ABSTRACT
The hyperimmunoglobulin E syndrome, or Job's syndrome is a rare primary immunodeficiency characterized by recurrent skin abscesses, recurrent respiratory tract infections, and high levels of IgE, eosinophilia, bone and dental changes. We report the case of a fourteen-year-old male patient presenting this disease, with both typical and also some relatively sporadic manifestations. We performed a literature review on the syndrome and its associated clinical findings.
A síndrome de hiperimunoglobulinemia E, ou síndrome de Job é uma imunodeficiência primária rara, caracterizada por abcessos cutâneos recorrentes, infecções de repetição do trato respiratório, títulos elevados de IgE, eosinofilia, alterações ósseas e dentárias. Relata-se o caso de um paciente do sexo masculino, 14 anos, portador da doença, com manifestações desde típicas a algumas relativamente esporádicas, fazendo-se uma revisão da síndrome e dos achados clínicos associados.
Subject(s)
Adolescent , Humans , Male , Job Syndrome/pathology , Skin Diseases/pathology , Immunoglobulin E/blood , Job Syndrome/physiopathology , Skin Diseases/physiopathologyABSTRACT
Molluscum contagiosum is a disease caused by a poxvirus. It is more prevalent in children up to 5 years of age. There is a second peak of incidence in young adults. In order to examine its ultrastructure, three lesions were curetted without disruption, cut transversely with a scalpel, and routinely processed for scanning electron microscopy (SEM). The oval structure of molluscum contagiosum could be easily identified. In its core, there was a central umbilication and just below this depression, there was a keratinized tunnel. Under higher magnification, a proliferation similar to the epidermis was seen. Moreover, there were areas of cells disposed like a mosaic. Under higher magnification, rounded structures measuring 0.4 micron could be observed at the end of the keratinized tunnel and on the surface of the lesion.
Molusco contagioso é uma dermatovirose causada por um poxvírus, sendo mais prevalente em crianças com até 5 anos de idade. Um segundo pico de incidência é encontrado em adultos jovens. Com o objetivo de demonstrar sua ultraestrutura três lesões foram curetadas sem rompê-las, cortadas transversalmente e processadas de rotina para microscopia eletrônica de varredura. A estrutura oval do Molusco contagioso pôde ser facilmente observada, no seu centro há uma umbilicação central e logo abaixo observa-se um túnel queratinizado. Com aumentos progressivos observam-se proliferações semelhantes à epiderme e áreas de células dispostas em mosaico. Com grandes aumentos estruturas arredondadas medindo 0,4 micron são vistas no final do túnel queratinizado e na superfície da lesão.
Subject(s)
Humans , Molluscum Contagiosum/pathology , Microscopy, Electron, ScanningABSTRACT
Molluscum contagiosum virus(MCV) infection occurs in both sporadic and epidemic forms, and affects children more frequently. The skin lesions are small, pinkish and wartlike tumor. MCV has not been transmitted to animals and nQt propagated in cell culture. Therefore the viruses in human lesions can only be demonstrated by electron microscopy. Histologically, characteristic intracytoplasmic inclusion bodies (called molluscum bodies) are formed in infected epithelial cells. They gradually enlarge and eventually fill the cell. Ultrastructure of molluscum bodies in infected cells showed a large number of MCV particles that are variable-sized, spherical, ellipsoidal and brick-shaped. Early viral replication stage shows small dense bodies in the nucleus and then reveal immature MCV that consist of homogeneous rounded core and separated capsidal membrane from the cytoplasm matrix In advanced stage, shrinked nucleus with irregular membrane moves into marginal area of the cell. The spherical fon-ns represented immature viruses in cytoplasm matrix, whereas the brick-sphaped forms represented mature viruses in the inclusion of cytoplasm.
Subject(s)
Child , Male , Female , Humans , AnimalsABSTRACT
BACKGROUND: Recent restriction enclonuclease analysis studies hsve revealed that MCV DNA can be classified into two major types, designated MCV-1 and MCV-2, by th:ir restriction enzyme cleavsge patterns. In earlier reports of MCV DNA analysis, MCV-2 was the main virus type found in genital lesions. However many recent studies cienied the relationship between virus type and anatomical distribution. OBJECTIVE: The purpose of this study was to examine the ratio of MCV-l to MCV-2 in Korean isolates of MCV DNA and the relationship between MCV subtypes and with clinical features such as anatomical location, age, sex, numiber of lesions, and atopic dermatitis. METHODS: MCV DNA extrated from 112 cases of Korean patients waa examined by restriction endonuclease analysis using Brtm HI. RESULTS: 1. MCV-1 was found in 108 of 112 (96.4%) patients and MCV-2 in of 112 (3.6%) patients. The ratio of MCV-1 to MCV-2 wss 28:1. 2. There was no significant ciprrelation between MCV subtypes and the age, sex, number of lesions, atopic dermatitis, and anatoimic loction. 3. Lesions induced by MCV-1 MCV-2 were indistinguishable on the brsis of size and form. CONCLUSION: This study showis that the ratio of MCV-1 to MCV-2 was 28:1 in Korean molluscum contagiosum patients and there was no relationship between MCV subtyies and lesional morphology or snatomical distribution.