Pyoderma gangrenosum and sickle cell vaso-occlusive crisis: fortuitous association or causal relationship ?: a case report
- 1Department of Clinical Hematology, National Blood Transfusion Center, Cheikh Anta Diop University of Dakar
- 2Medical Oncology and Clinical Hematology department, Principal Hospital of Dakar
- 3Dermatology department, Albert Royer National Children’s Hospital of Dakar
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.
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BibTeX
@article{kaltam2026pyoderma,
title = {{Pyoderma gangrenosum and sickle cell vaso-occlusive crisis: fortuitous association or causal relationship ?: a case report}},
author = {Abderamane Yacoub Hadje Kaltam and Moussa Seck and Elimane Seydi Bousso and Khadim Diop and Alioune Badara Diallo and Fatma Dieng Bousso and Blaise Felix Faye and Eric Loic Bana and Hadje Zara Issa and Saliou Diop},
journal = {Journal of Medical Case Reports},
year = {2026},
month = aug,
doi = {10.1186/s13256-026-06185-7},
url = {https://doi.org/10.1186/s13256-026-06185-7},
note = {CC BY},
}
RIS
TY - JOUR
TI - Pyoderma gangrenosum and sickle cell vaso-occlusive crisis: fortuitous association or causal relationship ?: a case report
AU - Abderamane Yacoub Hadje Kaltam
AU - Moussa Seck
AU - Elimane Seydi Bousso
AU - Khadim Diop
AU - Alioune Badara Diallo
AU - Fatma Dieng Bousso
AU - Blaise Felix Faye
AU - Eric Loic Bana
AU - Hadje Zara Issa
AU - Saliou Diop
PY - 2026
DA - 2026/08/01
JO - Journal of Medical Case Reports
DO - 10.1186/s13256-026-06185-7
UR - https://doi.org/10.1186/s13256-026-06185-7
AB - 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.
SN - 1752-1947
ER -