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Mucocutaneous Leishmaniasis -A Review of Clinical Aspects

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Mucocutaneous Leishmaniasis -A Review of Clinical Aspects MUCOCUTANEOUS LEISHMANIASIS - A REVIEW OF CLINICAL ASPECTS P. D. Marsden and R.R. Nonata* INTRODUCTION We shall use a simple classification in this discussion. The skin lesions will .be considered In 1910 Gaspar Vianna suggested at a under the general headings of closed and open conference in Belo Horizonte that antimonial since the usual evolution is from a closed lesion therapy was effective in the treatment of to an open ulcerated one. For mucosal lesions mucocutaneous leishmaniasis and this proved to we will use the three evolutionary stages be the case98. In 1926, Montenegro evaluated described by Klotz e Lindenberg60, namely (1) a skin test antigen which is of diagnostic value. nodulation w ithout ulceration; (2) Early It is some measure o f the slow development of ulceration and (3) Late ulceration. clinicai reasearch in this important human A marked feature of both our clinicai infection that today both these discoveries are observations and those in the literature as still being applied as standard practice and still regards these lesions is the extreme variability the laboratory diagnosis and treatment is far in the course of the disease. For example in from satisfactory. some patients severe mucosal damage appears We have written this review because in our relatively early and children may present clinicai work we have been in doubtsometimes irreparable facial damage. Other patients give a about what is the best line of management for history of a minor skin lesion many years our patients. Turning to the literature we did before the onset o f mucous membrane not always find the answer to our questions. involvement and yet others only have a minor This is, as the title suggests, a review concemed skin lesion. Host immunity and the type of with the patients side of the problem and for parasite probably plays an important part in this reason begins with the clinically important this and the little we know about this subject is aspects. Later on we disciiss the relevance of mentioned in our subsequent discussion. some recent biomedical research to our understanding of how this infection behaves in CUTANEOUS LESIONS man. CLOSED LESIONS CLINICAL FEATURES The site o f the bite of the sandfly is generally regarded as the site of the initial The lesions of Mucocutaneous leishmaniasis lesion. The frequency with which innoculation are polymorphic and can closely resemble many of promastigotes by sandflies produces a lesion other skin diseases. This has ied to a number of is not known. Determining factors may include classifications o f skin and mucous membrane the number o f flagellates innoculated, the strain lesions by different workers. For example. of Leishmania and the previous immunological Azulay5 in his thesis in 1952 lists eight experience and genetic makeup of the host. classifications. Although these classifications Quite frequently patients are seen with multiple have value they have become extraordinary early lesions ali about the same stage and these complex and since they are largely descriptive it probably represent the bites of several infected is natural to search fo r a simpler one. sandflies. * University of Brasília, Brazil Subm ittedto publication on 7.20.1975 310 Rev. Soc. Bras. Med. Trop. VOL. IX - NP 6 Like Kala Azar the incubation period must workers6, S8, 1 s. and is essentially a parasite be very variable. Lainson (personal granuloma o f the dermis with secondary communication) has well documented evidence changes in the epidermis. The sequence by it may be as short as 15 days. Azulay and which innoculated promastigotes result in Salgado, in 19668, noted 18— 33 days in intracellular amastigotes in tissue macrophages paratroopers dropped into the Amazonian has not been directly observed in the dermis. It forest. Guimarães, in 1955S3, calculated the is not clear whether promastigotes penetrate average incubation period in a field" study as tissue cells or whether they round up to form being about 2 months. amastigotes which are then ehgulfed by If a lesion does develop it initially takes the macrophages. form of an erythematous macule which The initial histology consists of parasitised develops into a papule and then grows into a macrophages and undifferentiated histiocytes. nodule. The site of the lesions is most After a variable period lymphocytes appear frequently the limbs or face probably because indicating the appearance of a cell mediated they are most frequently exposed. In Central immune response. The intensity of this America lesions of the pinna o f the ear caused response varies greatly and is a major factor by Leishmania mexicana are very common12. influencing the chronicity of the