Abstract:
:Muckle-Wells syndrome is a rare autosomal dominant disease that belongs to a group of hereditary febrile syndromes. It is characterized by recurrent and self-limited episodes of fever, urticaria, arthralgia, myalgia and conjunctivitis since childhood, which are related to exposure to cold temperatures. Lately, progressive sensorineural hearing loss occurs. Amyloidosis is the main complication and can be found in about 25% of the cases. It has been demonstrated that there is an association with mutations in the NLRP3 gene, which codifies cryopyrin, a protein responsible for regulating the production of proinflammatory cytokines, such as interleukin-1Beta. The authors report four cases of the disease within a family.
journal_name
An Bras Dermatoljournal_title
Anais brasileiros de dermatologiaauthors
Pereira AF,Pereira LB,Vale EC,Tanure LAdoi
10.1590/s0365-05962010000600022subject
Has Abstractpub_date
2010-11-01 00:00:00pages
907-11issue
6eissn
0365-0596issn
1806-4841pii
S0365-05962010000600022journal_volume
85pub_type
杂志文章abstract::Reports of child abuse have increased significantly. The matter makes most physicians uncomfortable for two reasons: a) Little guidance or no training in recognizing the problem; b - Not understanding its true dimension. The most common form of child violence is physical abuse. The skin is the largest and frequently t...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962011000300015
更新日期:2011-05-01 00:00:00
abstract:BACKGROUND:Pemphigusis a bullous, rare and chronic autoimmune disease. There are two major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and clinical outcome in patients diagnosed in the Brazilian Amazon states are still rare. OBJECTIVES:To study the occurrence of the disease during the study period...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20142679
更新日期:2014-07-01 00:00:00
abstract:BACKGROUND:The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. OBJECTIVES:We aimed to assess the ABQOL and TABQOL in the Arabic population. METHODS:The ...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20197198
更新日期:2019-01-01 00:00:00
abstract::Christ-Siemens-Touraine syndrome (hypohidrotic ectodermal dysplasia) is a rare syndrome characterized by the triad of absent or reduced sweating, hypotrichosis, and defective dentition. The prominent forehead, saddle nose, thick lower lip and pointy chin produce a distinctive facies. The full syndrome only occurs in m...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962009000200015
更新日期:2009-03-01 00:00:00
abstract:BACKGROUND:Skin cancer is common in Brazil and is related to sun exposure, among other risk factors. There are no data on the incidence of malignant skin neoplasm in rural workers in western Paraná. OBJECTIVE:To analyze the incidence and profile of rural workers who were diagnosed with skin cancer at a reference servi...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20197335
更新日期:2019-03-01 00:00:00
abstract::Sweet's syndrome may be associated with hematological malignancies, particularly with acute myelogenous leukemia, but there are few reports of its association with polycythemiavera. We describethe case of a 65-year-old male patient, diagnosed with polycythemiavera, which developedinto paraneoplastic Sweet's syndrome. ...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962009000600014
更新日期:2009-11-01 00:00:00
abstract::Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do t...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章,评审
doi:10.1590/abd1806-4841.20187459
更新日期:2018-06-01 00:00:00
abstract:BACKGROUND:Vitiligo is a chronic disease characterized by the appearance of achromic macules caused by melanocyte destruction. Surgical treatments with melanocyte transplantation can be used for stable vitiligo cases. OBJECTIVES:To evaluate treatment response to the autologous transplantation of noncultured epidermal ...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20175700
更新日期:2017-05-01 00:00:00
abstract::Woolly hair nevus is a rare condition characterized by a structural anomaly of the hair, restricted to certain areas of the scalp. The hair becomes coiled and slightly hypopigmented. The term woolly hair refers to changes that affect all the scalp and has a hereditary character. We present a case of woolly hair nevus,...
