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https://www.readbyqxmd.com/read/28528074/skin-perforator-flap-pedicled-by-intercostal-muscle-for-repair-of-a-tracheobronchoesophageal-fistula
#1
Nicolas Bertheuil, Caterina Cusumano, Cécile Meal, Yann Harnoy, Eric Watier, Bernard Meunier
A tracheobronchial fistula (TBF) is a rare complication when an operation is performed to treat esophageal carcinoma; no consensus treatment strategy has emerged. We describe a surgical interposition strategy, using a new flap, to repair a TBF that arose when esophageal squamous cell carcinoma was treated with neoadjuvant chemoradiation and minimally invasive esophagectomy (the 3-stage McKeown procedure). We performed a skin perforator flap pedicled by the intercostal muscle. It is a valuable option that may be the optimal first-line treatment, especially in the context of neoadjuvant radiation therapy...
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28525403/genetic-diseases-associated-with-an-increased-risk-of-skin-cancer-development-in-childhood
#2
Alexander L Fogel, Kavita Y Sarin, Joyce M C Teng
PURPOSE OF REVIEW: Childhood skin cancers are relatively rare and may indicate an underlying genetic disorder. The increasing elucidation of genetic pathways is changing the diagnosis and management of genetic skin cancer susceptibility syndromes. In this review, we provide an overview of genetic conditions that predispose to skin cancer development in childhood and signs that providers should assess when evaluating affected individuals. RECENT FINDINGS: In basal cell nevus syndrome (BCNS), the patched2 (PTCH2) and suppressor of fused (SUFU) genes have been implicated in disease pathogenesis...
May 18, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28523894/primary-cutaneous-mucormycosis-in-an-extremely-preterm-infant-successfully-treated-with-liposomal-amphotericin-b
#3
Christopher D Lowe, Rebecca J Sainato, David R Stagliano, Maribel M Morgan, Brian P Green
Cutaneous mucormycosis is a rare but often fatal invasive fungal infection that occurs most commonly in patients with diabetes, malignancy, and other immunocompromising conditions. We report an extremely preterm (<28 weeks) baby boy who developed polymicrobial sepsis and primary cutaneous mucormycosis within his first 10 days of life. He was successfully treated with medical management alone since he was not a candidate for surgery. Successful treatment of cutaneous mucormycosis without surgical debridement has been reported on only two other occasions...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28523885/intraepidermal-type-vii-collagen-by-immunofluorescence-mapping-a-specific-finding-for-bullous-dermolysis-of-the-newborn
#4
Gillian Heinecke, M Peter Marinkovich, Kerri E Rieger
BACKGROUND: Bullous dermolysis of the newborn (BDN) is a subtype of dystrophic epidermolysis bullosa (DEB) characterized by skin fragility and blister formation at birth that typically resolves within the first year of life. Abnormal intraepidermal retention of type VII collagen (C7) has been reported as a characteristic feature of BDN, but few studies have investigated the specificity of this finding. METHODS: We retrospectively reviewed pathology reports of patients diagnosed with DEB using immunofluorescence mapping from January 2001 to January 2015...
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28523880/halolike-phenomenon-around-a-caf%C3%A3-au-lait-spot-superimposed-on-a-mongolian-spot
#5
Iria Neri, Martina Lambertini, Vera Tengattini, Beatrice Rivalta, Annalisa Patrizi
An 8-month-old Caucasian infant with neurofibromatosis type 1 presented with a congenital plexiform neurofibroma and multiple café au lait spots. A pale area surrounded one of the café au lait spots located on the left gluteus in the area of dermal melanocytosis. This halolike phenomenon results from the disappearance of the Mongolian spot around the café au lait spots, revealing normal pigmented skin. This sign has been described rarely in the literature and the pathogenic mechanism is unclear.