lesion. Usually These lesions can take a variety of forms and the lymphocytic infiltration becomes intense may resemble other infections. They can be with associated plasma cells and eosinophils. listed as.follows: Actual intradermal micro abscess formation can occur with polymorp-h invasion. In chronic 1) Papules with a pustular element — a rare lesions well defined granulomas with giant cells form which could be confused with impetigo — may be encounted. Vascular lesion are also therefore called impetiginous. important with endarteritis and new vessel 2) Follicular papules the induration being at formation. Fibrinoid necrosis of venules was the site o f the f ollicles. regarded by Bittencourt and Andrade1 5 to be a 3) A small furuncular like nodule — has to part of what they considered to be a be distinguished from a simple boil or a histological picture suggesting a hypersensitivity Dermatobia hominis infection. reaction. The epidermis may show irregular 4) Discreet nodules multiple and variable in acanthosis, pseudoepitheliomatous hyperplasia, size sometimes d ifficu lt to differentiate on sight hyperkeratosis, parakeratosis, keratin plugs or from skin tuberculosis, sarcoid or leprosy. partial atrophy. On occasion hyperplasia may These are probably the result of blood stream be so marked as to suggest carcinoma. dissemination and have been termed Diffuse induration of the dermis with little leishmanids" although this term has also been apparent surface skin lesion have been applied to lichenoid, hypochromic lesions described. Lymph gland enlargement is appearing many years afterthe initíal infection. common, usually in primary nodes drainingthe Such lesions have a tuberculoid histology often site o f the lesion. Since spread by the without parasites49. Sometimes on a smaller lymphatics to the circulation is believed to be scale they may resemble the apple jelly nodules the method of dissemination, such enlargement of lupus vulgaris (more common in L. tropica) is not suprising even in closed lesions. In open or give rise to a diffuse infiltration w ith a raised lesions secondary bacterial infection plays an margin resembling the lesion o f tuberculoid important role. leprosy. 5) Hyperkeratotic lesions — such lesions The lymph gland histology exibits the same with a histological appearance o f marked basic processes already described in the skin epithelial activation can produce papillomatous lesions. An initial leucocytic infiltration is lesions resembling the framboesia o f secondary rapidly followed by a marked cellular activation yaws. Condylome type lesions as well can be of lymphocytes, plasma cells and histiocytes. confused with the treponematoses. Verrucose Often leishmania can be seen in the latter. granulomatous lesions may resemble Giant cells are less common than in skin lesions histoplasmosis or chromoblastomycosis. but well defined tuberculoid granulomas may Many of these forms are uncommon and are be present and these may even go on to presumeably governed by the tissue reaction to caseation. the invading parasite. The histopatology has Special mention must be made here of been studied by several groups of diffuse cutaneous leishmaniasis (leishmaniasis NOV-DEZ/75 Rev. Soc. Bras. Med. Trop. 311 tegumentaria diffusa) which is usually a closed (Mycoòacterium ulcerans infection). This entity type of lesion and only rarely ulcerates. The has yet to be reported in Brazil but the lesions take the form of erythematous nodules overhanging edge of skin so characteristic of which closely resemble lepromatous leprosy Buruli ulcer is not a feature of such leishmanial both in their individual appearance and their ulcers. A scrofula like lesion, should be symmetrical distribution with prominent ear distinguished from true tuberculous scrofula or involvement. For this reason patients o f diffuse actinomycosis. cutaneous leishmaniasis have been interned in leprosaria. Similar ear lobe nodulation is also MUCOSAL LESIONS seen in Lobo's Keloidal mycosis96. Histology shows histiocytes rich in leishmania with little A variety of mucous membranes may be lymphocytic infiltration. The Montenegro test affected. Pessoa and Barreto, in 194881, is negative. Usually these patients do not summarising Barbosa's large series producesthe respond to treatment but if they do following incidence figures — Nasal lesions, lymphocytic infiltration of the skin lesion has 1790 patientes; buccal lesions, 209; pharyngeal, been noted to begin and Montenegro test has 170 and laryngeal, 50. Rarer sites include the converted in some instances from negative to conjunctiva and genitals. The over ali incidence positive. The significance of this rare form of of mucosal involvement in different series varies leishmaniasis is discussed later. widely and Azulay5 cites 7 sources with percentages varying from 8% — 80.9%. Since OPEN LESIONS
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