Lucio's phenomenon: exuberant case report and review of Brazilian cases

Abstract

Lucio’s phenomenon is an uncommon reaction characterized by severe necrotizing cutaneous lesions that occurs in patients with Lucio’s leprosy and lepromatous leprosy. It is considered by some authors as a variant of type 2 or 3 reaction. Death can occur because of blood dyscrasia or sepsis. Precipitating factors include infections, drugs and pregnancy. We report a 31-year-old female patient exhibiting both clinical and histopathological features of lepromatous leprosy and Lucio’s phenomenon presenting favorable response to treatment. We complement our report with a literature review of the Brazilian cases of Lucio’s phenomenon published in Portuguese and English.

Hansen’s disease with Lucio’s phenomenon in a country in the post elimination era: A case report

“…[2,9] The Lucio's phenomenon corresponds to a necrotizing vas-culitis with microscopic thrombosis and is one of the clinical manifestations of lepromatous leprosy, [8] requires for its diagnosis to document the presence of multibacillary leprosy and the histopathological diagnosis of necrotizing vasculitis in patients without visceral symptoms with painful plaques or nodules that tend to ulcerate, who generally have not received treatment. [6,7,9,10] This usually starts in the feet and advances cephaladically towards the hands, back and face, initially the lesions debut as erythematous macules of different sizes and shapes, which evolve at 24 to 48 hours, and on the third and fourth day to dark purpurical lesions that end in small blisters due to central necrosis, nevertheless, our case presented in the latter phase with necrosis of the fleshy parts of the distal phalanges. [7,8] Usually these patients present alopecia, lagoftalmos, nasal septum destruction or perforation, some of them compatible with the presented case.…”

“…[5,6] During the chronic evolution of the disease may appear acute inflammatory manifestations called leprotic reactions, which constitute expression of immunoreactivity in which there is compromise of the general condition of the patient with polymorphous skin lesions and severe neural, ocular and visceral involvement that may even lead to death. [4,7] Leprotic reactions may occur before, during, or after appropriate treatment; occur spontaneously or in relation to other infections, stress situations or in situations that alter the patient's immune status.…”

“…[7,8] The latter is relatively rare and there are no descriptions in Colombia about it, as a reference in South America, in Brazil approximately 11 cases have been reported since 2000. [6] Lucio's phenomenon is almost exclusively limited to Mexican and Central American patients, although it is rarely reported in South America especially in Colombia. [6] Taking into account that Colombia leprosy was considered eliminated (with a prevalence of less than 1 case per 10,000 inhabitants since 1997), and considering that the country is in a post-elimination phase, it is quite rare to find such cases, as well as the previously presented.…”

“…[6] Lucio's phenomenon is almost exclusively limited to Mexican and Central American patients, although it is rarely reported in South America especially in Colombia. [6] Taking into account that Colombia leprosy was considered eliminated (with a prevalence of less than 1 case per 10,000 inhabitants since 1997), and considering that the country is in a post-elimination phase, it is quite rare to find such cases, as well as the previously presented. [2,9] The Lucio's phenomenon corresponds to a necrotizing vas-culitis with microscopic thrombosis and is one of the clinical manifestations of lepromatous leprosy, [8] requires for its diagnosis to document the presence of multibacillary leprosy and the histopathological diagnosis of necrotizing vasculitis in patients without visceral symptoms with painful plaques or nodules that tend to ulcerate, who generally have not received treatment.…”

“…[7,8] Usually these patients present alopecia, lagoftalmos, nasal septum destruction or perforation, some of them compatible with the presented case. [6,8] In spite of the above, it is demonstrated that leprosy continues to have a stable incidence with 400 to 500 new cases each year, with which the cohabiting ones become a population of high risk for new infections with percentages near 4%, demonstrated by IgM titers for PGL1 according to RomeroMontoya's work et al [9] Treatment should be done with the anti-tuberculosis medicines as previously described (dapsone, rifampicin and clofazimine); however, Lucio's phenomenon usually responds to steroids at a dose of 1 mg/kg/day with a gradual descent pattern, [10] which corresponded to the handling instituting in the exposed case. The syndrome usually occurs in untreated leprosy patients or in patients taking irregular treatment, which agrees with the case presented.…”