Ultrastructural aspects of pseudoxanthoma elasticum

Abstract: We report the ultrastructural findings in a case of a 72-year-old black woman with confluent yellowish papules in the cervical region. She had no comorbidities. Ophthalmological examination, electrocardiogram, and echocardiogram were normal. Hematoxylin-eosin staining of the affected skin showed strong alterations in the mid-dermis with irregular clumps of eosinophilic material and loss of the normal parallel arrangement of collagen bundles. Orcein staining revealed that the elastic fibers lost their normal linear configuration, showing clump fragmentation, sometimes forming square structures. Transmission electron microscopy showed aberrant elastic fibers with an irregular outline and heterogenic inner structures. We also observed small elastic fibers. Collagen fibers showed a normal structure with irregular distribution. Scanning electron microscopy revealed important disorganization of collagen fibers and small stone-like deposits measuring around 5 µm associated with bigger structures ranging from 10-16 µm. Higher magnification revealed that these small stone-like structures were sometimes polyhedral-shaped or squared.

Oclusão da artéria central da retina em paciente com poliangeíte microscópica / Central retinal artery occlusion in patient with microscopic polyangiitis

RESUMO A poliangeíte microscópica é uma vasculite necrotizante sistêmica que acomete arteríolas, capilares e vênulas, mas também pode atingir pequenas e médias artérias. É considerada uma doença rara, idiopática e autoimune. Diversas anormalidades oculares e sistêmicas estão associadas às oclusões arteriais retinianas. Dentre as doenças vasculares do colágeno, a literatura cita como possíveis causas de obstrução das artérias retinianas o lúpus eritematoso sistêmico, a poliarterite nodosa, a arterite de células gigantes, a granulomatose de Wegener e a granulomatose linfóide de Liebow. Até o presente momento, não se encontrou na literatura relatos da associação de casos de oclusão arterial retinana associados à PAM. Os autores relatam o caso de um paciente com poliangeíte microscópica que apresentou comprometimento renal importante e oclusão da artéria central da retina unilateral. Atenta-se para a inclusão de pesquisa da PAM, através do p-ANCA, na avaliação de possível origem sistêmica em pacientes acometidos por oclusão arterial retiniana.

ABSTRACT The microscopic polyangiitis is a systemic necrotizing vasculitis that affects arterioles, capillaries and venules, but can also reach small and medium-sized arteries. It is considered a rare disease, idiopathic in nature but clearly autoimmune. Several ocular and systemic abnormalities are associated with retinal arterial occlusions. Among the collagen vascular diseases, the literature cited as possible causes of retinal artery obstruction lupus erythematosus, polyarteritis nodosa, giant cell arteritis, Wegener’s granulomatosis and lymphoid Liebow. Until now, there were no reports in the literature of the association of cases of arterial occlusion retinana associated with PAM. The authors report a case of a 53 years old patient diagnosed with microscopic polyangiitis who presented with important renal artery occlusion and associated unilateral central retinal artery occlusion. An extended systemic evaluation of patients presenting with central retinal artery occlusion should include research of PAM through analysis op p-ANCA.

Three-dimensional aspects of superficial disseminated porokeratosis with scanning electron microscopy

The three-dimensional findings of the surface and from a cross section from a case of disseminated superficial porokeratois using scanning electron microscopy are reported. On the surface of the skin, irregular keratin with a serpiginous distribution was seen. A gross aspect of keratin in the hyperkeratotic wall was also observed and compared to the normal area, in which the release of corneocytes seemed normal. The cross-sectional imaging easily identified the cornoid lamella, with compact keratin surrounded by normal stratum corneum.

Tireóide lingual / Lingual thyroid

A tireóide lingual é uma doença rara, definida como presença de tecido glandular tireóideo localizado ectopicamente em base de língua. Deve ser suspeitada em paciente que apresente massa de aspecto submucoso em base de língua, sem tireóide identificada no pescoço. Apresenta-se o relato do caso de uma paciente de 45 anos, discutindo-se as características clínicas, diagnóstico e opções terapêuticas.

Lingual thyroid is a rare disease, defined as the presence of ectopic glandular thyroid tissue localized in the base of tongue. It must be suspected in a patient who presents a mass of submucous aspect in the base of tongue without the thyroid gland identifiable in the neck. It is presented a case report of a 45-year-old woman patient discussing the clinical aspects, diagnose and therapeutic options.