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Case for diagnosis; Caso para diagnóstico

Gonçalves, Sheila Viana Castelo Branco; Valente, Neusa Yuriko Sakai; Oliveira Junior, José Vitor de; Paiva, Daniele Loureiro Mangueira
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica
ENG
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27.97%
Porokeratosis is a primary keratinizing disorder of unknown etiology. This disorder is characterized by the presence of centrifugally enlarging hyperkeratotic plaques, associated with the histopathological hallmark of cornoid lamellae. Genital porokeratosis is extremely rare. No more than thirty cases have been reported in the literature, including only one case of linear porokeratosis confined to the genital area. This case report describes a patient with genital linear porokeratosis, who was successfully treated with cryotherapy. Over two years of follow-up, the lesion improved and there was no evidence of recurrence or signs of malignant transformation. Nevertheless, there is a need for long-term follow-up data on recurrence and malignant transformation.

Case for diagnosis

Gonçalves,Sheila Viana Castelo Branco; Valente,Neusa Yuriko Sakai; Oliveira Junior,José Vitor de; Paiva,Daniele Loureiro Mangueira
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/06/2012 EN
Relevância na Pesquisa
27.97%
Porokeratosis is a primary keratinizing disorder of unknown etiology. This disorder is characterized by the presence of centrifugally enlarging hyperkeratotic plaques, associated with the histopathological hallmark of cornoid lamellae. Genital porokeratosis is extremely rare. No more than thirty cases have been reported in the literature, including only one case of linear porokeratosis confined to the genital area. This case report describes a patient with genital linear porokeratosis, who was successfully treated with cryotherapy. Over two years of follow-up, the lesion improved and there was no evidence of recurrence or signs of malignant transformation. Nevertheless, there is a need for long-term follow-up data on recurrence and malignant transformation.

Genitogluteal porokeratosis - Case report

Ferreira,Flavia Regina; Lessa,Priscila Pacheco; Alvarenga,Marcia Lanzoni de
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/06/2013 EN
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37.63%
We report the case of a patient diagnosed with genitogluteal porokeratosis, a disorder of epidermal keratinization. The location described is extremely rare and very often late diagnosed or even misdiagnosed. Histopathology showed a typical cornoid lamella of great value to support this diagnosis. The importance of awareness of this entity by the specialist is emphasized as a differential diagnosis among genital diseases of chronic evolution and difficult treatment.

Unilateral punctate porokeratosis - Case report

Teixeira,Vera Barreto; Reis,Jose Pedro; Vieira,Ricardo; Tellechea,Oscar; Figueiredo,Americo
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/06/2013 EN
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37.83%
This case report involves a 20-year-old man with unilateral punctate porokeratosis. The patient presented an 8-year history of numerous asymptomatic keratotic papules and pits with linear distribution on his left pal-mar surface and fifth finger of the left hand. Histopathological examination of the keratotic plug revealed findings of distinct epidermal depressions containing cornoid lamellae. This report review draws attention to differential diagnoses of punctate porokeratosis.

Porokeratosis of Mibelli - literature review and a case report

Ferreira,Flávia Regina; Santos,Leopoldo Duailibe Nogueira; Tagliarini,Fernando Augusto Nogueira Mendes; Lira,Marcia Lanzoni de Alvarenga
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/12/2013 EN
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37.97%
Porokeratosis is a disorder of epidermal keratinization characterized by annular plaques with an atrophic center and hyperkeratotic edges, and includes a heterogeneous group of disorders that are mostly inherited in an autosomal dominant form. This report describes a 5 year-old female patient, with porokeratosis of Mibelli confirmed histopathologically. The rarity of this disorder, its clinical exuberance and the destructive character of the lesions, as well as the facial and mucosal involvements, unusual in this form of porokeratosis, and also its onset in early childhood motivated this report.

Genital porokeratosis of Mibelli.

Neri, I; Marzaduri, S; Passarini, B; Patrizi, A
Fonte: PubMed Publicador: PubMed
Tipo: Artigo de Revista Científica
Publicado em /12/1995 EN
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27.97%
Porokeratosis of Mibelli is a disorder of epidermal proliferation in which many different clinical forms can be distinguished. Two male patients with a localized type of porokeratosis limited to the genitalia are reported. Later in life they developed an annular skin lesion with peripheral keratotic ridge. The histological examination of a biopsy specimen showed the characteristic features of porokeratosis. There was no family history of similar skin disorders and the patients were not on any drugs. Genital porokeratosis is probably underdiagnosed and we believe that these patients should be followed up on account of the precancerous potential of this disease.

