RESUMO
Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder characterized by nevus sebaceus, seizures, and mental retardation, and is often accompanied by ocular anormalies. We recently experienced a case of conjunctival choristoma associated with linear nevus sebaceous syndrome in a 17-month-old boy. The child was the product of a full-term uncomplicated pregnancy and normal delivery with normal karyotype of 46, XY. He was noted at birth to have a conjunctival mass on the right eye and linear nevi over the midline facial region and right temporoparietal scalp. Ophthalmologic examination revealed optic atrophy, peripapillary staphyloma, macular hypogenesis, and osseous choristoma of the choroid. A computed tomography scan demonstrated widening of the subarachnoid space and moderate atrophy of the cerebral and cerebellar hemispheres. He has been followed by ophthalmology and completely free of seizures since birth. The patient underwent excision of the conjunctival mass and biopsies of the skin lesion at 17 months of age. Histologic examination of the skin biopsies was characteristic for the linear sebaceous nevus of Jadassohn. Histologic findings of the conjunctival lesion showed a complex choristoma that contained lacrimal gland tissue and cartilage.
Assuntos
Criança , Humanos , Lactente , Masculino , Gravidez , Atrofia , Biópsia , Cartilagem , Coristoma , Corioide , Deficiência Intelectual , Cariótipo , Aparelho Lacrimal , Síndromes Neurocutâneas , Nevo , Nevo Sebáceo de Jadassohn , Oftalmologia , Atrofia Óptica , Parto , Couro Cabeludo , Convulsões , Pele , Espaço SubaracnóideoRESUMO
The Kaposi's sarcoma, which was found in an immunosuppressed patient of renal transplantation, may have been developed by long term use of immunosuppressant agent after the renal transplantation. The case was a 29-year-old woman who was diagnosed as chronic renal failure in 1988, and since then, she had been on CAPD until May, 1997. After the renal transplantation in May 1997, the patient has been prescribed cyclosporin and prednisone as immunosuppressant agent. In June 1997, she showed clinical symptom of Kaposi's sarcoma with multiple papules and nodules in the skin and viscera, such as ureter, urinary bladder, stomach, duodenum and subcutaneous tissue of the chest. Multiple excisional biopsies were carried out in the skin, ureter, urinary bladder, stomach and duodenum. All of excisional biopses indicated nodular stages with extensive proliferation of spindle shaped, somewhat pleomorphic cells which have slit-like vascular spaces, proliferation of small vessels, and extravasation of erythrocytes. These lesions nearly diminished after sytemic chemotherpy, excision and discontinuity of immunosuppressive agents.
Assuntos
Adulto , Feminino , Humanos , Biópsia , Ciclosporina , Duodeno , Eritrócitos , Imunossupressores , Falência Renal Crônica , Transplante de Rim , Diálise Peritoneal Ambulatorial Contínua , Prednisona , Sarcoma de Kaposi , Pele , Estômago , Tela Subcutânea , Tórax , Ureter , Bexiga Urinária , VíscerasRESUMO
The Kaposi's sarcoma, which was found in an immunosuppressed patient of renal transplantation, may have been developed by long term use of immunosuppressant agent after the renal transplantation. The case was a 29-year-old woman who was diagnosed as chronic renal failure in 1988, and since then, she had been on CAPD until May, 1997. After the renal transplantation in May 1997, the patient has been prescribed cyclosporin and prednisone as immunosuppressant agent. In June 1997, she showed clinical symptom of Kaposi's sarcoma with multiple papules and nodules in the skin and viscera, such as ureter, urinary bladder, stomach, duodenum and subcutaneous tissue of the chest. Multiple excisional biopsies were carried out in the skin, ureter, urinary bladder, stomach and duodenum. All of excisional biopses indicated nodular stages with extensive proliferation of spindle shaped, somewhat pleomorphic cells which have slit-like vascular spaces, proliferation of small vessels, and extravasation of erythrocytes. These lesions nearly diminished after sytemic chemotherpy, excision and discontinuity of immunosuppressive agents.