May 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28522936/bladder-cancer-that-metastasized-to-the-skin-a-unique-presentation-that-signifies-poor-prognosis
#6
Tal Cohen, Daniel Ricchiuti, Mark Memo
In the United States in 2015, an estimated 74,000 new cases of bladder cancer were diagnosed and approximately 16,000 deaths were due to bladder cancer. We present a rare case of a patient with aggressive bladder cancer who presented with multiple inguinal and scrotal skin lesions that were proven to be metastatic urothelial malignancy. Bladder malignancy can involve the skin by direct tumor invasion, hematogenous routes, lymphatic spread, and direct seeding due to iatrogenic implantation. The cutaneous lesions have an extremely variable appearance, are resistant to therapies, and signify a dismal prognosis...
2017: Reviews in Urology
https://www.readbyqxmd.com/read/28522775/purpura-fulminans-mimicking-toxic-epidermal-necrolysis-additional-value-of-16s-rrna-sequencing-and-skin-biopsy
#7
K H W Dautzenberg, F N Polderman, R J van Suylen, M A M Moviat
Both purpura fulminans and toxic epidermal necrolysis (TEN) are rare and life-threatening disorders with a high mortality. We present a case of suspected rapidly progressive, severe pneumococcal sepsis-induced purpura fulminans complicated by multiple organ failure, severe epidermolysis and cutaneous necrosis. We show the diagnostic challenge to differentiate between purpura fulminans and TEN, as the extensive epidermolysis in purpura fulminans may mimic TEN and we highlight the additional value of repeated skin biopsies and 16S rRNA gene sequencing...
May 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28522647/prkar1a-mutation-causing-pituitary-dependent-cushing-disease-in-a-patient-with-carney-complex
#8
Florian W Kiefer, Yvonne Winhofer, Donato Iacovazzo, Marta Korbonits, Stefan Wolfsberger, Engelbert Knosp, Franz Trautinger, Romana Höftberger, Michael Krebs, Anton Luger, Alois Gessl
CONTEXT: Carney complex (CNC) is an autosomal dominant condition caused, in most cases, by an inactivating mutation of the PRKAR1A gene, which encodes for the type 1 alpha regulatory subunit of protein kinase A. CNC is characterized by the occurrence of endocrine overactivity, myxomas and typical skin manifestations. Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine disease observed in CNC. CASE DESCRIPTION: Here we describe the first case of a patient with CNC and adrenocorticotropic hormone (ACTH)-dependent Cushing disease due to a pituitary corticotroph adenoma...
May 18, 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28522113/neonatal-glaucoma-associated-with-juvenile-xanthogranuloma-case-report
#9
A Ramos Suárez, I Ayet Roger, A Serra Castanera
CASE REPORT: The case concerns a 22 day-old male child with juvenile xanthogranuloma (JXG), which manifested as a unilateral glaucoma and with fibrinous haemorrhagic exudate in the anterior chamber affecting the angle of the right eye. Despite a high level of suspicion, the definitive diagnosis was not possible until the infant reached the age of 10 months, after the appearance of the skin lesions typical of this condition and histopathological study of them. CONCLUSION: JXG is a rare disease, characterised by yellowish skin lesions on the trunk, neck, or head...
May 15, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28521835/adult-autoimmune-enteropathy-presenting-initially-with-acquired-acrodermatitis-enteropathica-a-case-report
#10
Erina Lie, Sarah Sung, Steven Hoseong Yang
BACKGROUND: Acrodermatitis enteropathica (AE) is a rare dermatitis secondary to zinc deficiency most commonly seen as an inherited disease in infants. In the last decade, increased number of reports have been published on the acquired form that presents in adulthood. Unlike its inherited counterpart, acquired AE (AAE) is often secondary to underlying pathologic or iatrogenic etiologies that interfere with nutritional absorption, such as inflammatory bowel disease or alcoholism. Various gastrointestinal pathologies have been associated with AAE, but there is currently no report on its association with adult autoimmune enteropathy (AIE), a rare gastrointestinal disorder commonly seen in infants, with limited cases reported in adults...
