[Cardiac arrest caused by anomalous origin and course of the right coronary artery].

The anomalous origin of the right coronary artery from the left sinus with interarterial course is a rare but life-threatening coronary abnormality. Coronary computed tomography is crucial in identifying this disease whose treatment, based on coronary artery bypass grafting, is recommended in symptomatic patients but is more controversial in asymptomatic patients. The case report presented offers an opportunity to discuss the pathophysiological, diagnostic and therapeutic aspects of this congenital coronary artery disease.

Tumors in patients with neurofibromatosis type 1: a single- center retrospective study.

Objective: To investigate the risk and pattern of tumors in italian neurofibromatosis type 1 (NF1) patients.

Materials And Methods: A retrospective single institution case review of 711 patients (seen between March 1992 and February 2018) with NF1 was conducted to identify individuals with diagnoses of both NF1 and neoplasm. NF1-associated tumors have been collected and analyzed.

Mite bites, comet signs and possible mammary prosthesis rejection after returning to a vacation home: a diagnostic challenge.

Pyemotes ventricosus is a free-living mite feeding on larvae or nymphs of insects, including moths, beetles, wasps and bees, that are usually found in grain, straw and firewood. When present in great number or when its food is lacking, it could accidentally bite mammals, including humans, causing a highly pruritic self-limiting dermatitis, sometimes followed by a lymphangitis known as "comet sign".We present a singular case of mite lymphangitis that surrounds and delimitates breast prosthesis in a 30-year-old Caucasian woman.

Ectropion Uveae in neurofibromatosis type 1: a new manifestation.

Neurofibromatosis type 1 (NF1) is a rare genetic disorder with an autosomal dominant transmission and an estimated incidence of 1:2500-3500 live birth. Penetrance is virtually 100%, but the expression is highly variable and almost every organ can be affected. Diagnosis of NF1 is made with at least two of the following diagnostic criteria: six or more cafè-au-lait spots, two neurofibromas or one plexiform neurofibroma, axillary or groin freckling, optic glioma, two Lisch nodules, bone dysplasia and first-degree relative with NF1.

Madelung's disease. Two case reports with pseudoathletic appearance.

Madelung's disease is a rare syndrome characterized by the pre-sence of multiple masses of unencapsulated adipose tissue, symme-trically distributed throughout different regions. It predominantly affects middle-aged men of Mediterranean origin with a history of alcoholism. The pathogenesis is still unknown.

Neurofibromatosis Type 1: Ocular Electrophysiological and Perimetric Anomalies.

Introduction: Neurofibromatosis type 1 (NF1) is a multisystemic disease caused by the mutation of gene located on chromosome 17q11.2. The mutation determines the loss of function of the protein neurofibromin with consequent uncontrolled cellular proliferation.

Ultrastructure study of skin fibroblasts in patients with Ehlers-Danlos Syndrome (EDS): preliminary results.

Aim Of The Study: To investigate, in vivo and in vitro, the fibroblast-to-myofibroblast transition in patients with hypermobile Ehlers-Danlos Syndrome (EDS). To analyze the dermis of patients with classical form of EDS (cEDS) and with hEDS, to identify qualitative and/or quantitative differences in ECM component and ultrastructural changes in collagen.

Materials And Methods: Seven subjects, aged over 18, two with cEDS and five with hEDS underwent two skin biopsy.

Lichen sclerosus and hidradenitis suppurativa: Two case reports of a new possible association.

We report the cases of two women affected by lichen sclerosus also having clinical signs of hidradenitis suppurativa. Lichen sclerosus is a chronic autoimmune disease, in which activated fibroblasts produce significantly altered collagen leading to fibrosis Hidradenitis suppurativa is a chronic relapsing inflammatory disease affecting folliculopilosebaceous unit and apocrine gland, which lesions are nodules and abscesses. The association between lichen sclerosus and autoimmune disorders is well known, but not the one with hidradenitis suppurativa.

Ocular surface involvement in patients with neurofibromatosis type 1 syndrome.

Purpose: The aim of this study is to evaluate ocular surface morphological and functional changes in patients with neurofibromatosis type 1 (NF1).

Methods: Twenty-eight patients with NF1 and 14 healthy subjects were included in this study. All participants underwent a medical history collection, a complete ophthalmological examination including slit lamp exam and assessment of best-corrected visual acuity (BCVA), corneal sensitivity, and lacrimal function (Schirmer test and fluorescein tear break-up time test).

Tridimensional Matryoshka Tattoo: An Important.

Dear Editor, Tattooing is a global and ancient practice that has endured until the present day. It was originally used to indicate religious beliefs, tribal affiliation, loyalty to a leader, or had a therapeutic function. Adverse reactions from tattooing are common, and cutaneous reactions to red pigment have been widely reported (1,2).

Ocular manifestations in Gorlin-Goltz syndrome.

Background: Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare genetic disorder that is transmitted in an autosomal dominant manner with complete penetrance and variable expressivity. It is caused in 85% of the cases with a known etiology by pathogenic variants in the PTCH1 gene, and is characterized by a wide range of developmental abnormalities and a predisposition to multiple neoplasms. The manifestations are multiple and systemic and consist of basal cell carcinomas in various regions, odontogenic keratocistic tumors and skeletal anomalies, to name the most frequent.

Linear cutaneous hypopigmentation and atrophy associated with intralesional steroid injection: A rarely described adverse reaction.

Intralesional steroid injection is a common and highly effective treatment procedure, which can be successfully used for several dermatologic, rheumatologic, and orthopedic disorders. However, it may be associated with various local side effects, such as pain, granulomatous reaction, allergic reactions, hypo or hyperpigmentation and, uncommonly, skin atrophy. A peculiar and rare side effect of steroid injection is linear hypopigmentation and atrophy, which is probably an expression of lymphatic spread of corticosteroid crystals.

Lichen sclerosus et atrophicus and allergic contact dermatitis: A significant association.

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory skin disease of unknown etiology. It mainly affects the anogenital area, while the extragenital involvement is uncommon. It manifests as pale, ivory-colored lesions and partially atrophic skin.

Retinal microvascular abnormalities in neurofibromatosis type 1.

Purpose: The aim of this study was to provide a classification of the different retinal vascular arrangements in neurofibromatosis 1 (NF1), with appropriate qualitative and quantitative information.

Methods: This study was conducted on 334 consecutive patients with NF1 and 106 sex-matched and age-matched healthy control subjects. Each patient underwent a comprehensive ophthalmological examination inclusive of near-infrared reflectance retinography by using the spectral domain Optical coherence tomography (OCT), a complete dermatological examination and 1.