Sickle cell crisis presenting as livedo racemosa

Area of Science:

• Hematology
• Dermatology
• Genetics

Background:

• Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin S, leading to sickle-shaped red blood cells.
• Vaso-occlusive crisis (VOC) is a hallmark complication of SCD, causing severe pain due to microcirculatory blockage by sickled cells.
• Tissue ischemia during VOC can initiate inflammatory responses, contributing to various dermatological manifestations in SCD patients.

Observation:

• Chronic leg ulcers are the most frequent skin issue observed in individuals with sickle cell disease.
• A 58-year-old female patient with a history of SCD presented with widespread edematous plaques.
• The patient's bilateral legs displayed characteristic reticulated purpuric patches with central pallor, indicative of livedo racemosa.

Findings:

• This case details an atypical presentation of livedo racemosa.
• The livedo racemosa was the initial sign noted in a patient experiencing a sickle cell vaso-occlusive crisis.
• The findings suggest a potential link between VOC and this specific dermatological presentation.

Implications:

• Recognizing livedo racemosa as a potential indicator of VOC in SCD patients is crucial for timely diagnosis and management.
• This case expands the understanding of dermatological manifestations associated with sickle cell disease complications.
• Further research may elucidate the pathogenic mechanisms connecting VOC, inflammation, and livedo racemosa in sickle cell disease.