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Dermatology- uncommon disorders

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11 papers 100 to 500 followers
By Faye Kehler Family Physician and GP Anesthetist since 1987 interested in all aspects of Medicine
https://www.readbyqxmd.com/read/27745906/interventional-treatments-for-hailey-hailey-disease
#1
Benjamin Farahnik, Collin M Blattner, Michael B Mortazie, Benjamin M Perry, William Lear, Dirk M Elston
Hailey-Hailey disease or familial benign chronic pemphigus is a rare blistering dermatosis that is characterized by recurrent erythematous plaques with a predilection for the skin folds. For extensive Hailey-Hailey disease that is recalcitrant to conventional therapy, laser ablation, photodynamic therapy, electron beam radiotherapy, botulinum toxin type A, dermabrasion, glycopyrrolate, and afamelanotide have been reported as useful treatments, but comparative trials are lacking. This review discusses the various treatment modalities for Hailey-Hailey disease and a summary of the evidence for the most recommended treatments...
October 13, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27609179/monogenic-polyarteritis-the-lesson-of-ada2-deficiency
#2
REVIEW
Roberta Caorsi, Federica Penco, Francesca Schena, Marco Gattorno
The deficiency of Adenosine Deaminase 2 (DADA2) is a new autoinflammatory disease characterised by an early onset vasculopathy with livedoid skin rash associated with systemic manifestations, CNS involvement and mild immunodeficiency.This condition is secondary to autosomal recessive mutations of CECR1 (Cat Eye Syndrome Chromosome Region 1) gene, mapped to chromosome 22q11.1, that encodes for the enzymatic protein adenosine deaminase 2 (ADA2). By now 19 different mutations in CECR1 gene have been detected...
September 8, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27380773/atypical-clinical-presentation-of-primary-and-secondary-cutaneous-follicle-center-lymphoma-fcl-on-the-head-characterized-by-macular-lesions
#3
Cesare Massone, Regina Fink-Puches, Lorenzo Cerroni
BACKGROUND: Primary cutaneous follicle center lymphoma (pcFCL) usually presents with reddish nodules, plaques, and tumors on the head and neck or the back. OBJECTIVE: We sought to describe a peculiar clinical presentation of pcFCL and secondary cutaneous follicle center lymphoma (FCL). METHODS: We report a series of 13 patients (7 male and 6 female; median age 58 years, mean age 58.2 years, age range 26-83 years) with either pcFCL (11 patients) or secondary cutaneous FCL (2 patients) presenting with lesions on the head deviating from the classic clinical manifestations characterized by plaques and tumors...
July 2, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27050235/images-in-clinical-medicine-creeping-eruption-cutaneous-larva-migrans
#4
Dong-Lai Ma, Sergio Vano-Galvan
A 42-year-old man presented with a 1-month history of an intensely pruritic eruption on his right foot after a vacation in Nigeria. The eruption was migratory, moving a few millimeters to a few centimeters daily. Physical examination revealed a serpiginous, erythematous raised tract on his right..
April 7, 2016: New England Journal of Medicine
https://www.readbyqxmd.com/read/26973062/heat-urticaria-a-revision-of-published-cases-with-an-update-on-classification-and-management
#5
REVIEW
E Pezzolo, A Peroni, P Gisondi, G Girolomoni
Heat urticaria (HU) is a rare type of physical inducible urticaria, characterized by itchy erythema and well-demarcated weals appearing soon after heat exposure. Most cases occur in female patients aged 20-45 years. Both localized and generalized forms exist, depending on the limitation of the reaction to the skin area directly exposed to the physical stimulus or the involvement of distant sites, respectively. In most cases, HU is an immediate reaction, but delayed forms (mostly familial) have been described...
