RESUMO
Mal de Meleda is an hereditary palmoplantar keratoderma named for the Mljet Island in Croatia. The lives of those affected by this disease represent a complex situation that encompasses members of a vulnerable group. They require enlightenment and should be approached with awareness, taking into account their overall psychophysical status and the environment of each patient. Those afflicted with Mal de Meleda not only have to deal with a difficult life due to their affliction, but they also must cope with the hardships of socialization while trying to realize a normal life within their island community. This is compounded by the frequent interviews and examinations of researchers interested in the various aspects of their illness. The subject of this contribution is not about the nature of this disease, rather about the traces it has left on the (sub)consciousness of the population. It is also concerned with exploring ways of how to access patients and understanding the depth of their vulnerability. We present some thoughts tied to the interpersonal experiences of researchers and patients afflicted with Mal de Meleda.
Assuntos
Ceratodermia Palmar e Plantar , Estereotipagem , Conscientização , Croácia , Ética , História do Século XVIII , História do Século XIX , História do Século XX , História Medieval , Humanos , Ceratodermia Palmar e Plantar/genética , Ceratodermia Palmar e Plantar/história , Ceratodermia Palmar e Plantar/patologia , Ceratodermia Palmar e Plantar/psicologia , Hanseníase , Distância Psicológica , Qualidade de Vida , Isolamento Social , Rede Social , Percepção Social , Populações VulneráveisRESUMO
Meleda disease is an indigenous dermatological disease classified as a hereditary palmoplantar keratoderma. The disease was first described on the island of Mljet, Croatia, by Luko Stulli in 1826. We present a historical review of the literature data throughout the centuries till today. Recently, the gene responsible for the disease has been identified on chromosome 8qter within the cluster of Ly-6 homologous human genes. Various mutations in the secreted LY6/PLAUR-related protein1 gene (SLURP1) located on the aforementioned chromosome were identified as the cause of the disease. Due to similarity between the islands of Malta and Mljet, we are proud of the fact that, to the credit of Croatian researchers and scientists, Mal de Meleda entered the international scientific literature under that very name and has preserved it until today.
Assuntos
Ceratodermia Palmar e Plantar/história , Cromossomos Humanos Par 8 , Croácia/epidemiologia , História do Século XIX , História do Século XX , História do Século XXI , Humanos , Ceratodermia Palmar e Plantar/epidemiologia , Ceratodermia Palmar e Plantar/genética , MutaçãoRESUMO
Nowadays, hereditary diseases are viewed through molecular mechanisms, and one of them, which keeps occurring rather frequently in medical publications, has been named after the Island of Mljet. The world first learned about mal de Meleda from a Dubrovnik physician Luka Stulli in 1826. He described it in a number of his island patients as a non-contagious hereditary skin disease, and named it mal de Meleda (a disease of Mljet). After Stulli, numerous scientists continued to investigate its aetiology and distinctive properties, introducing new scientific procedures to research the disease. The article keeps track of the way people and medicine perceived and treated mal de Meleda patients, starting from the early 19th century to the present day. It pays special attention to how the disease was perceived and described in medical literature through history. There are no reports in writing about the disease before the 19th century, but in oral tradition it was perceived as a punishment for the sins of sacrilege, sins of piracy, or even as leftovers of leprosy brought by the crusaders. We investigated if these legends have any support in preserved historical documents and to what extent they are related to real historical events and circumstances. Influenced by the booming research in microbiology, end 19th century physicians believed the disease was an isolated focus of leprosy. However, early 20th century physicians defined it as a hereditary skin disease with changes which distinguish it from other skin conditions. Genetic nature of the disease was later confirmed by molecular science. As for its geographical origin, the most recent medical research has shown that mal de Meleda is not restricted to the island of Mljet, and that it is spread worldwide, particularly in regions that, historically, were the trading routes of the Dubrovnik Republic. This implies that the mutation has spread through migration and persists only because it is not lethal and does not affect reproduction.
Assuntos
Croácia , História do Século XIX , História do Século XX , Humanos , Ceratodermia Palmar e Plantar/históriaAssuntos
Displasia Arritmogênica Ventricular Direita/história , Displasia Arritmogênica Ventricular Direita/genética , Eletrocardiografia , Grécia , História do Século XX , Humanos , Ceratodermia Palmar e Plantar/genética , Ceratodermia Palmar e Plantar/história , Mitologia , Terminologia como AssuntoRESUMO
Luca Stulli of Dubrovnik (Ragusa), 1772-1828, was one of the first to make epidemiologic studies of heritable skin disorders. His treatise of what became the 'mal de Meleda' on the Adriatic island of Mljet (Meleda) is a classic in the dermatologic literature. The present study documents his life, his birth record, his portrait and recalls his original publication (in Italian).
