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Leg ulcer in a child with sickle cell disease - Is it a complication of the disease or a cutaneous side effect of hydroxyurea treatment?

Filipa Sutre¹, Carolina Amaro Gonçalves², Anabela Ferrão^2
1Pediatric Department, Hospital de Santarém, Santarém, Portugal, ²Pediatric Haematology Unit, Pediatric Department, Centro Hospitalar e Universitário Lisboa Norte - Hospital de Santa Maria, Lisbon, Portugal

Address for Correspondence: Filipa Sutre, Pediatrics Department, Hospital de Santarém, Avenida Bernardo
Santareno 3737B, 2005-177 Santarém, Portugal.
Email: filipasutre@gmail.com
Keywords : hydroxyurea, leg ulcer, sickle cell disease
Question:
An 8-year-old girl with sickle cell disease (SCD) is under the care of a Pediatric Hematology Unit at a tertiary hospital. She has a history of multiple bone vaso-occlusive crises, some of which required hospitalization and two episodes of acute chest syndrome. The patient has been receiving hydroxyurea treatment at 20 mg/kg/day. After three months of therapy, she presented with a non-traumatic superficial leg ulcer on the internal malleolar region of her right foot. There were no reported cutaneous side effects aside from this ulcer and she did not experience other symptoms such as pain crises, anemia or jaundice. Physical examination revealed a superficial leg ulcer in the internal malleolar region of the right foot, clinically resembling an abrasion. It measured 2.0 cm by 1.5 cm and had irregular hyperpigmented edges, a pinkish base and peri-ulcer induration, as shown in Figure 1.

Figure 1. Leg ulcer in a sickle cell patient's internal malleolar region of the right foot.


Figure 2. The ulcer's healing progress four weeks after discontinuing hydroxyurea treatment.

What is the diagnosis?