Basal cell nevus syndrome (BCNS), also referred to as nevoid basal cell carcinoma syndrome or Gorlin-Goltz syndrome, was first described by Gorlin and Goltz in 1960 as an autosomal dominant disorder characterized by the early appearance of multiple basal cell carcinomas (BCCs), keratocysts of the jaw, ectopic calcifications, palmar and plantar pits, and anomalies of the ocular, skeletal, and reproductive systems. The genesis of this cancer's etiology in relation to BCNS was unclear until a few years ago when molecular analysis studies suggested a relationship between BCC and the loss-of-function mutations of the patched gene (PTCH) found on chromosome arm 9q. PTCH inhibits signaling by the membrane protein Smoothened (Smo), and this inhibition is relieved by binding sonic hedgehog (SHH) to PTCH. We describe a patient with multiple BCCs associated with x-ray anomalies of BCNS and review the basis of the SHH signaling pathway and clinical aspects of BCNS.

BackgroundSkin cancer is a highly prevalent condition with a multifactorial etiology resulting from genetic alterations, environmental and lifestyle factors. In Brazil, among all malignant tumors, skin cancers have the highest incidences.ObjectiveTo retrospectively evaluate the incidence, prevalence and profile of basal cell carcinoma, squamous cell carcinoma and cutaneous melanoma in Campos dos Goytacazes and region.MethodsIn total, 2,207 histopathological reports of a local reference hospital were analyzed between January 2013 and December 2015, of which 306 corresponded to the neoplasms studied.ResultsOf the 306 reports evaluated, 232 basal cell carcinomas (75.9%), 55 squamous cell carcinomas (18%) and 19 cutaneous melanomas (6.5%) were identified. The face was the most involved anatomical site (58.8%) and women (51%) were the most affected gender. The temporal analysis revealed a decrease in the overall incidence of 3.4% from 2013 to 2014 and 5.4% from 2014 to 2015. There was a 10.1% increase in basal cell carcinomas and 38% in melanomas in this period; however, there was a decrease in the number of squamous cell carcinomas of 14.8% during the studied years.Study limitationsSome samples of cutaneous fragments had no identification of the anatomical site of origin.ConclusionResearch that generates statistical data on cutaneous tumors produces epidemiological tools useful in the identification of risk groups and allows the adoption of more targeted and efficient future prevention measures.

GPD is an inflammatory dermatological condition represented by papulo-erythematous eruptions on perioral, nasal and periorbital regions, more prevalent in children and adolescents. It rarely extends to the genital region, trunk, and extremities, which characterizes its extra-facial manifestation. Its etiology is unknown, but it seems to have a correlation with the use of topical corticosteroids and other agents.

Marshall´s syndrome is a form of acquired cutis laxa without systemic involvement, which is preceded by an inflammatory dermatitis with a neutrophilic component. We report a case of a 6-year-old boy with clinical and histopathological features of this syndrome. The etiology remains unknown and there is no definitive treatment.

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Erysipelatoid Carcinoma (EC), also known as Inflammatory Metastatic Carcinoma, is a rare form of cutaneous metastasis, secondary to an internal malignancy, more often related to breast cancer. Clinically, the lesion has a well-marked, bound erythematous appearance, much like an infectious process, such as erysipelas and cellulitis, these being the most common differential diagnoses. It is characterized by an acute or subacute appearance with an erythematous plaque, sometimes hot and painful, being more often situated in the primary tumor vicinity, especially in the thorax wall in the region of a mastectomy due to breast cancer. Here we present the case of a 75-year-old patient with ductal infiltrated carcinoma for 3 years, who presented an acute erythematous and infiltrated plaque in the region of a previous mastectomy, with a final diagnosis of EC.

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