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https://www.readbyqxmd.com/read/27920986/fulminant-ecchymosis-as-the-initial-manifestation-of-antiphospholipid-syndrome-aps-triggered-by-respiratory-syncytial-virus-rsv-infection-a-case-report-and-review-of-the-literature
#1
Jun Makino, Sanjana Koshy, Sonal Bajaj, Young-Gwang Jeong, David C Perlman
We present a unique and informative instance of respiratory syncytial virus (RSV) infection associated with antiphospholipid syndrome (APS), and discuss this case in the context of the literature addressing the immunopathogenesis of APS associated with diverse infections. We describe the case of a 43-year-old man with no significant past medical history who presented with the acute onset of fever, hemoptysis, and extensive bullous, ecchymotic lesions in both lower extremities. Punch biopsy of the lesion demonstrated thrombotic vasculopathy...
2017: IDCases
https://www.readbyqxmd.com/read/27920816/concomitant-extraspinal-hyperostosis-and-osteoporosis-in-a-patient-with-congenital-ichthyosis
#2
Niloufar Torkamani, Pramit Phal, Ravi Savarirayan, Peter Simm, George Varigos, John Wark
Ichthyosiform dermatosis is a term referred to a group of disorders that have as their basis a disorder of keratinization (1). These conditions which are present at birth result in a generalized dry, scaly skin without any inflammation. There are several types of ichthyosis based on their clinical presentation and mode of inheritance. The most common types are: ichthyosis vulgaris, X-linked recessive ichthyosis, epidermolytic hyperkeratosis (bullous), lamellar ichthyosis and non-bullous ichthyosiform erythroderma...
May 2016: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/27920678/diffuse-bullous-eruptions-in-an-elderly-woman-late-onset-bullous-systemic-lupus-erythematosus
#3
Prajwal Boddu, Mojtaba Nadiri, Owais Malik
Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician. Diagnosis requires a careful review of clinical history, attention to detail on physical and histomorphological examination, and appropriate immunofluorescence testing. We describe the case of a 73-year-old female who presented to our hospital with a painful blistering skin rash developed over 2 days. Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities...
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27917718/the-role-of-rho-kinase-inhibitors-in-corneal-endothelial-dysfunction
#4
Naoki Okumura, Shigeru Kinoshita, Noriko Koizumi
BACKGROUND: The cornea is transparent tissue that serves as the window of the eye, allowing light to enter from the outer environment. The corneal endothelium maintains corneal transparency; thus, its dysfunction results in corneal haziness and causes severe vision loss. The only available therapeutic choice for treating corneal endothelial dysfunction has been transplantation using donor corneas; consequently, researchers have been pressed to develop new therapeutic options. METHODS: The goal of the review is to provide an overview of the research into Rho kinase (ROCK) inhibitors in the field of corneal endothelium therapy and the potential for clinical use of ROCK inhibitors...
December 5, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27914659/corneal-collagen-cross-linking-a-review-of-its-clinical-applications
#5
K Balparda, M J Maldonado
OBJECTIVE: To perform a literature review of the current clinical applications of corneal collagen cross-linking. METHODS: An exhaustive literature search was made, including the main biomedical databases, and encompassing all years since the introduction of cross-linking in ophthalmology practice. RESULTS: Corneal collagen cross-linking using UVA irradiation and riboflavin is a surgical technique that is currently being optimised, and is supported by a good amount of pre-clinical and clinical studies...
November 30, 2016: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/27914107/non-paraneoplastic-autoimmune-subepidermal-bullous-disease-associated-with-fatal-bronchiolitis-obliterans
#6
Mari Orime, Katsuhiro Tomiyama, Hideki Hashidate, Satoru Yoshida, Satoshi Hokari, Akiko Tsuda, Hisashi Yokoyama, Jun-Ichi Narita, Youhei Uchida, Takuro Kanekura, Riichiro Abe, Norito Ishii, Takashi Hashimoto, Kazuhiro Kawai
Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation...
December 3, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27902593/angiosarcoma-in-the-chest-radiologic-pathologic-correlation-case-report
#7
Sara Piciucchi, Alessandra Dubini, Sara Tomassetti, Stefano Sanna, Claudia Ravaglia, Angelo Carloni, Christian Gurioli, Carlo Gurioli, Thomas V Colby, Venerino Poletti
RATIONALE: Angiosarcomas are rare, malignant vascular tumors. PATIENT CONCERNS: They represents about 2% of all soft tissue sarcoma, which can often metastasize through the hematogenous route. The radiological features have been analyzed in 4 patients with metastatic angiosarcoma in the chest. DIAGNOSES: The main radiologic findings included nodules, cysts, nodules with halo sign, and vascular tree-in-bud. Morphologic features, as observed in the histologic specimen, have been correlated with radiologic appearance...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27899204/stevens-johnson-syndrome-in-childhood
#8
N Blanco, B Gutiérrez, I Valls, D Puertas, C Martín, M Rivera, Á Hernández, A Torrelo
INTRODUCTION: Stevens-Johnson syndrome and toxic epidermal necrolysis are two serious immune diseases within the context of bullous mucocutaneous syndrome. These have varying degrees of involvement of the skin and usually at least two mucous membranes. CLINICAL CASES: Three clinical cases are presented, two of them with significant ophthalmological sequelae, who had received drug treatment as a possible trigger, and another milder clinical case caused by Mycoplasma pneumoniae...
