Abstract: Panniculitis is a rare clinical finding in dermatomyositis, with less than 30 cases reported and there is only one case associated with the amyopathic subtype described in the literature. The present report describes a 49-year-old female patient that one year after being diagnosed with amyopathic dermatomyositis, presented indurated, painful, erythematous to violaceous nodules located on the upper limbs, thighs and gluteal region. Skin biopsy revealed lobular panniculitis with a lymphocytic infiltrate. The patient was treated with prednisone and methotrexate, but remained unresponsive to treatment.

Abstract Primary cutaneous B-cell lymphomas (PCBCLs) constitute 25% of all primary cutaneous lymphomas. They present in the skin with no evidence of systemic or extracutaneous disease at the time of diagnosis, after adequate staging. Primary cutaneous B-cell lymphomas differ significantly from their nodal counterparts in relation to both clinical behavior and prognosis. The distinction between primary and secondary B-cell lymphomas is essential for defining prognosis/course of action. Such distinction is also very difficult to make, since primary and secondary B-cell lymphomas are clinically and histologically indistinguishable. We report the case of a patient with primary cutaneous follicle center lymphoma who underwent surgical excision.

A síndrome de Bannayan-Riley-Ruvalcaba é afecção rara, causada por mutações no gene PTEN. Apresenta associação de múltiplos lipomas, macrocefalia, hemangiomas, polipose hamartomatosa intestinal, atraso do desenvolvimento e máculas salpicadas na genitália masculina. Relatamos o caso de um menino de nove meses com lesões tumorais de crescimento rápido e progressivo em três meses de evolução, macrocefalia e lentigos no pênis. Exames de imagem demonstraram extensa lipomatose com invasão da musculatura paraespinhal, alargamento do canal vertebral e compressão medular. Depois da excisão cirúrgica da massa, o anatomopatológico foi compatível com lipoma. A análise do cariótipo em cultura de adipócitos demonstrou mutação do PTEN. Apresentamos este caso por sua exuberância e raridade.

Bannayan-Riley-Ruvalcaba Syndrome is a rare condition caused by mutations in the PTEN gene. It displays association of multiple lipomas, macrocephaly, hemangiomas, hamartomatous intestinal polyposis, developmental delay and speckled pigmented maculae on the male genitalia. We report the case of a nine-month-old boy who had fast growing and progressive tumors for three months, macrocephaly and lentigines on the penis. Imaging tests showed extensive lipomatosis with invasion of paraspinal muscles, enlargement of the spinal canal and spinal cord compression; after surgical excision of the mass, the pathology was consistent with lipoma. Adipocyte culture karyotype demonstrated PTEN mutation. We present this case for its rarity and exuberance.

Tumor de células granulares é uma neoplasia incomum com origem nas células de Schwann. Raramente tem origem no trato respiratório, havendo descrição de apenas 32 pacientes com tumor primário situado na traquéia. Relata-se o caso de uma mulher, jovem, portadora de tumor de células granulares da traquéia, tratada cirurgicamente com bons resultados.

Granular cell tumors are uncommon neoplasm arising from Schwann cell. They are uncommon in the respiratory tract and there are only 32 cases described with primary tracheal involvement. A case is described of a 28 year-old woman with granular cell tumor located primarily in the trachea. The tumor was treated surgically with good result.