Hemoglobinopathies and Leg Ulcers

Abstract

Major hemoglobinopathies, including sickle cell anemia, are becoming a global health issue. Leg ulcers are the most common cutaneous manifestation of sickle cell disease and an important contributor to morbidity burden in this population. Leg ulcers following sickling disorders are extremely painful, and hard to heal. The clinical evidence for the optimal management of these ulcers is limited. Treating the cause and the strategies to prevent sickling are the mainstay of treatment. The basic principles of wound bed preparation and compression therapy is beneficial in these patients.

Leg ulcer induced by hydroxycarbamide in sickle cell disease: What is the therapeutic impact?

“…Patients exhibit increases in pain because of microcirculatory ischaemia . Recently, vascular anomalies, including tone anomalies and activation of endothelial adhesions molecules, have been identified .…”

“…Transfusion management can be considered (reduction in haemoglobin levels, partial correction of anaemia), but to our knowledge neither its objectives nor its actual contribution to scarring have been assessed, and there is no consensus on this point (potentially involving risks of iron overload and risks of alloimmunisation) . Other treatments have been mentioned such as arginine butyrate or hypomethylating agents but the level of evidence remains low for this indication …”

Use of a National Electronic Health Record Network to Describe Characteristics and Healing Patterns of Sickle Cell Ulcers

“…1 An aging sickle cell population suffers from numerous chronic complications that arise in the late adolescent years and fully manifest in adulthood, including sickle cell ulcers, which are estimated to occur in *5-25% of patients with sickle cell disease in the United States. [2][3][4][5][6] Diagnosis is made by identifying a leg, ankle, or dorsal foot ulcer in the context of sickle cell disease. Clinical features typically include a painful ulcer with a punched-out appearance with slough, located on the leg, ankle, or dorsum of the foot.…”

“…Clinical features typically include a painful ulcer with a punched-out appearance with slough, located on the leg, ankle, or dorsum of the foot. 4 Sickle cell ulcers may be similar in appearance to other types of leg ulcers, such as venous stasis ulcers or ulcers associated with rheumatic disease.…”

Infection, Immune Disorders, Hemoglobinopathies, and Lower Extremity Wounds

“…These leg ulcers have a component of venous disease, are 16 times slower to heal with a high recurrence rate of 97%. 4 The Buruli ulcer affects the younger age group resident largely in tropical and subtropical countries. Patients are often contaminated by the bacteria Mycobacterium ulcerans possibly through skin lesions.…”