November 26, 2016: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/27895364/a-10-year-review-of-indications-for-penetrating-keratoplasty-in-a-tertiary-care-setting-in-karachi-pakistan
#9
Tanveer Ajum Chaudhry, Salman Naveed Sadiq, Zubaida Sirang, Maheen Akhtar Syed, Mustafa Kamal, Khabir Ahmad
The retrospective study was conducted to determine the indications and outomes of penetrating keratoplasty(PKP) in a tertiary care setting in Pakistan. All eyes that had undergone PKP between January 2005 and December 2014 at the Aga Khan University Hospital, Karachi, were included.Data were collected on the indications of PKP, graft survival (graft clarity at final follow-up), andbest corrected visual acuity (BCVA).Kaplan-Meier\'s method was usedto analyse graft survival.Out of 437 eyes, 383(87%) were for visual purpose...
October 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/27893467/bullous-pyoderma-gangrenosum-with-subungual-involvement-associated-with-ulcerative-colitis
#10
Ezgi Aktaş Karabay, Asl Aksu Cerman, İlknur Kvanc Altunay, Özben Yalçn
Pyoderma gangrenosum (PG) is a rare inflammatory and ulcerative skin disease of unknown etiology characterized by neutrophilic infiltration of the dermis, mainly affecting the lower extremities. Bullous PG is a rare variant of this disease, usually associated with hematologic disorders. Here, we report a case of pathergy-positive bullous PG with subungual involvement associated with ulcerative colitis.
November 22, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27885526/twelve-year-follow-up-of-penetrating-keratoplasty
#11
Takashi Ono, Sosuke Ishiyama, Takeshi Hayashidera, Yosai Mori, Ryohei Nejima, Kazunori Miyata, Shiro Amano
PURPOSE: To evaluate the long-term outcomes of penetrating keratoplasty (PKP) according to the corneal disease diagnosis and the number of PKP procedures performed. METHODS: Five-hundred-and-nine eyes from 403 patients who underwent PKP at Miyata Eye Hospital in Japan from 1998 through 2014, were included in this study. Medical charts were retrospectively examined to ascertain the corneal disease diagnosis and the period of graft survival. Graft survival rates were compared among various corneal disease diagnoses and among the number of PKP procedures performed...
November 24, 2016: Japanese Journal of Ophthalmology
https://www.readbyqxmd.com/read/27882389/linear-iga-bullous-dermatosis-associated-with-immunoglobulin-light-chain-amyloidosis
#12
Yasuyuki Yamaguchi, Hideyuki Ujiie, Hiroyuki Ohigashi, Hiroyuki Iwata, Ken Muramatsu, Tomoyuki Endou, Takanori Teshima, Hiroshi Shimizu
is missing (Short communication).
November 24, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27881946/atypical-clinical-presentation-of-lichen-planus-bullous-in-a-systemic-sclerosis-patient
#13
Zofia A Gerlicz-Kowalczuk, Jolanta D Torzecka, Marek Kot, Bożena Dziankowska-Bartkowiak
No abstract text is available yet for this article.
October 2016: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/27878477/paraneoplastic-pemphigus-and-autoimmune-blistering-diseases-associated-with-neoplasm-characteristics-diagnosis-associated-neoplasms-proposed-pathogenesis-treatment
#14
REVIEW
Saritha Kartan, Vivian Y Shi, Ashley K Clark, Lawrence S Chan
Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm...
November 22, 2016: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27878308/-systemic-lupus-erythematosus-unusual-cutaneous-manifestations
#15
REVIEW
T Stockinger, L Richter, M Kanzler, M Melichart-Kotik, H Pas, K Derfler, E Schmidt, K Rappersberger
BACKGROUND: Various different mucocutaneous symptoms may affect up to 80 % of systemic lupus erythematosus (SLE) patients. OBJECTIVES: To investigate, various unspecific, but otherwise typical clinical symptoms of skin and mucous membranes that arise in SLE patients other than those defined as SLE criteria such as butterfly rash, chronic cutaneous lupus erythematosus, oral ulcers, and increased photosensitivity. MATERIALS AND METHODS: Extensive search of peer-reviewed scientific articles was performed, medical histories of several SLE patients seen in our department were analyzed, and the rare disease courses in three SLE patients are presented...
