This is a report on four persons in one family with a condition similar to that described by Ramon et al [Oral Surg 24:436-48, 1967] in two sibs born to a consanguineous couple. Our patients also had mental deficiency, epilepsy, cherubism due to fibrous dysplasia of the maxillae, gingival fibromatosis, hypertrichosis, and stunted growth. This appears to be an autosomal recessive trait in both families. Our patients are the second set reported with this syndrome; they also have juvenile rheumatoid arthritis, which was not described in the family reported by Ramon et al [Oral Surg 24:436-48, 1967]. We conclude that the Ramon syndrome should also include juvenile rheumatoid arthritis.

Axillary masses are uncommon alterations when detected as an isolated finding. We evaluated 31 patients with isolated axillary masses. Patients with alterations of the breasts or the upper limbs or with ipsilateral chest lesions were excluded from the study. Nine patients had occult breast cancer, 5 of them in the contralateral breast. Seven had metastatic lymph nodes of non-ductal origin, and 1 had carcinoma of apocrine cells with metastasis to the axilla. Four patients had benign lymphadenopathy which disappeared spontaneously, and 4 others had ruptured infundibular follicular cyst, nodular fibromatosis, inflammatory tuberculous and inflammatory rheumatoid lymphadenitis. Five had an ectopic breast (2 with a fibroadenoma and 3 with fibrocystic changes). One patient had an axillary lipoma. The mean age of patients with malignant pathology was 55.1 ± 10.9 years, and the mean age of patients with a benign pathology was 43.1 ± 14.7 ( P <0.01). Chest X-ray and bilateral mammography are useful when the cause of the mass cannot be determined by taking a detailed history of neoplastic or infectious antecedents, by careful physical examination of the skin of the arms, trunk and neck, or by palpation of the breasts and thyroid. Fine needle aspiration biopsy distinguishes between benign and malignant pathologies. In cases of indeterminate neoplasia, complete axillary dissection for diagnosis is indicated.

We report the second case of infection with Chromobacterium violaceum that occurred in Brazil. A farm worker living in the State of São Paulo presented fever and severe abdominal pain for four days. At hospitalization the patient was in a toxemic state and had a distended and painful abdomen. Chest X-ray and abdominal ultrasound revealed bilateral pneumonia and hypoechoic areas in the liver. The patient developed failure of multiple organs and died a few hours later. Blood culture led to isolation of C. violaceum resistant to ampicillin and cephalosporins and sensitive to chloramphenicol, tetracyclin, aminoglicosydes, and ciprofloxacin. Autopsy revealed pulmonary microabscesses and multiple abscesses in the liver. The major features of this case are generally observed in infections by C. violaceum: rapid clinical course, multiple visceral abscesses, and high mortality. Because of the antimicrobial resistance profile of this Gram-negative bacillus, for appropriate empirical antibiotic therapy it is important to consider chromobacteriosis in the differential diagnosis of severe community infections in Brazil.

A 12-year-old girl had been presenting a woody infiltration and erythema in the frontal region and on the entire left half of the face, leading to deformity of the nose and buccal fissure, and adenomegaly in a posterior cervical chain, for the last 18 months. Sinusitis was diagnosed and treated with antibiotics, and submitted to ethmoid sinusotomy, with no improvement. Several laboratory tests were made to find the correct diagnosis. An intradermal test for delayed hypersensitivity showed a positive reaction (5 mm) with necrosis for metabolic antigens for Conidiobolus. An oral mucosa biopsy showed a dense lymphohistiocytic infiltrate and focal points of necrosis. Gomori staining for fungi revealed countless wide, nonseptate hyphae. Amphotericin B was prescribed during 35 days, with no improvement. Terbinafine given orally was started in association with amphotericin B. Reduction of the lesions was observed 2 months later. No side effects were seen during 4 months of treatment.

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Casos de hidatidose policística têm sido des critos nos países amazônicos vizinhos do Brasil2 e também na Amazônia brasileira^ 9. O agente etioló-gico dessa doença é o Echinococcus vo g elfi11 que tem como hospedeiro intermediário principal a paca (Cuniculus paca). Fora do território brasileiro, cistos de E. vogeli já foram identificados em pacas captu radas na Colômbia3 e na Bolívia5. O homem, hospe deiro intermediário alternativo, adquire a doença contaminando-se por via oral com ovos emitidos nas fezes de cães domésticos ou de caça que previamente tenham ingerido vísceras de pacas portadoras da doença.Todos os sete pacientes com hidatidose policís tica que estudamos** 9, eram provenientes do Estado do Acre, e referiam contato com cães que freqüen temente se alimentavam com vísceras de pacas; 4 desses pacientes já haviam identificado pacas com doença no fígado, com características que julgamos altamente sugestivas de hidatidose. Com a finalidade de comprovar a doença hidática nesses hospedeiros intermediários, instruímos alguns de nossos pacientes

We report on the clinical evolution of the Brazilian family with Ramon syndrome described by de Pina-Neto et al. [1986, Am J Med Genet 25:441-443]. Three members (patients IV-2, IV-18, and IV-19) have developed pigmentary changes in the retina and paleness of the optic disk. Patient IV-18 also has developed giant hypertrophy of the labia minora that, when examined histopathologically, was found to be due to neoplastic fibroblast and epithelial proliferation caused by a fibromatous process similar to that reported in the gingivae of the patients with this syndrome. Audiologic function of patient IV-2 was normal, and no skin lesions were detected. The articular signs and symptoms show that the affected relatives developed rheumatoid arthritis, which is currently inactive in patient IV-18, whereas patient IV-2 did not develop these alterations.