journal_title:Anais brasileiros de dermatologia
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doi:10.1590/abd1806-4841.20175289
更新日期:2017-01-01 00:00:00
abstract::Noonan Syndrome is one of the most common genetic syndromes and also an important differential diagnosis in children presenting with syndromic facies similar to Turner's syndrome phenotype. This syndrome is characterized by facial dysmorphism, congenital heart defects, short stature and also a wide phenotypic variatio...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20131934
更新日期:2013-07-01 00:00:00
abstract::Adult-onset Still's disease is a systemic inflammatory disorder of unknown etiology, characterized by skin rash, spiking fever, arthralgias or arthritis, and leukocytosis. The typical skin rash is evanescent, salmon-pink, nonpruritic and maculopapular, predominantly on the extremities. It is considered one of the majo...
journal_title:Anais brasileiros de dermatologia
pub_type:
doi:10.1590/abd1806-4841.20186836
更新日期:2018-01-01 00:00:00
abstract::Calciphylaxis, also known as calcific uremic arteriolopathy, is a severe complication often observed in patients with hyperparathyroidism secondary to chronic renal failure, which occurs mainly in women. It is characterized by ischemic tissue loss secondary to progressive vascular compromise. This is a rare and severe...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962012000500014
更新日期:2012-09-01 00:00:00
abstract::A 37-year-old man complained of a refractory posterior malleolar ulceration on his left ankle. He was diagnosed with Werner syndrome according to the progeroid clinical features and genetic testing. To approach the ulceration, a free flow-through right anterolateral thigh perforator flap with anterolateral thigh cutan...
journal_title:Anais brasileiros de dermatologia
pub_type:
doi:10.1590/abd1806-4841.20187517
更新日期:2018-01-01 00:00:00
abstract::Eccrine syringofibroadenoma is a rare benign neoplasia derived from acrosyryngium cells of the eccrine sudoriferous glands. It affects the extremities of elderly individuals as solitary tumors, or may also present as multiple lesions. There are controversies about the pathogenesis and differentiation of the tumor. Ecc...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:
更新日期:2014-05-01 00:00:00
abstract::Lobomycosis or lacaziosis is a chronic granulomatous fungal infection caused by Lacazia loboi. Most cases are restricted to tropical regions. Transmission is believed to occur through traumatic inoculation in the skin, mainly in exposed areas. It is characterized by keloid-like nodules. There are only a few hundred ca...
journal_title:Anais brasileiros de dermatologia
pub_type:
doi:10.1590/abd1806-4841.20187044
更新日期:2018-03-01 00:00:00
abstract::Ultraviolet radiation can damage the DNA, cause immunosuppression, chemical and histological alterations in the epidermis, early photoaging, cataracts and carcinogenesis, among others. Photoprotection prevents these and other harmful effects of ultraviolet radiation. Sunscreens, protective clothing, proper accessories...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章,评审
doi:10.1590/s0365-05962011000400016
更新日期:2011-07-01 00:00:00
abstract::Basal Cell Carcinoma is the most common skin cancer. We describe a single-staged technique for correction of nasal ala defect after the excision of a basal cell carcinoma. This technique allows correction of surgical defects of the ala rebuilding the original anatomy, maintaining cosmetic units, without need for a gra...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962011000700042
更新日期:2011-07-01 00:00:00
abstract::Metastatic ovarian cancer uncommonly presents with skin metastasis. When present, skin metastases of ovarian cancer are usually localized in the vicinity of the primary tumor. We report a case of a 58-year-old woman with a rapid growing erythematous, well-defined nodule localized on the left nasal ala. A skin biopsy w...