May 18, 2017: BMC Dermatology
https://www.readbyqxmd.com/read/28520453/-indeterminate-cell-histiocytosis-disappearance-of-skin-infiltration-following-electron-beam-therapy-and-an-application-of-2-chlorodeoxyadenosine-case-report
#11
Zdeněk Adam, Marta Ježová, Pavel Šlampa, Leoš Křen, Vladimír Vašků, Renata Koukalová, Zdeněk Řehák, Luděk Pour, Marta Krejčí, Zdeněk Král, Jiří Mayer
Indeterminate cell histiocytosis is a rare disease belonging to the group of malignant histiocytic diseases. The disease predominantly affects the skin. The disease appeared in the described patient at the age of 80 years. Morphs began to develop on the skin and rapidly spread over the whole body including the face. Only the hands and feet were left uncovered. The patients skin samples were taken from 2 sites for histological examination. The resulting conclusion was indeterminate cell histiocytosis. The treatment we chose was analogous to the procedures for Langerhans cell histiocytosis...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28520320/-pathergic-postsurgical-induced-pyoderma-gangrenosum
#12
N Wirtzfeld, P Paquet, T Lemineur, M Lutgen, J-D Bouaziz, C Denoel
Pyoderma gangrenosum (PG) is a rare pustular and ulcerative inflammatory disease belonging to the group of neutrophilic dermatoses. It is frequently associated with systemic immune diseases. In this context the PG can be exceptionally triggered by tissue trauma such as surgery (pathergy). We report the case of a patient with stabilized rheumatoid arthritis who developed aggressive and disseminated PG at all surgical wounds following an abdominal dermolipectomy associated with breast pexy. Systemic corticosteroid methylprednisolone allowed a rapid control of skin ulcerations...
May 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28518224/t-helper-1-related-molecules-as-well-as-il-15-are-hyperexpressed-in-the-skin-lesions-of-patients-with-pyoderma-gangrenosum
#13
Emiliano Antiga, Roberto Maglie, Walter Volpi, Beatrice Bianchi, Emilio Berti, Angelo Valerio Marzano, Marzia Caproni
Pyoderma gangrenosum (PG) is a rare, immune-mediated skin disease classified into the group of neutrophilic dermatoses. Whether a number of studies confirmed the central role of innate immunity, only few studies investigated the possible contributing role of acquired immunity. In particular, no reports about Th1 and Th2 cells are available yet. Therefore, 15 patients with PG, 5 with Sweet's syndrome (SS) and 9 skin specimens from healthy controls (HC) were investigated, evaluating the expression of Th1-related markers IL-12, IFN-γ, CXCR3, CCR5, of the Th2 related molecules IL-4, IL-5, IL-13 and CCR3, of the co-stimulatory axis CD40/CD40L, of IL-15 and the NK cell marker CD56 in skin lesions by immunohistochemistry...
May 18, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28516493/diagnosis-and-treatment-of-dermatophytosis-in-dogs-and-cats-clinical-consensus-guidelines-of-the-world-association-for-veterinary-dermatology
#14
REVIEW
Karen A Moriello, Kimberly Coyner, Susan Paterson, Bernard Mignon
BACKGROUND: Dermatophytosis is a superficial fungal skin disease of cats and dogs. The most common pathogens of small animals belong to the genera Microsporum and Trichophyton. It is an important skin disease because it is contagious, infectious and can be transmitted to people. OBJECTIVES: The objective of this document is to review the existing literature and provide consensus recommendations for veterinary clinicians and lay people on the diagnosis and treatment of dermatophytosis in cats and dogs...
June 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28515032/bilateral-painless-testicular-mass-acute-uveitis-and-annular-cutaneous-lesions-an-unusual-presentation-of-sarcoidosis-and-literature-review
#15
Enver Şimşek, Tülay Şimşek, Meliha Demiral, Funda Canaz, M Fuat Açıkalın
BACKGROUND: Sarcoidosis is a systemic inflammatory disease characterized by non-caseating epitheloid granulomas; whereas it usually involves the lungs and lymph nodes, genitourinary involvement is extremely rare. For this reason, no consensus regarding the diagnostic and management options for testicular sarcoidosis has been reached. OBJECTIVE: The aim of this case report was to describe an unusual clinical presentation of sarcoidosis. An 8-year-old boy presented with a 6-month history of painless scrotal enlargement and a 2-month history of photophobia, blurred vision, and pain in both eyes...
