Finally, in 1911, mucosal leishmaniasis joined the list of parasitic woes. That’s when an Italian doctor in Brazil reported the case of a 45-year-old agricultural worker with growths in his mouth. “The entire palate showed gross alteration,” Antonio Carini wrote in the Bulletin de la Societé de Pathologie Exotique. “Its surface was irregular and covered with vegetating protuberances. [In addition,] the uvula was destroyed, and in its place there was a fleshy mass the volume of a walnut.”
Later that year, a Peruvian doctor expanded upon la espundia, a local term for the same scourge described by Carini. According to Edmundo Escomel, espundia sometimes destroyed an entire nose and mouth, condemning many to recurrent bouts of pneumonia or even fatal suffocation. Fortunately, we now know that only a handful of South American strains — most notably Leishmania brasiliensis (which today is also called Vianna brasiliensis) — can metastasize to the face years after the bite of a small, hairy sandfly that had acquired its cargo from Leishmania-infected rodents.
A century after Escomel’s report, one more fact may help to explain Luciana’s long-delayed diagnosis. In espundia, ravaged mucosae contain few Leishmania parasites. Instead, like miniature storm troopers, inflammatory cells carpet affected tissues. Medically speaking, their distinctive cellular rosettes are called granulomas.
After viewing her original biopsy laden with rosettes, Luciana’s doctors mistakenly labeled her with Wegener’s granulomatosis. Until the patient received her final immunosuppressive drug, that is. Once on cyclophosphamide, her parasites burgeoned.
Looking back, you could say that destino (Portuguese for “destiny”) had finally dealt Luciana a lucky hand.
A Difficult Cure, a Happy Ending
I first met Luciana a few days after the biopsy that would change her life. After quietly trading hellos and scanning her face and skin, I moved into the hall to discuss her treatment with other specialists. The first step was to taper off immunosuppressives like prednisone and cyclophosphamide as quickly as possible.
But most important was a drug that would quash Leishmania brasiliensis. Once Centers for Disease Control and Prevention experts reviewed her case and approved the drug’s release, Luciana would qualify to receive Pentostam, an old, toxic, but powerful weapon containing the heavy metal antimony. In the meantime, she started on the next best alternative: an intravenous anti-fungal that also storms macrophages, thus stun-gunning Leishmania.
The anti-fungal brought respite, but its effects were short-lived. Eventually, rather than make multiple daily trips for outpatient infusions of Pentostam, Luciana and her husband returned to Brazil. There, comfortably hospitalized and surrounded and supported by family, Luciana received two extended courses — one lasting 30 days, the other 45 — of a close relative of Pentostam. And, yes, the toxic salvos cured her. Years later, proof can be found in serial photos in a medical journal. The final image, taken two years after treatment, shows a pleasing face with normal contours and a clear, healthy hue.