Atypical Porokeratosis Developing Following Bone Marrow Transplantation in a Patient with Myelodysplastic Syndrome

Cha, Sang Hee; Park, Hyun Jeong; Lee, Jun Young; Cho, Baik Kee
Fonte: Korean Dermatological Association; The Korean Society for Investigative Dermatology Publicador: Korean Dermatological Association; The Korean Society for Investigative Dermatology
Tipo: Artigo de Revista Científica
EN
Relevância na Pesquisa
27.83%
Porokeratosis is an abnormal disease of keratinization of epidermis. It is clinically characterized by margins covered with keratin layer and it typically has an atrophied macule with a protruded, circular form. Histopathologically, it shows the findings of cornoid lamella. Risk factors for its development include organ transplantation, long-term use of corticosteroids, immunocompromised status, including AIDS, and exposure to ultraviolet light. We herein report a case of atypical porokeratosis in a 38-year-old man who developed porokeratosis involving multiple sites following bone marrow transplantation for myelodysplastic syndrome.

Porokeratosis: Two Faces, One Family

Lembo, S.; Panariello, L.; Nugnes, L.; Lembo, C.; Alaya, F.
Fonte: S. Karger AG Publicador: S. Karger AG
Tipo: Artigo de Revista Científica
Publicado em 02/10/2009 EN
Relevância na Pesquisa
27.97%
Porokeratosis is a disorder of keratinisation whose pathogenesis is yet unclear. It has been postulated that it results from the proliferation of an abnormal clone of keratinocytes, triggered by several factors, such as immunosuppression or prolonged ultraviolet exposure. Various clinical forms are recognized whose common denominator is a keratotic ring surrounding a central zone of atrophy. The histological hallmark is the cornoid lamella, a thin column of hyperproliferative abnormal keratinocytes. We describe two cases of porokeratosis. A 67-year-old woman with an erythematous purplish round plaque surrounded by a keratotic border that had appeared 6 years previously on the left sural region was diagnosed as ‘giant’ porokeratosis. A 49-year-old man presented with small papules coalescent in an erythematous oval plaque on the lateral side of the left foot consistent with linear porokeratosis.

A Case of Linear Porokeratosis Superimposed on Disseminated Superficial Actinic Porokeratosis

Löhrer, Rebecca; Neumann-Acikel, Aysegül; Eming, Rüdiger; Hartmann, Karin; Rasokat, Heinrich; Krieg, Thomas; Happle, Rudolf; Eming, Sabine
Fonte: S. Karger AG Publicador: S. Karger AG
Tipo: Artigo de Revista Científica
Publicado em 06/08/2010 EN
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27.97%
We present a female patient with linear porokeratosis of her right arm since childhood. At the age of 67 years she additionally developed disseminated superficial actinic porokeratosis (DSAP) involving both lower legs. This uncommon coexistence of two different types of porokeratosis fulfils the clinical criteria of a type 2 segmental manifestation of an autosomal dominant skin disorder, being superimposed on the ordinary nonsegmental lesions and reflecting loss of heterozygosity that occurred at an early developmental stage. In DSAP molecular evidence of this concept is so far lacking, but such proof has already been provided in several other autosomal dominant skin disorders. Molecular analysis of cases of type 2 segmental involvement may help elucidate the genetic defect causing DSAP.

Acute onset disseminated superficial porokeratosis associated with exacerbation of diabetes mellitus due to development of anti-insulin antibodies

Nakamura, Motonobu; Fukamachi, Shoko; Tokura, Yoshiki
Fonte: Landes Bioscience Publicador: Landes Bioscience
Tipo: Artigo de Revista Científica
Publicado em //2010 EN
Relevância na Pesquisa
27.83%
The development of disseminated superficial porokeratosis is occasionally observed in association with renal transplant, autoimmune diseases and various hematological disorders, suggesting a certain immunosuppression may trigger a widespread abnormal keratinization. Here we report a case of sudden onset disseminated superficial porokeratosis associated with an exacerbation of diabetes mellitus due to an anti-insulin antibody formation. To our knowledge, this is the first report of disseminated superficial porokeratosis in a patient with severe diabetes mellitus.

