Reação Hansênica Tipo 1 Ulcerada

Abstract

At the thirtie's Rodriguez e Wade described a patient who they labeled as borderline that after an evolution of ten years presented ulcerated lesions all over his body. Ryrie at Malaysia also wrote about ulcerated tuberculoid cases. Today all leprosy workers are familiar with these cases but so far there are not many studies about them. Reactional tuberculoid (RT) and Reactional borderline (RB) cases with ulcerated lesions are not
frequent. During the period of 10 years (1987 to 1998) it were admitted in our Institute 316 reactional cases (183 RT and 133 DR), and only 20 of such patients had ulcerated lesions. We studied 15 of these cases. There were 3 males and 12 females. The age ranged from 30 to 76 years, and all of them were white. All cases were reactional ones and showed several lesions all over their skin. Data about classification, number of reactional
episodes, bacilloscopy and Mitsuda test were provided . The patients were classified as RT and RB based on clinical, bacterioscopic,  istopathological and Mitsuda test results. RT cases showed Mitsuda tests equal 6 mm or higher, and the bacilloscopy was negative or with few bacilli. There were 13 RT and 2 RB patients. All patients showed several erythematous papules, nodules and plaques. The RB ones showed more edema and more neural involvement. Characteristic lesions were seen on the face, around the eyes, nose and mouth, palms and soles. Some patients had many ulcerated lesions and others had only a few Only two cases showed all the lesions with ulceration. Circular shallow ulcers involved all the lesions or were observed only near their edges, given it an annular configuration. From a histopathological point of view we observed a moderate epidermal hiperplasia. The tuberculoid granulomas were large and soft formed by epithelioid cells, foreign body giant cells, Langhans giant cells and lymphocytes. Often the histiocytes spread to the neigbouring stroma and sometimes they invaded the epidermis. There was intersticial and intracellular edema and the lymphocytes showed variations in its distribution. Necrosis exhibited a fibrinoid pattern and in some places there was a caseous pattern of these necrosis. In general, we observed a uncharacteristic vascular proliferative reactivity and in rare cases we noted a granulomatous vasculitis. Themajority of these ulcerated cases showed high levels of cell mediated immunity as showed by the values of Mitsuda reaction and the frequent negative bacilloscopy.The ulcerations occurred during the second or third reactional episodes in almost all the patients, and only in 3 of them the lesions ulcerated during the first episode. We know that the activation of macrophages by lymphocytes may limit the infection, but continuous stimulation may lead to tissue damage through the release of macrophage products including reactive oxygen intermediates and hydrolases and this is due to increase in TNF-a. In granulomatous reactions the activated macrophages become a major source of TNF-a and the granulomas develop by auto-amplification, with differentiation of macrophages into epithelioid cells which produce large amounts of TNF-a leading to tissue necrosis.This could be an explanation for the appearance of ulceration in our patients. In our opinion, in the reactional cases occur amultiplication of bacilli, its destruction by body defences or treatment, and consequently a hipersensitivity reaction due to the antigens released. If the immunity level is high the bacilli are destroied and there will not be another reactional episode. However, in cases where the immunity is not so efficient, some persisters bacilli may remain and when the conditions become more favourable for its multiplication there will be a new reactional episode. If the interval between these episodes is short the stimulation of the macrophages becomes continuous and there will be the possibility of ulcerations by the mechanism discussed above. Another hypothesis to try to explain why ulcerations occur in some reactional tuberculoid and borderline cases, could be to admit the possibility of theses cases to be a selected genetic group that show homozigose for the allelus TNFB2 which is related with patients that show very high plasmatic concentrations of TNFa when compared to heterozigotous and homozigotous individuals for the alellus TNF B1. TNF genes are polimorphic and are localized on the short armof cromosome 6 inman, close to the genes of the Major Histocompatibility Complex (MHC), in the class II region. This would explain liaisons among some MHC genotypes and the potential to produce seric TNF