Ictiosis canina, más allá de una simple descamación: Reporte de caso en un Boston terrier / Canine ichthyosis, beyond a simple scaling disorder: A case report in a Boston Terrier / Ictiose canina além de uma simples descamação: Relato de caso em um Boston terrier

Resumen La ictiosis canina es un trastorno queratoseborreico primario de carácter hereditario, el cual se ha reportado en Golden retriever, Bull dog americano, Jack Russell terrier, Cavalier King Charles spaniel y Gran danés. En el presente reporte se describe un caso clínico en un Boston terrier que desde cachorro ha presentado diferentes lesiones cutáneas, tiene años de evolución y varios tratamientos previos sin éxito. El diagnóstico se realizó mediante el descarte de otras dermatopatías y un estudio histopatológico. Se instauró un tratamiento multimodal de por vida para reestablecer la barrera cutánea y manejar el desorden de la cornificación.

Abstract Canine ichthyosis is a hereditary primary keratoseborreheic disorder, which has been reported in Golden retrievers, American bulldogs, Jack Russell terriers, Cavalier King Charles spaniel and Great danes. This report describes a clinical case in a Boston terrier that has presented multiples skin lesions since she was a puppy, has years of evolution and several previous unsuccessful treatments. The diagnosis was made by ruling out other dermatopathies and a histopathological study. Lifelong multimodal treatment was instaured to reestablish the skin barrier and manage the cornification disorder.

Resumo A ictiose canina é um transtorno queratosseborreico primário de caráter hereditário, sendo reportado em Golden retriever, Buldogue americano, Jack Russell terrier, Cavalier King Charles Spaniel e Dogue alemão. No presente relato se descreve um caso clínico em um Boston terrier que, desde filhote, apresentou diferentes lesões cutâneas, tendo evoluído por anos com tratamentos prévios sem êxito. O diagnóstico se realizou mediante o descarte de outras dermatopatias e um estudo histopatológico. Se instaurou um tratamento multimodal de uso contínuo, visando reestabelecer a barreira cutânea e manejar a desordem de cornificação.

Cell Death and Its Different Modes: History of Understanding and Current Trends

Discussions about what is life continue to struggle; there are pros and cons for whether a virus is alive. However, an opposite thing –cell death –appears to be tantamount important and equally not-easygoing to define. Nevertheless, our current knowledgeabout eukaryotic cell death has made a long way and resulted in a fruitful outcome: starting from three types of cell death (type I, II and III which are mainly applicable to eukaryotic cells of organisms from the biological kingdom animalia) in 1970s, Nomenclature Committee on Cell Death has named already twelve cell death forms in 2018, including the above mentioned apoptosis, autophagy and necrosis among them. How the scientific attitude towards cellular demise evolved and various aspects of different cell death modes are reviewed in this article.

Ictiosis hereditaria: desafío diagnóstico y terapéutico / Hereditary ichthyosis: A diagnostic and therapeutic challenge

Las ictiosis hereditarias son un grupo de trastornos genéticos de la cornificación, que se caracterizan por presentar hiperqueratosis y/o descamación. La nueva clasificación identifica 36 tipos de ictiosis, las cuales se subdividen según su frecuencia, patrón de herencia y compromiso extracutáneo. El diagnóstico se basa principalmente en las características clínicas, ya que los estudios genéticos no se encuentran disponibles en nuestro medio. El tratamiento es sintomático y su manejo debe ser realizado por un equipo multidisciplinario. En este artículo se revisan los aspectos diagnósticos y terapéuticos de los distintos tipos de ictiosis, considerando la nomenclatura y modificaciones expuestas en la nueva clasificación.

Hereditary ichthyoses are a group of genetic disorders of cornification, which are characterised by hyperkeratosis and scaling. The new classification identifies 36 types of ichthyosis, which are subdivided according to their frequency, pattern of inheritance and extracutaneous involvement. The diagnosis is mainly based on clinical features, since genetic studies are not available in our setting. Treatment is symptomatic and management should be performed by a multidisciplinary team. In this article, the diagnostic and therapeutic aspects of different types of ichthyosis are reviewed, taking into account the nomenclature and modifications presented in the new classification.

Characterization of mesenchymal cells beneath cornification of the fetal epithelium and epidermis at the face: an immunohistochemical study using human fetal specimens / 대한해부학회지

Fetal development of the face involves a specific type of cornification in which keratinocytes provide a mass or plug to fill a cavity. The epithelial-mesenchymal interaction was likely to be different from that in the usual skin. We examined expression of intermediate filaments and other mesenchymal markers beneath cornification in the fetal face. Using sections from 5 mid-term human fetuses at 14–16 weeks, immunohistochemistry was conducted for cytokeratins (CK), vimentin, nestin, glial fibrilary acidic protein, desmin, CD34, CD68 and proliferating cell nuclear antigen (PCNA). Fetal zygomatic skin was composed of a thin stratum corneum and a stratum basale (CK5/6+, CK14+, and CK19+) and, as the intermediate layer, 2–3 layered large keratinocytes with nucleus. The basal layer was lined by mono-layered mesenchymal cells (CD34+ and nestin+). Some of basal cells were PCNA-positive. In the keratinocyte plug at the external ear and nose, most cell nuclei expressed PCNA, CK5/6, CK14, and CK19. Vimentin-positive mesenchymal cells migrated into the plug. The PCNA-positive nucleus as well as mesenchymal cell migration was not seen in the lip margin in spite of the thick keratinocyte layer. The lingual epithelium were characterized by the CK7-positive stratum corneum as well as the thick mesenchymal papilla. CD68-positive macrophages were absent in the epidermis/epithelium. Being different from usual cornification of the skin, loss of a mesenchymal monolayer as well as superficial migration of mesenchymal cells might connect with a specific differentiation of keratinocyte to provide a plug at the fetal nose and ear.

