The diffuse
leprosy
Leprosy, also known as Hansen's disease (HD), is a long-term infection by the bacteria ''Mycobacterium leprae'' or ''Mycobacterium lepromatosis''. Infection can lead to damage of the nerves, respiratory tract, skin, and eyes. This nerve damag ...
of
Lucio
Lucio is an Italian and Spanish male given name derived from the Latin name ''Lucius''. In Portuguese, the given name is accented Lúcio.
Lucio is also an Italian surname.
Given name
* Lúcio (Lucimar Ferreira da Silva) (born 1978), Brazilian ...
and
Latapí, also known as diffuse lepromatous leprosy or "pretty leprosy" is a clinical variety of
lepromatous leprosy
Lepromatous leprosy is a form of leprosy characterized by pale macules in the skin.
It results from the failure of Th1 cell activation which is necessary to eradicate the mycobacteria (Th1 response is required to activate macrophages that engulf ...
. It was first described by Lucio and Alvarado in 1852 and re-identified by Latapí in 1936. It is common in
Mexico
Mexico (Spanish: México), officially the United Mexican States, is a country in the southern portion of North America. It is bordered to the north by the United States; to the south and west by the Pacific Ocean; to the southeast by Guatema ...
(23% of leprosy cases) and in
Costa Rica and very rare in other countries.
History
The spotted or lazarine leprosy was first described by Ladislao de la Pascua in 1844.
[Vargas-Ocampo F (2007) Diffuse leprosy of Lucio and Latapí: a histologic study. Lepr. Rev. 78(3):248-260] Lucio and Alvarado published a description of the disease with the same names in 1852. Latapí re-described it in 1938 and reported it as 'spotted' leprosy of Lucio in 1948. It was named the diffuse leprosy of Lucio and Latapí in 1963 by Frenken.
The underlying pathology was explained by Chévez-Zamora as a diffuse generalised cutaneous infiltration. He named it pure and primitive diffuse lepromatosis, upon which necrotising lesions develop. He proposed the name Fenómeno de Lucio or erythema necrotisans for these lesions.
Clinical features
This condition is characterized by:
[Saúl A, Novales J (1983) Lucio-Latapí leprosy and the Lucio phenomenon. Acta Leprol. 1(3):115-132]
* a diffuse infiltration of all the skin which never transforms into nodule
* a complete alopecia of eyebrows and eyelashes and body hair
* an
anhydrotic and
dysesthesic zones of the skin
* a peculiar type of lepra reaction named
Lucio's phenomenon or necrotic
erythema
Erythema (from the Greek , meaning red) is redness of the skin or mucous membranes, caused by hyperemia (increased blood flow) in superficial capillaries. It occurs with any skin injury, infection, or inflammation. Examples of erythema not asso ...
Lucio's phenomenon consists of well-shaped erythematous spots which later become necrotic with scabs, ulcerations and scars. These lesions are usually located on the lower extremities and may be extensive. They are frequently painful and rarely fatal.
Pathology
The main pathological features of this disease are a
vasculitis
Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused ...
affecting all cutaneous vessels.
There are by five characteristic features:
* colonisation of endothelial cells by acid-fast bacilli
* endothelial proliferation and marked thickening of vessel walls to the point of obliteration
*
angiogenesis
* vascular
ectasia Ectasia (), also called ectasis (), is dilation or distention of a tubular structure, either normal or pathophysiologic but usually the latter (except in atelectasis, where absence of ectasis is the problem).
Specific conditions
* Bronchiectasis ...
* thrombosis of the superficial and mid-dermal blood vessels
The likely pathogenesis is endothelial cell injury due to colonization/invasion followed by proliferation, angiogensis, thrombosis and vessel ectasia.
Treatment
Lucio's phenomenon is treated by anti-leprosy therapy (
dapsone,
rifampin
Rifampicin, also known as rifampin, is an ansamycin antibiotic used to treat several types of bacterial infections, including tuberculosis (TB), ''Mycobacterium avium'' complex, leprosy, and Legionnaires’ disease. It is almost always used tog ...
, and
clofazimine
Clofazimine, sold under the brand name Lamprene, is a medication used together with rifampicin and dapsone to treat leprosy. It is specifically used for multibacillary (MB) leprosy and erythema nodosum leprosum. Evidence is insufficient to sup ...
), optimal
wound care
The history of wound care spans from prehistory to modern medicine. Wounds naturally heal by themselves, but hunter-gatherers would have noticed several factors and certain herbal remedies would speed up or assist the process, especially if it w ...
, and treatment for
bacteremia including
antibiotics
An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting bacterial infections, and antibiotic medications are widely used in the treatment and prevention o ...
. In severe cases
exchange transfusion may be helpful.
[Kasper DL, Braunwald E, Fauci AS, et al. Harrison's Principles of Internal Medicine. 16th edition. McGraw-Hill. 2005. Vol I. p.971.]
References
{{DEFAULTSORT:Diffuse leprosy of Lucio and Latapi
Leprosy