Vulvar edema: diagnostic clue to Crohn disease.

Vulvar lesions are clinically challenging for physicians because the differential diagnosis may include many entities. Vulvar edema is one of the most frequent symptoms and is normally attributed to a local cause. Herein, we present a case report of vulvar Crohn disease (VCD) in a 9-year-old girl, in which skin lesions preceded the systemic gastrointestinal symptoms. Both clinical features and histopathological findings guided us to an early Crohn disease (CD) diagnosis. Dermatologists often have the opportunity to detect systemic diseases at early stages. A good knowledge of the CD skin manifestations could lead to an early CD diagnosis, especially in children. The suspicion of CD in those cases in which cutaneous involvement precedes digestive symptoms is crucial to prevent future psychological and physical consequences.

Fat1 deletion promotes hybrid EMT state, tumour stemness and metastasis.

FAT1, which encodes a protocadherin, is one of the most frequently mutated genes in human cancers1-5. However, the role and the molecular mechanisms by which FAT1 mutations control tumour initiation and progression are poorly understood. Here, using mouse models of skin squamous cell carcinoma and lung tumours, we found that deletion of Fat1 accelerates tumour initiation and malignant progression and promotes a hybrid epithelial-to-mesenchymal transition (EMT) phenotype. We also found this hybrid EMT state in FAT1-mutated human squamous cell carcinomas. Skin squamous cell carcinomas in which Fat1 was deleted presented increased tumour stemness and spontaneous metastasis. We performed transcriptional and chromatin profiling combined with proteomic analyses and mechanistic studies, which revealed that loss of function of FAT1 activates a CAMK2-CD44-SRC axis that promotes YAP1 nuclear translocation and ZEB1 expression that stimulates the mesenchymal state. This loss of function also inactivates EZH2, promoting SOX2 expression, which sustains the epithelial state. Our comprehensive analysis identified drug resistance and vulnerabilities in FAT1-deficient tumours, which have important implications for cancer therapy. Our studies reveal that, in mouse and human squamous cell carcinoma, loss of function of FAT1 promotes tumour initiation, progression, invasiveness, stemness and metastasis through the induction of a hybrid EMT state.

Titanium mesh in reconstructive surgery of the nasal pyramid. Follow-up of our 11 initial cases.

BACKGROUND: Titanium mesh has been employed in neurosurgery, plastic surgery, and maxillofacial surgery as a bone replacement in diverse conditions. We first reported on its use as a substitute for osteocartilaginous nasal structure with satisfactory functional and aesthetic results in 2009. OBJECTIVES: To assess the utility and tolerance of titanium mesh as a substitute of the osteocartilaginous portion of the nose. MATERIALS AND METHODS: Eleven patients were identified who had a nasal reconstruction with titanium mesh. We retrospectively searched for infection, extrusion, nasal valve collapse, second surgery, and patient satisfaction in the patients' records. All patients presented with various types of non-melanoma skin cancer in the nasal pyramid. In the reconstruction of full-thickness nasal defects, local flaps were used, employing titanium mesh as a support structure. RESULTS: No mesh extrusions, infections, or collapse of the nasal valve were noted in patients with a mean follow-up of five years. Only one patient needed a second surgery to debulk the flap. Patient satisfaction achieved a mean score of 7.5 out of 9 in 10 patients. CONCLUSIONS: As cartilage grafts need a second surgical site, with consequent morbidity, the use of titanium mesh proves useful and safe in the reconstruction of nasal full-thickness defects. The results of our series of patients demonstrate the suitability of this material in the repair of nasal osteochondral defects, as well as its low rate of complications.

Reconstruction of full-thickness defect of the alar rim with a combined advancement chondro-mucous and bilobed myocutaneous flap.

BACKGROUND: The nasal pyramid is frequently affected by nonmelanoma skin cancer. In full thickness defects of the nose, reconstruction of all the layers results in better outcomes. METHODS: We present a case in which the combination of two flaps adequately reconstructs a full thickness defect of the nasal ala. RESULTS: The images provide proof of the excellent functional and aesthetic results. CONCLUSIONS: Our technique is a simple, one-stage procedure, with no distant donor site morbidity that achieves good coverage of the internal nasal lining with good external aesthetic result.

Necrobiosis lipoidica therapy with biologicals: an ulcerated case responding to etanercept and a review of the literature.

Necrobiosis lipoidica (NL) is an idiopathic chronic granulomatous skin condition. There is currently no standardized effective treatment of NL. Ulceration occurs in up to 35% of cases. Treatment of ulcerative lesions is challenging and often unsuccessful. On the basis of the implication of the tumor necrosis factor alpha (TNF-alpha) on the formation of granulomas, since 2003 anti-TNF-alpha agents have been employed in cases of NL refractory to other therapeutic agents. We report a 50-year-old white woman with treatment-resistant chronic ulcerative NL of both shins successfully treated with subcutaneous etanercept. A review of the published literature suggests that biological agents (etanercept and infliximab) should be considered as a therapeutic alternative mainly in ulcerative NL unresponsive to prior conventional regimens. The dose and duration of treatment with these agents is not defined, therefore it is required to report management of these patients in order to develop an optimal therapeutic strategy.