Squamous Cell Carcinoma Developing within Lesions of Disseminated Superficial Actinic Porokeratosis

Lee, Hyung Rae; Han, Tae Young; Son, Sook-Ja; Lee, June Hyunkyung
Fonte: Korean Dermatological Association; The Korean Society for Investigative Dermatology Publicador: Korean Dermatological Association; The Korean Society for Investigative Dermatology
Tipo: Artigo de Revista Científica
EN
Relevância na Pesquisa
27.83%
Disseminated superficial actinic porokeratosis (DSAP) consists of multiple annular, hyperkeratotic lesions that have a bilateral distribution on sun-exposed areas, particularly the extremities. DSAPs have a wider distribution than porokeratosis of Mibelli and usually develop during the 3rd or 4th decade of life. Squamous cell carcinoma that arises in the classical type of porokeratosis of Mibelli is well-documented, but there are only a few reports of squamous cell carcinoma in DSAP. Here, we describe a 62-year-old man with DSAP who developed squamous cell carcinoma on his right forearm.

Identification of an Interstitial 18p11.32-p11.31 Duplication Including the EMILIN2 Gene in a Family with Porokeratosis of Mibelli

Occella, Corrado; Bleidl, Dario; Nozza, Paolo; Mascelli, Samantha; Raso, Alessandro; Gimelli, Giorgio; Gimelli, Stefania; Elisa Tassano,
Fonte: Public Library of Science Publicador: Public Library of Science
Tipo: Artigo de Revista Científica
Publicado em 10/04/2013 EN
Relevância na Pesquisa
28.14%
Porokeratosis is a rare disease of epidermal keratinization characterized by the histopathological feature of the cornoid lamella, a column of tightly fitted parakeratocytic cells, whose etiology is still unclear. Porokeratosis of Mibelli is a subtype of porokeratosis presenting a single plaque or a small number of plaques of variable size located unilaterally on limbs. It frequently appears in childhood and occurs with a higher incidence in males. Cytogenetic analyses were performed in all members of the family on lesioned and uninvolved skin. An array-CGH analysis was also performed utilizing the Human Genome CGH Microarray Kit G3 400 with 5.3 KB overall median probe spacing. Gene expression was performed on skin fibroblasts. In this study, we describe a Caucasian healthy 4-year-old child and his father showing features of porokeratosis of Mibelli. Array-CGH analysis revealed an interstitial 429.5 Kb duplication of chromosome 18p11.32-p11.3 containing four genes, namely: SMCHD1, EMILIN2, LPIN2, and MYOM1 both in patient and his father. EMILIN2 resulted overexpressed on skin fibroblasts. Also other members of this family, without evident signs of porokeratosis, carried the same duplication. Among these genes, we focused our attention on elastin microfibril interfacer 2 (EMILIN2) gene. Apoptosis plays a fundamental role in maintaining epidermal homeostasis...

Disseminated Superficial Actinic Porokeratosis on the Face Treated with Imiquimod 5% Cream

Riad, Hassan; Mansour, Khaled; Sada, Hussein Al; Shaika, Samya Abu; Ansari, Hamda Al; Mohannadi, Haya Al
Fonte: S. Karger AG Publicador: S. Karger AG
Tipo: Artigo de Revista Científica
Publicado em 09/10/2013 EN
Relevância na Pesquisa
27.97%
Porokeratoses are a group of hereditary or acquired disorders characterized by annular lesions with an atrophic center and a prominent peripheral ridge. Pathologically, porokeratosis is characterized by the presence of abnormal clones of keratinocytes that form a column of parakeratotic cells, called the cornoid lamella. Fifteen percent of patients of disseminated superficial actinic porokeratosis (DSAP) have facial lesions; other regions like the extensor surface of extremities constitute the majority of reported cases. Exclusively facial lesions are probably less frequent. Rarely, actinic porokeratosis is confined to the nose only. Of reported porokeratosis cases, 7.5% have revealed a malignancy arising within the lesion. We present a case of facial sporadic DSAP that was treated with imiquimod 5% cream in conjunction with a regular sunscreen. Follow-up of this case is important to rule out the possibility of malignant transformation of the lesions.

Follicular Porokeratosis at alae Nasi; A Case Report and Short Review of Literature

Rifaioğlu, Emine N; Özyalvaçlı, Gülzade
Fonte: Medknow Publications & Media Pvt Ltd Publicador: Medknow Publications & Media Pvt Ltd
Tipo: Artigo de Revista Científica
Publicado em //2014 EN
Relevância na Pesquisa
27.97%
Porokeratosis are disorders of keratinization, characterized histologically by a parakeratotic column, the cornoid lamellae, and clinically by a distinct peripheral ridge. Porokeratosis usually occurs on the trunk or extremities, and facial lesions are rare. Follicular involvement, identified by follicular localization of cornoid lamellae, is uncommon and described only in association with disseminated superficial actinic porokeratosis (DSAP) and porokeratosis Mibelli (PM). A 34-Year- old woman is presented with asymptomatic 1 cm plaque lesion with hyperkeratotic ridge and depressed center on her nose since three years. Histopathological examination of a biopsy revealed cornoid lamellae confined exclusively to the follicle.