Hiperqueratosis epidermolítica / Epidermolytic hyperkeratosis / Hiperqueratose epidermolítica

Existe un grupo de desórdenes hereditarios de la cornificación, caracterizado por una excesiva acumulación de escamas en la piel, que varía desde leve y asintomático hasta amenazar la vida. El término ictiosis es derivado de la raíz griega "icti" que significa pescado. La Hiperqueratosis Epidermolítica (HE), también conocida como eritrodermia ictiosiforme congénita, es un desorden autosómico dominante de la cornificación, con una prevalencia de 1:100.000-300.000. Esta patología se evidencia al nacer o al poco tiempo por la presencia de ampollas y de eritrodermia. Existe un gran número de tipos de ictiosis, de los cuales, la mayoría son extremadamente raros. Entre los cuatro más comunes se encuentran: Ictiosis vulgar, Ictiosis ligada al gen X, Ictiosis lamelar, hiperqueratosis epidermolítica. Se presenta el caso de un paciente de 13 años, con diagnóstico clínico de hiperqueratosis epidermolítica que es llevado a consulta médica por compromiso severo de la piel.

There is a group of hereditary disorders of cornification characterized by an excessive accumulation of scales in the skin, which can range anywhere from mild and non-symptomatic to life threatening. The term ichthyosis derives from the Greek root "icti", which means fish. Epidermolytic hyperkeratosis (EHK), also known as bullous congenital ichthyosiform erythroderma, is an autosomal dominant disorder of cornification with a prevalence of 1:100,000 ­ 300,000. EHK is evident at birth or soon thereafter, characterized by blistering and erythroderma. There are numerous types of ichthyosis, of which the majority are extremely rare. Vulgar ichthyosis, X-linked ichthyosis, lamellar ichthyosis, and epidermolytic ichthyosis are amongst the 4 most common types. The case report presented involves a 13-year-old patient with a clinical diagnosis of Epidermolytic hyperkeratosis who was taken to medical consultation presenting severely compromised skin.

Existe um grupo de desordens hereditárias da cornificação, caracterizados por uma excessiva acumulação de escamas na pele; a qual varia desde leve e assintomático até ameaçar a vida. O termo ictiosis é derivado da raiz grega "icti" o qual significa peixe. A Hiperqueratose epidermolítica (HE) também conhecida como eritrodermia ictiosiforme congênita, é uma desordem autossômico dominante da cornificación, com uma prevalência de 1:100.000 ­ 300.000. Esta patologia se evidência ao nascer ou ao pouco tempo deste; pela presença de ampolas e de eritrodermia. Existe um grande número de tipos de ictiosis, dos quais a maioria são extremamente raros. Entre os 4 mas comuns se encontram: Ictiosis vulgar, Ictiosis unida ao gene X, Ictiosis lamelar, hiperqueratose epidermolítica. Apresentasse-se o caso de um paciente de 13 anos, com diagnóstico clínico de hiperqueratose epidermolítica que é levado a consulta médica por compromisso severo da pele.

Lipids Analysis of Epidermis and Stratum corneum Using Circumcised Prepuce / 대한피부과학회지

BACKGROUND: The changes in lipid composition during epidermal differentiation has been reported in human and animal models. Because of the difficulties in getting adeguate specimens from human subjects, the authors used easily obtainable circumcised prepuce for lipid analysis. OBJECTIVE: To investigate the changes in lipid composition duriig cornification of the epidermis, the lipid compositions of whole epidermis and stratum corneum were analyzed by thin layer chromatography(TLC). METHODS: From circumcied prepuce whole epidermis and stratum orneum were separated by 10mM EDTA(ethylene diamine tatraacetate) in PBS(phosphate-buffered saline) or heat(60C), and 0.5% trypsin in PBS respectively. Lipids were extracted with methanolctloroform-HO mixture(4:2:1.6, v/ v, Bligh-Dyer solvent), TLC was performed and lipid composition was quantitated by photodensitometer. RESULTS: In the composition of stratum corneum lipids, sphingoliids were the highest(33.3+2.9%) followed by cholesterol, free fatty acids and cholesterol esters in cleceasing order, there were small percentages of triglycerides, cholesterol sulfate and squalene. CONCLUSION: In this study the lipid composition of epidermis was similar to that of stratum corneum rather than those of previous reports on epidermal lipids, which may indicate the regional characteristics of epidermal/stratum orneum lipids in hyperkeratotic prepuie.