemma: corticosteroids, the standard treatment for PG, exacerbate the vaso-occlusive crises of sickle cell disease. Its interest is also educational, aiming to alert clinicians to this association. The novelty lies in the description of a complex and successful management of a PG flare-up during an active vaso-occlusive crisis, a poorly documented scenario. This case thus provides a practical model for managing this therapeutic paradox and paves the way for considering a potential shared inflammatory pathophysiology between the two diseases. Case presentation A 23-year-old female of Senegalese origin presented with persistent, hyperalgesic VOC involving the coastal region and limb pain. The symptom progression was marked by the appearance of sero-hemorrhagic bullous lesions, which evolved into irregular-bordered ulceronecrotic lesions affecting the limbs (anterior thighs). The patient’s history revealed similar episodes over the past seven years (since 2017). Bacteriological examination of the fluid was sterile. Consequently, a diagnosis of pyoderma gangrenosum (PG) was made. Treatment with corticosteroids, combined with local wound care and monthly transfusion exchanges, led to favorable outcomes. Conclusion Managing pyoderma gangrenosum (PG) in patients with sickle cell disease (SCD) is challenging because corticosteroid therapy, the treatment of choice for PG, often triggers vaso-occlusive crises (VOCs). Therefore, recognizing this association is crucial.