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Lepromatous leprosy: A review and case report. It is transmitted from person to person and has a long incubation period between two and six years. The disease presents polar clinical forms the "multibacillary" lepromatous leprosy and the "paucibacillary" tuberculoid leprosy , as well as other intermediate forms with hybrid characteristics.
They may take the form of multiple nodules lepromas that progress to necrosis and ulceration. The ulcers are slow to heal, and produce atrophic scarring or even tissue destruction. The lesions are usually located on the hard and soft palate, in the uvula, on the underside of the tongue, and on the lips and gums.
There may also be destruction of the anterior maxilla and loss of teeth. The diagnosis, based on clinical suspicion, is confirmed through bacteriological and histopathological analyses, as well as by means of the lepromin test intradermal reaction that is usually negative in lepromatous leprosy form and positive in the tuberculoid form.
The differential diagnosis includes systemic lupus erythematosus, sarcoidosis, cutaneous leishmaniasis and other skin diseases, tertiary syphilis, lymphomas, systemic mycosis, traumatic lesions and malignant neoplasias, among other disorders. Treatment is difficult as it must be continued for long periods, requires several drugs with adverse effects and proves very expensive, particularly for less developed countries.
The most commonly used drugs are dapsone, rifampicin and clofazimine. Quinolones, such as ofloxacin and pefloxacin, as well as some macrolides, such as clarithromycin and minocyclin, are also effective. The present case report describes a patient with lepromatous leprosy acquired within a contagious family setting during childhood and adolescence.
Key words : Mycobacterium leprae, lepromatous leprosy, tuberculoid leprosy, differential diagnosis. Hence it affects the skin, peripheral nerves, the mucosa of the upper airways and other tissues such as bone and some viscera The disease presents polar clinical forms the "multibacillary" lepromatous leprosy and the "paucibacillary" tuberculoid leprosy , as well as other intermediate forms with hybrid characteristics Table 1.
There may also be destruction of the anterior maxilla and loss of teeth 1,2,4,5. The diagnosis, based on clinical suspicion, is confirmed through bacteriological and histopathological analyses in which non-caseating granulomas are observed , as well as by means of the lepromin test intradermal reaction that is usually negative in lepromatous leprosy form and positive in the tuberculoid form 6.
The differential diagnosis includes systemic lupus erythematosus, sarcoidosis, cutaneous leishmaniasis and other skin diseases, tertiary syphilis, cicatricial pemphigoid, lethal midline granuloma, lymphomas, systemic mycosis, traumatic lesions and malignant neoplasias, as well as peripheral nerve pathology such as syringomyelia, among other disorders 2,6.
Quinolones, such as ofloxacin and pefloxacin, as well as some macrolides, such as clarithromycin and minocyclin are also effective 6. Evolution of the disease: Patients with lepromatous leprosy are highly infectious, although most exposed immunocompetent individuals do not contract the disease.
Around one-third of people with leprosy present the first clinical manifestations during childhood. The incubation period of the disease may last up to six years. Once signs have appeared the disease will generally progress slowly unless treated. Its preference for cooler anatomical regions means that vital organs are usually preserved, but also makes leprosy a disease that slowly destroys the distal areas of fingers and toes and causes nasal and facial deformities that lead to social marginalization.
Local pyogenic infections, secondary to skin ulcers, complicate and exaggerate these alterations. Despite these problems the tendency of the disease to preserve the main viscera means that life expectancy is barely reduced. Certain complications, such as pyogenic infection with abscess formation and sepsis, may produce an abrupt and fatal change in the disease course.
Although the disease is uncommon in our milieu the rising immigration of recent years is good reason to take it into consideration. Therefore, it seemed of interest to review the topic by means of a highly representative clinical case.
Male patient aged 65 with no known allergies; he smokes twenty cigarettes a day and has a history of moderate alcohol abuse. He attended a long-stay unit during the summer of for a limited period two months in order to provide family respite. Multiple amputations bilateral metacarpophalangeal, left metatarsophalangeal and nose due to relapsing infection of the septum, performed during the active phase of the disease Figure 1 secondary to the leprous infection.
Without anti-leprosy treatment since due to negativization. This is treated with NPH insulin. Presence of retinopathy, nephropathy and dysautonomia. He was a carrier of the hepatitis C virus HCV. General physical examination showed the patient to be conscious and oriented. His skin was dry, pale and sclerotic. No adenopathies were palpated. The bilateral amaurosis and hypoacusia were noted. He also had problems swallowing and presented solid and liquid dysphagia. He had spontaneous mobility of the extremities, where examination revealed the amputation of fingers and toes of the left foot, as well as the supracondylar amputation of the right lower extremity.
There were suppurating ulcers on the right trochanter and at the head of the left fibula. He suffered from protein-energy malnutrition. He lives with his wife, a daughter and five grandchildren. He is being monitored by his general practitioner and receives domiciliary care for his left knee lesions and diabetes. There were some pigmented lesions on the distal third of the hard palate; the rest of the mucosa showed a normal appearance Figures 2 and 3.
This presented an obstacle in terms of following an adequate dental treatment. Blood tests upon admission corroborated the anaemic state and the hepatic and renal alterations indicated previously. Secretion cultures from the ulcers on the lower extremities right trochanter and head of the left fibula were positive for Staphylococcus aureus. Administration of paracetamol, haloperidol, NPH insulin and rohypnol. Diet to include carbohydrate monitoring and dietary supplements. The diabetes and anaemia to be monitored by the general practitioner, and the hepatopathy by a digestive specialist.