May 17, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28514997/the-prevalence-of-food-allergy-and-other-allergic-diseases-in-early-childhood-in-a-population-based-study-healthnuts-age-4-year-follow-up
#16
Rachel L Peters, Jennifer J Koplin, Lyle C Gurrin, Shyamali C Dharmage, Melissa Wake, Anne-Louise Ponsonby, Mimi L K Tang, Adrian J Lowe, Melanie Matheson, Terence Dwyer, Katrina J Allen
BACKGROUND: The HealthNuts study previously reported interim prevalence data showing the highest prevalence of challenge-confirmed food allergy in infants internationally. However, population-derived prevalence data on challenge-confirmed food allergy and other allergic diseases in preschool-aged children remain sparse. OBJECTIVE: This study aimed to report the updated prevalence of food allergy at age 1 year from the whole cohort, and to report the prevalence of food allergy, asthma, eczema, and allergic rhinitis at age 4 years...
April 25, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28514411/-langerhans-cell-histiocytosis-skin-diseases-and-visceral-lesions
#17
V D Elkin, T G Sedova, E A Kopytova, E V Plotnikova
Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder that belongs to class I histiocytoses and is characterized by infiltration of one or many organs by Langerhans cells to form granulomas. The literature analysis could identify a lot of etiological, pathogenetic, and trigger factors and mechanisms for LCH development, which determine the diversity of the clinical picture and course of the disease. The clinical manifestations of LCH are very variable and depend on the severity of lesions and the age of patients...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28513988/aquagenic-cutaneous-disorders
#18
REVIEW
Fang Wang, Yu-Kun Zhao, Ze-Yu Luo, Qian Gao, Wei Wu, Rashmi Sarkar, Di-Qing Luo
Aquagenic cutaneous disorders, which include aquagenic urticaria, aquagenic pruritus, and aquagenic acrokeratoderma, are a group of rare diseases characterized by skin lesions or discomfort induced by brief contact with water. Aquagenic urticaria is characterized by pruritic wheals that occur at the sites of water contact, either immediately or within minutes, and disappear within 30-60 min after water removal. Aquagenic pruritus presents with severe pruritus or a stinging, tingling, or burning sensation without any visible skin changes...
May 17, 2017: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
https://www.readbyqxmd.com/read/28512590/acute-bacterial-meningitis-and-systemic-abscesses-due-to-streptococcus-dysgalactiae-subsp-equisimilis-infection
#19
M Jourani, T Duprez, V Roelants, H Rodriguez-Villalobos, P Hantson
Disseminated abscesses due to group G β-hemolytic Streptococcus dysgalactiae were observed in a 57-year-old cirrhotic patient with the skin being the putative way of entry for the pathogen. S. dysgalactiae is a rare agent in human infections responsible for acute pyogenic meningitis. The mortality rate associated with S. dysgalactiae bacteraemia and meningitis may be as high as 50%, particularly in the presence of endocarditis or brain abscesses. In our patient, main sites of infections were meningitis and ventriculitis, spondylodiscitis, septic arthritis, and soft-tissue infections...
2017: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/28512406/a-case-of-subcutaneous-sarcoidosis-occurring-along-the-superficial-veins-of-the-forearms-a-distinctive-cutaneous-manifestation-masquerading-venous-tropic-action-in-the-underlying-systemic-disease
#20
Haruka Koizumi, Noritaka Oyama, Yukinori Hayakawa, Minoru Hasegawa
Sarcoidosis is a multisystem disease of unknown etiology, developing granulomas in any tissues and organs. Approximately 25% of sarcoidosis patients have cutaneous involvement with various clinical manifestations, which are categorized into specific or nonspecific diseases based on the histopathology; the former represents the typical sarcoid granulomas. Subcutaneous sarcoidosis is one of the specific skin lesions and often affects extremities, to a much lesser extent with other anatomical sites. Herein, we report the case of an 82-year-old Japanese man with subcutaneous sarcoidosis whose skin nodules exclusively overlay the lines of superficial veins on the forearms...
January 2017: Case Reports in Dermatology
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