journal_title:Anais brasileiros de dermatologia
pub_type:
doi:10.1590/abd1806-4841.20165024
更新日期:2016-09-01 00:00:00
abstract::Reticulate acropigmentation of Dohi is a rare dyschromic disorder of autosomal dominant inheritance. Most cases have been originally described in Japan. The case of a girl with lesions of typical distribution and morphology is reported. Skin biopsy was not considered essential for diagnosis. After literature review, i...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962010000100020
更新日期:2010-01-01 00:00:00
abstract::Whether parapsoriasis represents an early stage of T-cell cutaneous lymphoma is still the subject of controversy. We evaluated the efficacy of phototherapy in the treatment of parapsoriasis and its relation with TCCL. Patients diagnosed with parapsoriasis and treated with phototherapy PUVA or UVB-NB were selected. Bet...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/S0365-05962013000200028
更新日期:2013-03-01 00:00:00
abstract::Dyschromicum erythema perstans, or ashy dermatosis, is a rare chronic acquired skin disease characterized by gray hyperpigmented patches with erythematous borders. Its etiology is unknown and there is no specific treatment for the condition. We report a case of ashy dermatosis in a 41-year-old patient with extensive l...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962012000100025
更新日期:2012-01-01 00:00:00
abstract::The purpose of this study is to compare scanning electron microscopy findings of the blister roof in three distinct bullous diseases: one intraepidermal acantholytic (pemphigus foliaceus); one due to hemidesmosomal dysfunction (bullous pemphigoid); and one secondary to anchoring fibril dysfunction - type VII collagen ...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20142161
更新日期:2014-03-01 00:00:00
abstract::Atopic dermatitis is a common inflammatory skin disease. New understanding in disease pathogenesis has led to a considerable number of promising new drugs in development. New topical agents can be especially helpful for children, providing an alternative to the need for chronic topical corticosteroid use. While many p...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20187682
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND:Dysplastic nevi are among the most important cutaneous melanoma simulators. They are important risk markers for this neoplasia and can be its potential precursors. Some authors found a statistically significant relationship between the degree of dysplasia and the risk for developing melanoma. However, reprod...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962013000100002
更新日期:2013-01-01 00:00:00
abstract::Pyoderma gangrenosum is a rare neutrophilic dermatosis of unknown etiology, of which the most frequent clinical manifestations are ulcers. The diagnosis difficulty is, among other things, to rule out other causes of ulcers, since it is considered a diagnosis of exclusion. Skin ulcerations may also occur in the iliac v...
journal_title:Anais brasileiros de dermatologia
pub_type:
doi:10.1590/abd1806-4841.20176109
更新日期:2017-01-01 00:00:00
abstract::The objective of this study was to analyze the trend of epidemiological and operational indicators of leprosy in Brazil, from 2001 to 2017. This was a time series study involving nine indicators. The inflection point regression model was used. Decreasing trends were observed for the following: general detection (-4.8%...
journal_title:Anais brasileiros de dermatologia
pub_type:
doi:10.1016/j.abd.2019.09.031
更新日期:2020-01-01 00:00:00
abstract::Tattoos are defined as the intentional or accidental deposit of pigment into the skin. These pigments have been associated with various dermatoses such as allergic contact dermatitis, lichenoid dermatitis, photoinduced reactions, and granulomatous, sarcoid and pseudolymphomatous reactions. The objective of this report...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/s0365-05962010000500019
更新日期:2010-09-01 00:00:00
abstract::Targetoid Hemosiderotic Hemangioma, also known as Hobnail Hemangioma, is a lesion of vascular origin, probably lymphatic. The most common clinical feature is a solitary violaceous papule surrounded by a pale, thin area and a peripheral ecchymotic ring, simulating a target. Histopathologically, there is a biphasic patt...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20143264
更新日期:2014-11-01 00:00:00
abstract::Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章,评审
doi:10.1590/s0365-05962009000100008
更新日期:2009-01-01 00:00:00
abstract:BACKGROUND:The behaviour of each basal cell carcinoma is known to be different according to the histological growth pattern. Among these aggressive lesions, sclerodermiform basal cell carcinomas are the most common type. This is a challenging-to-treat lesion due to its deep tissue invasion, rapid growth, risk of metast...
journal_title:Anais brasileiros de dermatologia
pub_type: 杂志文章
doi:10.1590/abd1806-4841.20186699
更新日期:2018-01-01 00:00:00