September 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/26816014/images-in-clinical-medicine-primary-and-secondary-syphilis
#6
Michael P Schön, Hans P Bertsch
A previously healthy 68-year-old man presented with a solid erosive sore on the inner aspect of his upper lip (Panel A). The painless lesion had been present for approximately 6 weeks. On examination, the patient had sharply demarcated erythematous, scaly plaques on his hands and feet, including..
January 28, 2016: New England Journal of Medicine
https://www.readbyqxmd.com/read/25583369/mycophenolate-mofetil-in-erosive-genital-lichen-planus-a-case-and-review-of-the-literature
#7
Kristyn Deen, Erin McMeniman
Erosive genital lichen planus is a disabling, inflammatory mucocutaneous condition that can cause significant patient morbidity and loss of function. Treatment initially involves topical corticosteroids but some patients can have severe treatment-resistant courses requiring systemic immunosuppression. With potentially unfavorable adverse effect profiles and subsequent intolerance of these agents by patients, erosive lichen planus can ultimately be a challenging condition to treat effectively. We present a case of a 66-year-old woman with treatment-resistant erosive genital lichen planus who was successfully managed with mycophenolate mofetil...
March 2015: Journal of Dermatology
https://www.readbyqxmd.com/read/25348001/targeting-tumor-infiltrating-b-cells-in-cutaneous-t-cell-lymphoma
#8
Sebastian Theurich, Max Schlaak, Harold Steguweit, Lukas C Heukamp, Kerstin Wennhold, Peter Kurschat, Anja Rabenhorst, Karin Hartmann, Hans Schlösser, Alexander Shimabukuro-Vornhagen, Udo Holtick, Michael Hallek, Rudolf Stadler, Michael von Bergwelt-Baildon
No abstract text is available yet for this article.
April 20, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/25228963/pseudoexfoliation-syndrome-and-cardiovascular-diseases
#9
REVIEW
Georgios K Andrikopoulos, Dimitrios K Alexopoulos, Sotirios P Gartaganis
Pseudoexfoliation (PEX) syndrome is a well-recognized late-onset disease caused by a generalized fibrillopathy. It is linked to a broad spectrum of ocular complications including glaucoma and perioperative problems during cataract surgery. Apart from the long-known intraocular manifestations, PEX deposits have been found in a variety of extraocular locations and they appear to represent a systemic process associated with increased cardiovascular and cerebrovascular morbidity. However, as published results are inconsistent, the clinical significance of the extraocular PEX deposits remains controversial...
August 26, 2014: World Journal of Cardiology
https://www.readbyqxmd.com/read/25162635/disseminated-lyme-disease-presenting-with-nonsexual-acute-genital-ulcers
#10
Justin J Finch, Jenna Wald, Katalin Ferenczi, Saima Khalid, Michael Murphy
IMPORTANCE: Nonsexual acute genital ulceration (NAGU) is a rare vulvar skin condition typically affecting girls and young women, characterized by acute onset of singular or multiple painful vaginal ulcers. The etiology of this ulcerative dermatosis has not been identified, although it has been associated with systemic infections. To our knowledge, this is the first report of an association with Lyme disease. OBSERVATIONS: A case of a woman with early disseminated Lyme disease presenting with NAGU is reported...
November 2014: JAMA Dermatology
https://www.readbyqxmd.com/read/24836079/subsequent-cancers-mortality-and-causes-of-death-in-patients-with-mycosis-fungoides-and-parapsoriasis-a-danish-nationwide-population-based-cohort-study
#11
Lise M Lindahl, Morten Fenger-Grøn, Lars Iversen
BACKGROUND: Data on subsequent cancers, prognostic factors for mortality, and causes of death are limited in mycosis fungoides (MF) and parapsoriasis. OBJECTIVES: To assess subsequent cancers, mortality, and causes of death in MF and parapsoriasis. METHODS: Using the Danish nationwide population-based registries, we identified 368 MF patients and 582 parapsoriasis patients and compared them with the general Danish population for subsequent cancers, mortality, and causes of death...
September 2014: Journal of the American Academy of Dermatology
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