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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.3 Rio de Janeiro May/June 2004
WHICH IS YOUR DIAGNOSIS?
Case for diagnosis*
Cyntia Sumire CootiI; Lílian NishinoII; Sérgio Zuñeda SerafiniIII
ISecond-year specialization, Dermatology
Service, the Federal University of Parana Hospital de Clinicas
IIAssistant M.D. and lecturer, Dermatology Service, Federal University of Parana Hospital de Clinicas
IIIM.D., Professor, Federal University of Parana Hospital de Clinicas
HISTORY OF THE DISEASE
A 48-year-old Caucasian, male patient, married and employed as a construction foreman, was born and raised in Campo Mourão (in the western part of Parana state). He received medical attention at the Outpatients Dermatology Service after complaining of an ulcer on the left thigh, and an erythematous and infiltrated lesion on the face.
The patient referred to the paucisymptomatic appearance (during infancy) of the ulcer on the left thigh, which kept developing over a long period of time. In addition, he had an erythematous and infiltrated lesion on the glabella, which appeared recently. He denied having any local trauma. The patient had already received medical assistance at another service (the Oncology Hospital) at which time he had been subjected to three histopathological tests that revealed only chronic and acute inflammation with fibrosis and an absence of malignancy. As for a previous and family pathological background, he did not show any relevant data. There were no similar cases among his family members or friends.
The dermatological examination revealed a deep ulcer, measuring roughly 15 cm in depth and localized on the anterior region of the left thigh. The ulcer border was raised, and the base granulous and rough. It was covered with a purulent secretion, with creams and lotions being visible within the ulcer. The glabella region revealed an erythematous and infiltrated lesion, 3 cm in diameter (Figures 1 and 2).
The patient was subjected to new biopsies and laboratory tests.
Subsequent to a clinical assessment, the following diagnostic hypotheses were made: leishmaniasis, gangrenous pyoderma, tuberculosis, factitial dermatitis, neoplasia, cryptococcosis, atypical mycobacteria and tertiary syphilis (lues).
Routine laboratory (hemogram, biochemical, metabolic) tests were normal. VDRL and FTA-Abs were not reactive. Examination for fungi, BAAR, atypical mycobacteria and common germs proved negative. Histopathological tests revealed a discretely reactive epidermis and predominantly mononuclear inflammatory infiltrate with scattered neutrophils within the dermis, as well as an absence of granuloma and microorganisms (Figure 3).
The case was presented and discussed at the 2nd Jornada Paranaense de Dermatologia (2nd Parana-State Meeting on Dermatology). The diagnostic hypothesis of gangrenous pyoderma was put forth, with an indication of systemic corticotherapy.
After 60 days of 1 mg/kg daily Prednisone use, the patient experienced a sudden worsening of the lesions, which included ulceration of the face lesion, and was then submitted to a new biopsy.
The new histopathological test demonstrated mononuclear inflammatory infiltrate with scattered neutrophils, but in the presence of an abundance of microorganisms and small quantities of epithelioid histiocytes (Figure 4). The patient was then submitted to two cycles of glucantime, and progressed until complete healing of the lesions (Figures 5 and 6).
DIAGNOSIS AND COMMENTS
American Cutaneous Leishmaniasis
Cutaneous/mucous leishmaniasis is a chronic infectious disease that is not contagious. It affects the skin and, depending on the leishmania type and other factors, may affect the mucosas and cartilage.1 It is transmitted by women infected by Phlebotominae sand flies (or "mosquitoes"), known in Brazil as birigui, mosquito-palha or tatuquira.2 The dermatological manifestations of American Cutaneous Leishmaniasis are varied and rarely specific. The lesions may present as ulcerated (like those observed in the case discussed herein), ulcerous-crustose, ulcerovegetant, tuberous, or verrucous. They may have an impetigo, eczematoid and lichenoid aspect.3 A diagnosis may be established by means of clinical and epidemiological data, in addition to microbiological and immunobiological tests. In cases of non-specific and long-term lesions immunobiological tests may be required so as to confirm the diagnosis, owing especially to a scarcity of microorganisms. In Brazil, the following forms have been described: Leishmania (Leishmania) amazonensis, Leishmania (Viannia) braziliensis and Leishmania (Viannia) guyanensis. Cutaneous localized leishmaniasis is endemic to Central and South America. It is caused by a species belonging to the Leishmania braziliensis and Leishmania mexicana complexes.4
Leishmania (V.) braziliensis has a vector in the interior of São Paulo state, while Lutzomyia intermédia has one in Parana state. It is present mainly in forest regions in São Paulo, Parana, Minas Gerais and Mato Grosso states, being responsible for the most destructive form of cutaneous leishmaniasis to be found in the New World. Characterized by a single ulcer, or very few of them, they are often large, persistent and disfiguring, with an abundance of inflammatory tissue reaction and lack of parasites.4 Leishmaniasis depends on virulence factors of the leishmania infectans species and the host's immune response. As such, depending on these factors, one might observe localized, generalized or mucous lesions of this subclinical infection that may even be anergic. In an experimental leishmaniasis model, type Th1 and Th2 lymphocytic responses were established. The latter relied on the species type of the inoculated animal to be resistant or sensitive to the species of inoculated leishmaniasis. It also had to be in agreement with cytokine production. This model was transmitted to man, which demonstrates that, in localized form, there is predominance of IL-2 production and, in mucocutaneous and diffuse forms, there is predominance of IL-4 and IL-10. These configure the Th1 and Th2 responses, respectively.4
Corticoids are known to suppress the function of monocytes and lymphocytes (Th1 and Th2 responses). This effect is clinically important since granulomatous infectious diseases have a propensity to exacerbation and relapses during prolonged corticotherapy.5
When confronted with atypical lesions in endemic areas, it is always important to consider leishmaniasis, mainly when faced with difficult to diagnose dermatological conditions. In the case reported, an equivocal medical action ended up making a definition of the case possible and subsequently led to a proper strategy for treating it.
1. Talhari S, Talhari AC, Ferreira LCL, Naiff R. Leishmaniose cutâneo-mucosa. In Dermatologia Tropical. São Paulo: MEDSI, 1995: 23-45.
2. Sampaio SAP, Rivitti EA. Leishmaniose e outras dermatoses por protozoários. In: Dermatologia. São Paulo: Artes Médicas, 1998: 565-74.
3. Brotas AM, Diniz AR, Volta AC, Russi DC, André ASD. An bras Dermatol 2002; 77(1): 109-112.
4. Medeiros ACR, Roselino AMF. Leishmaniose tegumentar americana: do histórico aos dias de hoje. An bras Dermatol 1999; 74(4): 329-336.
5. Werten VP, Lazarus GS. Dermatology in General Medicine. 5th ed, vol II, section 36, 2783-89.
Cyntia Sumire Cooti
Rua Coronel Amazonas Marcondes, 262 - Cabral
Curitiba PR 80035-230
Tel./Fax: (41) 252-3586
* Work done at Dermatology Service of "Hospital de Clínicas, Universidade Federal do Paraná - UFPR".