Clinical analysis and etiology of porokeratosis

GU, CHAO-YING; ZHANG, CHENG-FENG; CHEN, LIAN-JUN; XIANG, LEI-HONG; ZHENG, ZHI-ZHONG
Fonte: D.A. Spandidos Publicador: D.A. Spandidos
Tipo: Artigo de Revista Científica
EN
Relevância na Pesquisa
28.32%
The present study was performed in order to define the clinical manifestations of porokeratosis, with particular emphasis on genital porokeratosis. A total of 55 cases of porokeratosis were retrospectively reviewed between 2000 and 2007 from Huashan Hospital (Shanghai, China). Out of 55 cases, there were 22 cases of porokeratosis of Mibelli, 17 cases of disseminated superficial actinic porokeratosis (DSAP), 15 cases of disseminated superficial porokeratosis and one case of linear porokeratosis. The ratio of males to females was 39:16. Among them, 12 cases had a family history of porokeratosis. During the five-year follow-up period, no malignant transformation was observed and no further aggravation of lesions was detected. The results indicated that the initial region of DSAP in the Chinese population may differ from Caucasians. In combination with other studies, the present study found that genital porokeratosis in the Chinese population is often associated with pruritus. Since no recurrence was observed in cases treated with surgical excision, it was suggested that surgical excision is a viable treatment strategy and should be used for porokeratotic lesions if possible. In addition, regular follow-ups are required, since the aggravation of porokeratosis may cause the development of malignancy transformation.

A Case of Disseminated Superficial Porokeratosis Associated with Giant Porokeratosis in Pregnancy

Vasudevan, Biju; Chatterjee, Manas; Grewal, Rajan; Rana, Vandana; Lodha, Nikita
Fonte: Medknow Publications & Media Pvt Ltd Publicador: Medknow Publications & Media Pvt Ltd
Tipo: Artigo de Revista Científica
Publicado em //2014 EN
Relevância na Pesquisa
27.97%
A 23-year-old pregnant lady presented with dark raised lesions over face, axillae, and upper limbs of 15-day duration. She was 35 weeks pregnant at the time of onset of the lesions. Dermatological examination revealed hyperpigmented plaques on the face and papules with raised borders in the axillae and proximal arms. Skin biopsy from both the lesions revealed a diagnosis of porokeratosis. She was treated with emollients alone and the lesions regressed four weeks following delivery. This case is being reported for the rare occurrence of the combination of disseminated superficial porokeratosis with giant porokeratosis in pregnancy.

Porokeratosis - Head to toe: An unusual presentation

Bhaskar, Sapnashree; Jaiswal, Ashok K.; Raj, Niranjana; Reddy, Divya
Fonte: Medknow Publications & Media Pvt Ltd Publicador: Medknow Publications & Media Pvt Ltd
Tipo: Artigo de Revista Científica
Publicado em //2015 EN
Relevância na Pesquisa
28.14%
Porokeratosis is a disorder of epidermal proliferation in which many different clinical variants can be distinguished. Herein we report a rare case of porokeratosis involving almost all parts of the body with simultaneous occurrence of various forms of the disease in the same individual (classical porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, hypertrophic porokeratosis, and genital porokeratosis).

Porokeratosis ptychotropica of the scrotum: dermoscopic evaluation of an atypical presentation*

Cabete, Joana; Fidalgo, Ana; Lencastre, André; Diamantino, Filipa; João, Alexandre
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica
Publicado em //2015 EN
Relevância na Pesquisa
28.07%
Porokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.

Porokeratosis ptychotropica of the scrotum: dermoscopic evaluation of an atypical presentation

Cabete,Joana; Fidalgo,Ana; Lencastre,André; Diamantino,Filipa; João,Alexandre
Fonte: Sociedade Brasileira de Dermatologia Publicador: Sociedade Brasileira de Dermatologia
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/06/2015 EN
Relevância na Pesquisa
38.07%
AbstractPorokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.

Porokeratosis Ptychotropica of the Scrotum: Dermoscopic Evaluation of an Atypical Presentation

Cabete, J; Fidalgo, A; Lencastre, A; Diamantino, F; João, A
Fonte: Scielo Publicador: Scielo
Tipo: Artigo de Revista Científica
Publicado em //2015 ENG
Relevância na Pesquisa
38.14%
Porokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.