Mycetoma: A Long Journey from Neglect

Even in the tropical medicine community, mentioning mycetoma often raises eyebrows and requires further explanation. This condition has all the ingredients of neglect: it affects the poorest segment of the population in remote areas, the course of the disease is slow and chronic, and health services in the endemic areas do not have trained staff, adequate diagnostic tools, or treatment. There is uncertainty about the route of transmission, which contributes to lack of effective national control programs. The associated stigma has severe socioeconomic consequences: children drop out of school and their peer group, and young adults cannot finish their training or find a job or a partner. Patients are affected psychologically because of the lack of health services, the physical disability, and the lack of prospects, as the outcome of treatment is poor and often leads to amputation of the affected part [1]. Although mycetoma affects many countries in each part of the (sub)tropics, the disease burden is not well-known [2]. Mycetoma may be caused by bacteria (actinomycetoma), in particular Nocardia spp., or by fungi (eumycetoma), of whichMadurella mycetomatis is the most common. It is thought that the microorganisms enter the skin through a thorn prick or other breach of the skin, after which the typical subcutaneous mass develops, usually on the foot. Swelling, sinus formation, and discharge of grains are considered characteristic of the disease. Although the foot is most commonly affected, all parts of the body may be involved, either directly or through lymphatic or hematogenous spread that may include the spinal cord and the brain. Diagnosis in the field is usually clinical; there is no point-of-care diagnostic test. In referral centers, ultrasound, magnetic resonance imaging (MRI), and fine needle aspiration or biopsy are used for accurate description of the extent of the lesion and the causative microorganism [3]. The key neglect is in treatment; while repeated antibiotic courses with amikacin and cotrimoxazole are used in actinomycetoma with good result (cure rate>90%), this is not the case for eumycetoma. Ketoconazole, which was used previously in many countries, has been banned by the Food and Drug Administration (FDA) and the European Medicines Agency (EMA) because of toxicity, with its use restricted to certain indications. Currently, only itraconazole is used; the treatment duration is long, with a mean of 12 months, after which the remaining lesion is removed surgically. Not uncommonly, the fungus can still be cultured from the surgical specimen, which explains the low cure rate (26%); 55% of patients do not complete the treatment, often because they cannot afford the drug [4]. Recurrence is therefore common and may lead to amputation. The choice of drugs is extremely limited, as only azoles are used: all other classes of antifungals are ineffective in vitro. The issue of treatment is further compounded by concomitant secondary bacterial infection, the variability in the extent and severity of the lesion, and the presence of bone involvement.