Endemic pemphigus foliaceus or fogo selvagem (FS) in an organ-specific autoimmune skin disease characterized by epidermal vesicles and mediated by autoantibodies. Family cases are frequent and not everyone living in endemic region develops FS suggesting that host factors play a role in determining whether exposed individuals will be affected. Because our previous works with Brazilian Mestizos and with Xavante Indians have shown that particular HLA alleles confer increased risk for the disease, we decided to extend these studies to another homogeneous population, the Terena Indians. 19 out of 20 Terena patients were either positive for DRB1*0404, 1402 or 1406 (p < 0.005, RR = 14). These findings were in agreement with the data obtained from the Xavante study. In Mestizos the association was with DRB1*01. All these alleles involved in predisposition to the disease in different populations shared the same amino acid sequence at position 67-74 on the third hypervariable region of the DRB1 gene: LLEQRRAA, suggesting that inheritance of this sequence is involved in the susceptibility to FS. When patients and controls data from different studies were pooled and analyzed disregarding the ethnic background and the HLA alleles involved, the results obtained clearly supported the hypothesis that matching for this epitope is highly significant and predictive of FS predisposition (p < 0.00001, RR = 6.4).

Pemphigus are intraepidermal autoimmune bullous dermatoses that occur with lesions on the skin and / or mucous membranes. The most frequent types are pemphigus vulgaris and pemphigus foliaceus (classic and endemic). This consensus aims to present a complete and updated review of the treatment of these two more frequent forms of pemphigus, based on the literature and the personal experience of the authors. In moderate and severe cases of pemphigus vulgaris and foliaceus, systemic corticosteroid therapy (prednisone or prednisolone) is the treatment of choice. Adjuvant drugs, usually immunosuppressive drugs (azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide) may be prescribed as corticosteroid sparers in refractory cases or with contraindications to corticosteroids to minimize side effects. In severe and nonresponsive cases, corticosteroids in the form of intravenous pulse therapy, immunoglobulin and plasmapheresis / immunoadsorption can be administered. Immunobiological drugs, particularly rituximab, appear as a promising alternative. For milder cases, smaller doses of oral corticosteroid, dapsone and topical corticosteroids are options. At the end flowcharts are presented as suggestions for a therapeutic approach for patients with pemphigus vulgaris and pemphigus foliaceus.

FUNDAMENTOS: Leishmaniose Tegumentar Americana é zoonose de manifestações clínicas variadas, em expansão no Brasil, sendo o estado de Mato Grosso do Sul importante área endêmica. OBJETIVOS: Avaliar clínica, epidemiológica e laboratorialmente pacientes com Leishmaniose Tegumentar Americana , atendidos no Hospital Universitário Maria Aparecida Pedrossian, Universidade Federal de Mato Grosso do Sul, Brasil (HU/UFMS). MÉTODOS: Trata-se de um estudo observacional do tipo transversal com abordagem descritiva e analítica. Foram avaliados, retrospectivamente, dados de pacientes suspeitos de Leishmaniose Tegumentar Americana , atendidos no HU/UFMS de 1998 a 2008, e encaminhados ao Laboratório de Parasitologia/UFMS para complementação diagnóstica. Para a inclusão neste estudo foram considerados critérios clínicos e laboratoriais. RESULTADOS: Quarenta e sete pacientes foram incluídos no estudo. Houve predominância de homens de 45 a 59 anos, com a forma cutânea, lesão única, ulcerada, em áreas expostas do corpo e com duração menor que seis meses. O comprometimento de mucosas foi crescente com o aumento da idade e maior em pacientes que procuraram atendimento tardiamente. Intradermorreação de Montenegro foi o exame de maior sensibilidade e o encontro do parasito mostrou-se mais difícil em lesões antigas. CONCLUSÃO: Suspeição diagnóstica precoce é de extrema importância para diagnóstico preciso. Associação de exame parasitológico e imunológico torna mais seguro o diagnóstico laboratorial.

A study on the resident population of 150 inhabitants of Boa Sorte in the Municipality of Corguinho, Mato Grosso do Sul State, Brazil was made, from March 1991 to March 1994, to establish the prevalence of South American Cutaneous Leishmaniasis (SACL), and to characterize the affected population, in an area of recent transmission. Twelve of the inhabitants showed lesions suspected to be SACL, and in 8 cases it was possible to confirm this by biopsy and parasitology. The mucosal form was found in one patient only, the rest showed the following cutaneous forms: ulcerated (3), ulcero-verrucose (1), hyperkeratotic ulcer (1), infiltrated maccule (1), nodule with florid regional adenopathy (1). All patients reacted favorably to treatment with glucantime, with lesion scarring. Side-effects were rare. The parasite isolated from all patients was identified as Leishmania (Viannia) braziliensis. The Montenegro skin test, applied to the 150 inhabitants, showed 32 reactive ones. Of these, six were carriers of the disease, 21 showed sequelae suggestive of the disease and five showed no signs of infection. The age grouping of the cohort ranged from 22 to 78 years, 75% being male. To date, transmission is suspected to be in the peridomicile.