Domiciliary care to be arranged for treating the ulcers. He was advised to see a dental specialist in order to have the root remains removed, and it was explained to him that having full dental prostheses upper and lower fitted would help restore his masticatory function. The symptoms are often so non-specific that the disease is not suspected prior to the appearance of skin lesions.
The nasal mucosa become ulcerated and form scabs. These lesions sometimes bleed to the extent that medical attention is required. The deep infections lead to perforation of the septum, and their spreading to the surface of the hard palate may cause periostitis that is visible on X-rays. From the clinical point of view the greater evidence of pathology appears when the nasal bones, the nasal spine and even the midline maxillary region become affected; the erosion or complete destruction of the latter leads to tissue collapse, with sinking and spreading of the overlying skin and other soft tissues saddle nose.
This external soft tissue deformity constitutes one of the main facial alterations seen in patients with leprosy 1. The nasal secretions are literally teeming with leprous bacilli, and hence airborne dissemination, via sneezing, would seem to be the most likely way for leprosy to be spread nowadays.
This rhinomaxillary syndrome only corresponds to the lepromatous form of leprosy 7 , which is that suffered by the patient described here Figure 1 and, most likely, was what affected his close relatives. At some undetermined point the patient suffers an initial bacillaemia, which may be repeated through recurrent episodes or progress toward a continuous bacteraemia.
Multiple organs are exposed to the bacilli during these episodes. The circulating bacteria are so abundant that they can be detected in peripheral blood samples. However, those which settle in the internal organs are usually effectively eliminated by macrophages. As these bacteria multiply more in cooler areas the pattern of infection is usually focussed on the skin, superficial cutaneous structures especially peripheral nerves , the eyes and the testicles.
The facial skin shows areas of localized swelling, with deep furrows between them. The resulting nodules may become ulcerated. The swollen upper eyelids create a sleepy appearance and the repercussions in the supraorbital tissues usually lead to the loss of eyebrow hair, particularly in lateral areas. The subsequent damage to the sclera and iris may produce blindness.
This nodular dermal appearance, often referred to as leonine facies, is another facial deformity characteristic of leprosy 1,8. The patient reported here suffered both bilateral blindness and leonine facies Figure 1. Lepromatous rhinitis may spread to the maxilla and be complicated by secondary pyogenic infections. Beginning at the midline, the maxillary bone begins to erode to the extent that the palate becomes perforated. This leads to loss of periodontal support for the upper middle incisors, which eventually fall out.
In very advanced cases this process may spread to the sides and even reach the canine teeth. This loss of maxillary bone structures and teeth constitutes another characteristic facial alteration in leprosy 4 , which in the case of our patient contributed to the leonine appearance of the lip Figure 2.
A hard and rigid irregular nerve trunk is sometimes observed in the more superficial areas of the elbow cubital nerve or at the head of the fibula peroneal nerve. However, the absence of a granulomatous form or inflammatory response reduces the tissue destruction characteristic of the tuberculoid form. Hence the leprous bacilli may form large clusters in the nerve sheath, and in some patients this may at first lead to hyperaesthesia.
The persistence of the bacteria and the subsequent destruction of nerve fibres are the cause of anaesthesia in hands and feet 1,9. This was not the case of the patient reported here, who maintained sensitivity. These are hypopigmented in dark-skinned people and erythematous in those with light skin. Their border is much less defined than in the tuberculoid form of leprosy.
Although the trunk may be affected, few lesions are generally observed in this region 1. Our patient showed generalized changes in pigmentation, as can be seen in the figures. This invasion usually occurs indirectly, via lesions in the surrounding soft tissues or, less often, via the blood. In other cases the bone involvement is secondary to peripheral neuropathy.
The skin areas anaesthetised by the nerve infection are susceptible to tissue infection. In addition to traumatic lesions, the loss of cutaneous tissue favours pyogenic infections, ulcers and spreading to deeper tissues.
This leads to arthritis and septic osteomyelitis of non-leprous origin, accompanied by bone destruction and compromised movement due to muscular paralysis also secondary to nerve the damage , which results in osteoporosis and bone atrophy. The phalanx and metacarpal or metatarsal bones are often affected, with marked shortening that may leave only a stump 1.
This consists in the intradermal injection of lepromin extract of the leprous bacillus taken from sick patients. It is usually negative in lepromatous leprosy and positive in the tuberculoid form. However, as positive results are also obtained in patients without leprosy this test only serves to assess the immunological state in already-diagnosed patients 1,6.
Leprous patients with a positive Mitsuda reaction usually present a histological tissue reaction similar to that found in tuberculosis, and hence the name give to this clinical form is tuberculoid leprosy.
[Annular Lichenoid Lesion as Initial Manifestation of Lepra Tuberculoides]
Lepromatous leprosy: A review and case report. It is transmitted from person to person and has a long incubation period between two and six years. The disease presents polar clinical forms the "multibacillary" lepromatous leprosy and the "paucibacillary" tuberculoid leprosy , as well as other intermediate forms with hybrid characteristics. They may take the form of multiple nodules lepromas that progress to necrosis and ulceration. The ulcers are slow to heal, and produce atrophic scarring or even tissue destruction. The lesions are usually located on the hard and soft palate, in the uvula, on the underside of the tongue, and on the lips and gums.
2006, Número 3
The A. Studying by a new thecnics, which he baptised before as "Lleras' method", the scarching of the agent of leprosy in tuberculoid cases, by examination of sub-corium lymph obtained from the lesion, he discovered new forms of the Hansen bacillus, which describes briefly, arriving at the following conclusions: 1. Such bacteriological findigs and the proved mutation of tuberculoid leprosy into lepromatous type, demolished the basis of the so-called "polar" classification of leprosy. Considering the proved facts already referred to, the A.
[Nodular Lepra Tuberculoides in a Child]