December 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27876358/a-randomized-double-blind-trial-of-intravenous-immunoglobulin-for-bullous-pemphigoid
#16
Masayuki Amagai, Shigaku Ikeda, Takashi Hashimoto, Masato Mizuashi, Akihiro Fujisawa, Hironobu Ihn, Yasushi Matsuzaki, Mikio Ohtsuka, Hiroshi Fujiwara, Junichi Furuta, Osamu Tago, Jun Yamagami, Akiko Tanikawa, Hisashi Uhara, Akimichi Morita, Gen Nakanishi, Mamori Tani, Yumi Aoyama, Eiichi Makino, Masahiko Muto, Motomu Manabe, Takayuki Konno, Satoru Murata, Seiichi Izaki, Hideaki Watanabe, Yukie Yamaguchi, Setsuko Matsukura, Mariko Seishima, Koji Habe, Yuichi Yoshida, Sakae Kaneko, Hajime Shindo, Kimiko Nakajima, Takuro Kanekura, Kenzo Takahashi, Yasuo Kitajima, Koji Hashimoto
BACKGROUND: Patients with steroid-resistant bullous pemphigoid (BP) require an appropriate treatment option. OBJECTIVE: A multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic effect of high-dose intravenous immunoglobulin (IVIG; 400mg/kg/day for 5days) in BP patients who showed no symptomatic improvement with prednisolone (≥0.4mg/kg/day) administered. METHODS: We evaluated the efficacy using the disease activity score on day15 (DAS15) as a primary endpoint, and changes in the DAS over time, the anti-BP180 antibody titer, and safety for a period of 57days as secondary endpoints...
November 9, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/27874879/bullous-pemphigoid-associated-with-a-lymphoepithelial-cyst-of-the-pancreas
#17
Preston W Chadwick, Francis R Spitz, Daniel M Kwa, Waine C Johnson, Warren R Heymann
Bullous pemphigoid (BP) is an acquired, autoimmune, subepidermal blistering disorder. A possible paraneoplastic association has been suggested; however, debate remains regarding the precise relationship of these neoplasms with BP. We present a case of recalcitrant BP in a 67-year-old man with a pancreatic neoplasm that was found to be a lymphoepithelial cyst. Immunoperoxidase staining of the cyst demonstrated C3d along the basement membrane of the stratified squamous epithelium, suggesting that the BP may have involved the lymphoepithelial cyst itself...
October 2016: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/27869429/-topical-corticosteroids-as-a-therapeutic-alternative-in-linear-immunoglobulin-a-bullous-dermatosis-in-childhood-case-report
#18
Francisco José Gil Sáenz, Gabriel Durán Urdániz, Marta Fernández Galar, Juan Gimeno Ballester, Ana Herrero Varasa, Rosa Garcés Bordege
Linear immunoglobulin A dermatosis of childhood is a rare autoimmune disorder. Its etiology remains unknown, although it has been linked to drugs, infections, immunological diseases and lymphoproliferative processes. We report the case of a 6 year old girl who consulted for perioral bullous lesions without other symptoms. Neither treatment with mupirocin nor methylprednisolone therapy achieved remission of cutaneous lesions. Skin biopsy showed a linear immunoglobulin A dermatosis. It was not possible to start treatment with dapsone because of a partial glucose-6-phosphate dehydrogenase deficiency, so topical treatment was maintained with good evolution of lesions...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27868151/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-relapsing-as-bullous-eruption-a-rare-cutaneous-manifestation-with-an-emphasis-on-histopathological-features
#19
Shany Sherman, Noa Gal, Elena Didkovsky, Yair Molad, Lev Pavlovsky, Emmilia Hodak
is missing (Short communication).
November 10, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27868136/increased-activity-and-apoptosis-of-eosinophils-in-blister-fluids-skin-and-peripheral-blood-of-patients-with-bullous-pemphigoid
#20
Judith Engmann, Urda Rüdrich, Georg Behrens, Eleni Papakonstantinou, Manuela Gehring, Alexander Kapp, Ulrike Raap
Bullous pemphigoid (BP) is an autoimmune blistering skin disease that is more common in elderly individuals. The aim of this study was to determine the functional activity of eosinophils in patients with BP compared with healthy donors. Blood, skin and blister-derived eosinophils were strongly activated in patients with BP, seen by increased surface expression of CD69 compared with controls. CD11b was also increased in BP blood eosinophils, which may explain the striking accumulation of eosinophils in BP (1×10-6 per ml blister fluid)...
November 21, 2016: Acta Dermato-venereologica
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